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Valvular
Heart Disease
A Guide for Cardiovascular
Nurses and Allied Health
Professionals
Marian C. Hawkey
Sandra B. Lauck
Editors
123
Valvular Heart Disease
Marian C. Hawkey • Sandra B. Lauck
Editors

A Guide for Cardiovascular Nurses
and Allied Health Professionals
Editors
Marian C. Hawkey Sandra B. Lauck
Hackensack University Medical Center St. Paul’s Hospital
Hackensack, NJ University of British Columbia
USA Vancouver, BC
Canada
ISBN 978-3-030-86232-9 ISBN 978-3-030-86233-6 (eBook)

https://doi.org/10.1007/978-3-030-86233-6
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature
Switzerland AG 2022
This work is subject to copyright. All rights are solely and exclusively licensed by the Publisher, whether
the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of
illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and
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The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication
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protective laws and regulations and therefore free for general use.
The publisher, the authors and the editors are safe to assume that the advice and information in this book
are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the
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This Springer imprint is published by the registered company Springer Nature Switzerland AG
The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Contents
Part I Understanding Valvular Heart Disease

1 Acquired Valvular Heart Disease: Overview of Patient
Population �� 3
Marian C. Hawkey and Bettina Hoejberg Kirk
2 Anatomy and Pathophysiology of Valvular Heart Disease�� 15
Elizabeth M. Perpetua and Dmitry B. Levin
Part II Valvular Heart Disease Program Structure

3 The Heart Team: A Gold Standard of Care�� 59
Sandra B. Lauck and Amanda Smith
4 Processes of Care and Evaluation Pathway for Patients
with Valvular Heart Disease�� 73
Kimberly Guibone and Jennifer Munoz
Part III Assessing Valvular Heart Disease

5 Imaging Modalities in the Diagnosis and Treatment
of Acquired Heart Valve Disease�� 89
Sarah E. Clarke
6 Measuring Function, Frailty and Quality of Life in
People with Heart Valve Disease�� 123
Astri Tafjord Frantzen, Sandra B. Lauck, and Tone M. Norekvål
7 Making a High-Quality Treatment Decision:
Shared Decision-Making �� 135
Roseanne Palmer
Part IV Valvular Heart Disease Treatment Options

8 Surgical Treatment for Patients with Valvular Heart Disease �� 151
Britt Borregaard
v
vi Contents
9 Transcatheter Treatment Options for Acquired Valvular

Heart Disease�� 167
Martina Kelly Speight
Part V Nursing Care for Patients with Valvular Heart Disease

10 Safe Recovery after Valvular Heart Surgery�� 193
Patricia Keegan and Casey Panebianco
11 Safe Recovery After Transcatheter Heart Valve Procedures �� 215
Janet Fredal Wyman
12 Transitions of Care and Long-Term Follow-Up after
Heart Valve Procedures �� 237
Nicola Straiton
Introduction
People with acquired valvular heart disease present with diverse, complex and chal-
lenging diseases and care requirements. The progressive haemodynamic impact of
valve stenosis, regurgitation or other structural issues result in significant health
challenges, including escalating symptom burden and deteriorating quality of life,
worsening cardiac function and co-morbidities, hospital admissions and mortality.
The care of the growing number of people living with valvular heart disease is one
of the most innovative areas of cardiac clinical practice and research. The recent
advancement of treatment options and technology, cardiac imaging, clinical assess-
ments and processes of care have contributed to a highly innovative environment
that continues to improve outcomes and delivery of health services to address the
burden of valvular heart disease.
Experts Needed: Opportunities for Clinical Leadership
The assessment and treatment of valvular heart disease require unique expertise to
ensure that patients achieve the best possible outcomes, especially in the setting of
this practice environment of rapidly evolving treatment options and evidence.
Nurses and allied health professionals play an essential role in the screening, assess-
ment, case management and care during valvular heart disease patients’ journey of
care from referral to discharge, and in their long-term management and episodic
transitions of care. The professional development of this group of health care pro-
viders has lagged behind these advances, and has not fully equipped the team with
the specialized knowledge to care for this unique group of patients.
A Unique Resource for a New Field of Practice
This resource aims to help close this gap. The chapters in the text offer a compre-
hensive review to guide cardiovascular nurses and allied health professionals to care
for the diverse acquired valvular heart disease population throughout the continuum
vii
viii Introduction
of their complex journeys of care. Our goal is to build on the expertise of nurses and
other specialized health care providers who exercise diverse roles at different points
of care delivery, ranging from heart valve clinics to critical and cardiac care in-
patient units, and contribute to developing the specialized area of valvular heart
disease nursing and practice. Similar to the well-established clinical practices
focused on heart failure, heart rhythm and congenital heart disease, we believe that
it is time to acknowledge valvular heart disease as an important speciality within the
discipline of cardiovascular nursing, and the practice of physician assistants and
other allied health professionals.
The text focuses on the building blocks of the specialized care of valvular heart
disease. The first section establishes foundational knowledge to understand the
patient population and the pathophysiological burden of aortic, mitral, tricuspid and
pulmonary valve diseases. The complexity of how to provide patient-centred, inno-
vative and efficient access to treatment is discussed in the section focused on valvu-
lar heart disease program structure. In this second section, discussions about the
multidisciplinary heart team approach, processes of care and the assessment path-
way provide clinicians with a road map to guide the important structural compo-
nents of program development. Next, we discuss in detail the components of the
assessment of valvular heart disease that present unique challenges for clinicians to
gain expertise in imaging modalities, the consideration of functional status, frailty
and self-reported health status, and the integration of shared decision-making to
strengthen the shift to patient-centred care. The chapters outlining surgical and
transcatheter approaches to the treatment of valvular heart disease outline a begin-
ning understanding of contemporary options for valve replacement and repair. In
the last section, we shift our attention to the nursing care expertise and competen-
cies to facilitate patients’ safe recovery after treatment and their successful transi-
tion home to derive the quantity and quality of life benefit of the treatment of
valvular heart disease.
A Team of International “Practice-Close” Leaders
In assembling our team of co-authors, we purposefully sought the collaboration of

a diverse group of contributors who hold distinct clinical and/or academic roles,
have varied experience and expertise in the field of valvular heart disease, and prac-
tice in different international regions. We are particularly proud that this text is the
result of the collective efforts of nurses and allied health professionals who provide
direct care to patients and their families, hold “practice-close” clinical, administra-
tive or teaching leadership roles, or pioneer nurse-led research that is advancing
evidence in this field. This outstanding group is internationally recognized as some
of the most innovative nursing and allied health leaders in this field and has signifi-
cantly contributed to building a community of practice, teaching and scholarship to
guide practice and champion their role in multidisciplinary practice. The reader will
note how this intentional diversity is visible in the chapters in which regional
nomenclature is left intact (e.g., transcatheter aortic valve implantation vs.
Introduction ix
replacement) to recognize the differing contexts of care that enrich the field. We are
thankful for the co-authors’ commitment to this project, the excellence of their con-
tributions, and for their trust in our leadership.
Valvular Heart Disease: A Guide for Cardiovascular Nurses and Allied Health
Professionals is the first clinical resource that provides practical and evidence-based
guidance for nurses and health care providers to strengthen their knowledge and
skillset in the specialized care of the unique group of patients living with complex
heart valve disease. We hope that the collective efforts of the contributing team will
serve to improve patient care, support professional development, and strengthen the
leadership of nurses and allied health professionals to advance this exciting and
innovative field.
Marian C. Hawkey
Hackensack University Medical Center,
Hackensack, NJ
USA
Sandra B. Lauck
St. Paul’s Hospital,
University of British Columbia,
Vancouver, BC
Canada
About the Contributors
Britt Borregaard, PhD works as a Clinical Nurse and an Associate Professor at

the University of Southern Denmark and the Department of Cardiology, Department
of Cardiothoracic and Vascular Surgery, Odense University Denmark. Britt has
clinical and research experience optimizing clinical pathways among patients
undergoing conventional valve surgery. Her research focuses on patient-reported
outcomes, frailty assessments and organizational perspectives.
Sarah E. Clarke, DNP, ACNP-BC is a Senior Principal Program Development

Consultant for Medtronic. Sarah E. Clarke is a subject matter expert in clinical
infrastructure for valve programs and supports transcatheter aortic valve replace-
ment launches and programmatic efficiencies across the care continuum. Prior to
joining Medtronic, she was the Nurse Practitioner and Structural Heart Coordinator
for Scripps Health in La Jolla, CA, USA, and brings frontline experience in the
management of structural heart disease, market and service line development, clini-
cal operations, outreach, education, and quality initiatives.
Astri Tafjord Frantzen, RN, MSc is working as a TAVI coordinator at the Section
for cardiothoracic surgery, Department of Heart Disease, Haukeland University
Hospital in Bergen, Norway. Astri Frantzen is specialized as a cardiovascular nurse
with 25 years of experience. Her field of research is frailty and patient-reported
outcomes in patients undergoing advanced heart treatment. She holds a master’s
degree in frailty in TAVI (2014) and is an active member of PROCARD (Patient-
Reported Outcomes in CARDiology) research group.
Kimberly Guibone, DNP, ACNP-BC, FACC is the Structural Heart Clinical

Program Manager at Beth Israel Deaconess Medical Center in Boston, MA, USA,
and has led the Structural Heart program there since its inception and early clinical
trials. She completed her graduate work at Georgetown University, Washington DC,
USA, and her doctoral studies in Nurse Executive Leadership at Simmons University
in Boston, MA, USA. Her areas of interest include enhancement of the advanced
practitioner role, addressing disparities in care, development of the heart team, and
clinician burnout. She currently resides in NH with her husband and two nieces.
xi
xii About the Contributors
Marian C. Hawkey, RN has an extensive nursing background in cardiac and criti-

cal care and has specialized in the field of transcatheter heart valve therapy since
2006. She is an invited speaker at national and international cardiology meetings
and has co-edited and co-authored multiple publications on topics relevant to the
care of patients undergoing transcatheter valve replacement. Marian C. Hawkey has
collaborated on the development of national training courses for nurses and allied
health professionals working in the transcatheter valve therapy field. Her areas of
interest include fostering the development of valve program clinicians, engagement
of the extended heart team, and structural heart program process improvement.
Patricia Keegan, DNP, NP-C, FACC is originally from New Jersey and now
resides in Atlanta, GA. She received her master’s in nursing from the University of
Alabama at Birmingham and her Doctorate in Nursing Practice from the University
of South Alabama. Her career started at Emory University Hospital as a bedside
nurse in Cardiology. This is where she found her love of Structural Heart Disease.
Patricia Keegan participated in the care of the first TAVR performed at Emory in
2007. She now serves as the Program Director for the Structural Heart Program for
Emory Healthcare. She has co-authored multiple publications and has been invited
to speak nationally about Structural Heart Disease. Her platforms include patient
advocacy and nursing empowerment.
Bettina Hoejberg Kirk, RN, MScN is a Clinical Nurse Specialist in valvular heart
diseases and structural heart diseases, following the patients’ pathways in the
Department of Cardiology and Cardiac Catheterization Laboratory. She has exten-
sive experience as a TAVI Valve Coordinator, working on optimizing the patient
pathways and improving patients’ experiences and recovery. Additionally, Bettina
Hoejberg Kirk focuses on the importance of clinical leadership for best practices.
Sandra B. Lauck, PhD, RN, FCAN holds the St. Paul’s Hospital and Heart &
Stroke Foundation Professorship in Cardiovascular Nursing at the University of
British Columbia (UBC), Vancouver, BC, Canada. She has a joint appointment as
clinical associate professor at the UBC School of Nursing and nurse clinician scien-
tist at the Centre for Heart Valve Innovation, St. Paul’s Hospital. Her research pro-
gram focuses on the measurement of patients’ perspectives and the development of
processes of care to support innovation in cardiovascular care.
Dmitry B. Levin, BA is the Associate Director of the Center of CardioVascular

Innovation at the University of Washington in Seattle, WA, USA. As a pioneering
research scientist and engineer, Levin is internationally recognized for advancing
medical device development, education, and training in structural and valvular heart
disease. Levin has also paved the way for the application of anatomy and physiology
bioskills laboratories, 3D printing, virtual reality, and augmented reality for trans-
catheter structural heart therapy in pre-clinical and clinical arenas. For these domains
of expertise, Levin is an invited faculty member for scientific sessions across the
globe and a widely published author for peer-reviewed medical journals in cardiology.
About the Contributors xiii
Jennifer Munoz, MSN, APN-C received her Bachelor of Science Degree in

Nursing from Seton Hall University and her Master’s Degree in Nursing/Adult Nurse
Practitioner from Rutgers University. Her nursing background has been in Cardiology,
Cardiac Surgery and now Structural and Congenital Heart. She currently is a Nurse
Practitioner/Clinical Valve Coordinator of the Structural and Congenital Heart Group
at Hackensack University Medical Center in Hackensack, New Jersey.
Tone M. Norekvål, RN, MSC, PhD, FESC, FAHA is a Professor in Cardiovascular
Nursing at the Department of Clinical Science, University of Bergen, and Haukeland
University Hospital, Bergen, Norway, where she is the chair of the Patient-Reported
Outcomes in Cardiology (PROCARD) research group. She is the founder and for-
mer director of the Centre on Patient-reported Outcomes in Bergen leading national
initiatives in Patient-reported Outcomes in the national medical quality registries.
Tone Norekval is the principal investigator of the CARDELIR study researching
delirium as primary outcome after heart valve therapy. Frailty is also a great research
interest. Norekvål is actively involved in the European Society of Cardiology and is
an associate editor of the European Journal of Cardiovascular Nursing.
Roseanne Palmer, MSN RN whose career in critical care, palliative medicine,
health care administration, and most recently structural heart and valvular heart
disease, created a foundation and passion for shared decision-making in clinical
practice. She has participated in research, published and speaks on issues related to
patient-centred goals, shared decision-making, and structural heart disease.
Throughout her career, she has been a dynamic advocate for patient voice. Roseanne
was part of the Dartmouth Hitchcock Structural Heart Leadership team responsible
for developing and implementing one of the first 25 such programs in the nation.
She continues to practice at Dartmouth Hitchcock Medical Center, Lebanon, NH.
Casey Panebianco, DNP, APN-C has been a board-certified Advanced Practice

Nurse since 2004 and obtained her Doctorate in Nursing Practice in 2012. In the begin-
ning of her career her focus was in cardiac critical care and in 2006 Casey joined the
Division of Cardiothoracic Surgery at Robert Wood Johnson University Hospital in New
Brunswick, New Jersey. Casey became the Lead APN of this division in 2014. During
this time Casey has been involved in the development and implementation of multiple
programs and safe practice protocols. Some of these include an Enhanced Recovery after
Surgery (ERAS) program, Extracorporeal Membrane Oxygenation (ECMO) program,
and TAVR fast-track programs. Casey is a proud mother of two beautiful daughters.
Elizabeth M. Perpetua, DNP, ANP-BC, ACNP-BC, FACC is a first-generation

Filipina-American and board-certified Adult and Acute Care Nurse Practitioner. She
completed her Master of Nursing and Doctor of Nursing Practice degrees at the
University of Washington. Elizabeth Perpetua is the founder of Empath Health
Services LLC and serves as a board member, advisor, consultant, and educator for
health systems, cardiology societies, patient advocacy organizations, medical and life
sciences industry, and startup companies. Perpetua is an Adjunct Professor of Nursing
at Seattle Pacific University. She has presented and published widely on cardiac
xiv About the Contributors
nursing and program development in structural and valvular heart disease. Her pas-
sions are innovations in systems of care delivery and the advancement and empower-
ment of the profession of nursing. She resides in Seattle, WA, with her two sons.
Amanda Smith, DNP is the Nurse Practitioner and Coordinator for the Aortic
Valve Program at Hamilton Health Sciences, Hamilton, ON, Canada. Amanda is
originally from North Carolina and now resides in Ontario, Canada. She received
her Bachelor of Science in Nursing from East Carolina University, her Master of
Science in Nursing from the University of California, Los Angeles, and her
Doctorate of Nursing Practice from Duke University. Amanda Smith co-leads the
Canadian TAVR Community of Practice.
Martina Kelly Speight, MSN, RN, FNP is a board-certified Nurse Practitioner in

the Structural Heart Program at Stanford Health Care in California. Martina estab-
lished her role on the Stanford multidisciplinary heart team in 2008 where she coor-
dinated research efforts and greatly contributed to program development. In her role
as Nurse Practitioner, Martina Speight has become a clinical expert in the care and
management of patients undergoing treatment for Valvular Heart Disease. She is
passionate about leading efforts that improve program outcomes, efficiencies, and
patient experiences. Martina has contributed to multiple publications and speaks
nationally about Heart Valve Disease and Structural Heart Program development.
Nicola Straiton, MSc, RN is a nurse researcher and PhD student within the Faculty
of Medicine and Health at the University of Sydney, Australia. In addition, she is a
Project Manager at the Australian Clinical Trials Alliance (ACTA), the peak body
supporting the investigator-led research sector across Australia. Her clinical and
academic career centres on understanding and supporting cardiovascular disease
patients and their families, as demonstrated in her research in which she is exploring
acceptability of contemporary heart valve surgery by patients and families, and
quality of life outcomes. She is interested in clinical trial methodology, patient
engagement in research, and digital health.
Janet Fredal Wyman, DNP, ACNS-BC, FACC is the Administrative Director for
Structural Heart Disease Clinical Services for the Henry Ford Health System;
appointed in May 2019 to oversee clinical practice and outcomes as well as pro-
grammatic growth of the Structural Heart service line. She is an original member of
the multidisciplinary heart team who established the Structural Heart Program in
2010 and has been intimately involved in its growth to a nationally recognized
leader in innovative transcatheter therapies, having performed nearly 5000 trans-
catheter structural heart and multiple first in man procedures. She joined the Heart
and Vascular Institute in 2000 as a cardiovascular nurse practitioner.
Wyman completed her Doctorate in Nursing Practice at Wayne State University
in Detroit, Michigan (2013), and her Master’s Degree in Nursing (1991). She earned
her Baccalaureate in the Science of Nursing at the University of Michigan (1978).
She is a Board-Certified Clinical Nurse Specialist with a focus on Adult Health and
Cardiovascular diseases.
Part I
Understanding Valvular Heart Disease
Acquired Valvular Heart Disease:
Overview of Patient Population 1
Marian C. Hawkey and Bettina Hoejberg Kirk
1.1 Objectives
1. Provide a broad overview of the population of patients with acquired valvular

heart disease.
2. Identify challenges associated with the care of patients with valvular heart
disease.
3. Describe key features of acquired valvular heart disease pathologies.
1.2 hallenges Associated with the Care of Patients

C
with Valvular Heart Disease
Many patients with acquired valvular heart disease (VHD) present with complex
clinical profiles associated with age, comorbid burden, frailty and disability, and/or
multi-valve disease. These complexities necessitate a comprehensive evaluation
pathway that not only considers clinical and anatomic features, but also must
accommodate for patient preferences, goals, and expectations. A collaborative mul-
tidisciplinary team (MDT) provides the foundation for making balanced treatment
M. C. Hawkey (*)
Hackensack University Medical Center, Hackensack, NJ, USA
B. H. Kirk
Rigshospitalet, Copenhagen, Denmark
e-mail: bettina.hoejberg.kirk@regionh.dk
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 3

M. C. Hawkey, S. B. Lauck (eds.), Valvular Heart Disease,
https://doi.org/10.1007/978-3-030-86233-6_1
4 M. C. Hawkey and B. H. Kirk
Stage Definition Description
A At risk Patients with risk factors for development of VHD
B Progressive Patients with progressive VHD (mild to moderate severity and

asymptomatic)
C Asymptomatic Asymptomatic patients who have the criteria for severe VHD:
severe
C1: Asymptomatic patients with severe VHD in whom the LV

or RV remains compensated
C2: Asymptomatic patients with severe VHD with

decompensation of the LV or RV
D Symptomatic Patients who have developed symptoms as a result of VHD

severe
LV indicates left ventricle; RV, right ventricle; and VHD, valvular heart disease.
Fig. 1.1 Stages of VHD. (Reprinted with permission from Otto CM, Nishimura RA, Bonow RO,
Carabello BA, Erwin JP 3rd, Gentile F, Jneid H, Krieger EV, Mack M, McLeod C, O’Gara PT,
Rigolin VH, Sundt TM 3rd, Thompson A, Toly C. 2020 ACC/AHA Guideline for the Management
of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College
of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.
Circulation. 2021 Feb 2;143 (5):e35–e71. https://doi.org/10.1161/CIR.0000000000000932. Epub
2020 Dec 17. Erratum in: Circulation. 2021 Feb 2;143 (5):e228. Erratum in: Circulation. 2021 Mar
9;143 (10):e784. PMID: 33332149)
decisions. Shared-decision making is an essential component of the overall decision-

making process for engagement of patients and their families/significant others and
for adequate expression of goals of care. The AHA/ACC Guideline for the
Management of Patients with Valvular Heart Disease [1] includes a framework for
the staging of VHD with a range from those at risk for the development of VHD,
based on comorbid conditions (Stage A), to those with severe and symptomatic
VHD (Stage D). This classification of valve disease severity is based on criteria
such as symptoms, valve anatomy, valve hemodynamics, ventricular and vascular
function (Fig. 1.1) [1].
There are established and validated scoring algorithms which assess for surgical
mortality risk such as the Society of Thoracic Surgeons (STS) Adult Cardiac Surgery
Predicted Risk of Mortality Calculator [2] (https://riskcalc.sts.org/stswebriskcalc/
calculate) and the European System for Cardiac Operative Risk Evaluation
(EuroSCORE) [3] (http://www.euroscore.org/calc.html). However, these scores do
not fully consider many factors which can contribute to procedural mortality risk.
The impact of high comorbid burden, frailty, and disability must be factored into the
treatment plan and the associated influence on goals of care. The treatment
1 Acquired Valvular Heart Disease: Overview of Patient Population 5
Low-Risk SAVR Low-Risk Surgical Mitral Valve

(Must Meet Repair for Primary MR High Surgical Risk Prohibitive Surgical Risk
ALL Criteria (Must Meet ALL (Any 1 Criterion (Any 1 Criterion
Criteria in This Column) Criteria in This Column) in This Column) in This Column)
STS-predicted risk of death* <3% <1% >8% Predicted risk of death or major
AND AND OR morbidity (all-cause) >50% at 1 y
OR
Frailty† None None ≥2 Indices (moderate ≥2 Indices (moderate to severe)
AND AND to severe) OR
OR
Cardiac or other major organ system None None 1 to 2 Organ systems ≥3 Organ systems
compromise not to be improved AND AND OR OR
postoperatively‡
Procedure-specific impediment§ None None Possible procedure- Severe procedure-specific
specific impediment impediment
*Use of the STS Predicted Risk of Mortality (http://riskcalc.sts.org/stswebriskcalc/#/) to predict risk in a given institution with reasonable reliability is appropriate only if institutional outcomes
are within 1 standard deviation of the STS average observed/expected mortality ratio for the procedure in question. The EUROSCORE II risk calculator may also be considered
for use and is available at http://www.euroscore.org/calc.html.
*Seven frailty indices: Katz Activities of Daily Living (independence in feeding, bathing, dressing, transferring, toileting, and urinary continence) plus independence in ambulation (no walking
aid or assistance required, or completion of a 5-m walk in <6 s). Other scoring systems can be applied to calculate no, mild, or moderate to severe frailty.
‡Examples of major organ system compromise include cardiac dysfunction (severe LV systolic or diastolic dysfunction or RV dysunction, fixed pulmonary hypertension); kidney dysfunction
(chronic kidney disease, stage 3 or worse); pulmonary dysfunction (FEV1 <50% or DLCO2 <50% of predicted); central nervous system dysfuction (dementia, Alzheimer’s disease, Parkinson’s
disease, cerebrovascular accident with persistent physical limitation); gastrointestinal dysfunction (Crohn’s disease, ulcerative colitis, nutritional impairment, or serum albumin <3.0); cancer
(active malignancy); and liver dysfunction (any history of cirrhosis, variceal bleeding, or elevated INR in the absence of VKA therapy).
§Examples of procedure-specific impediments include presence of tracheostomy, heavily calcified (porcelain) ascending aorta, chest malformation, arterial coronary graft adherent to
posterior chest wall, and radiation damage.
DLCO2 indicates diffusion capacity for carbon dioxide; FEV1, forced expiratory volume 1 s: INR, international normalized ratio; LV let ventricular; MR, mitral regurgitation; RV, right
ventricular; SAVR, surgical aortic valve replacement; STS, Society of Thoracic Surgeons; and VKA, vitamin K antagonist.
Fig. 1.2 Risk assessment for surgical valve procedures. (Reprinted with permission of Otto CM,
Nishimura RA, Bonow RO, Carabello BA, Erwin JP 3rd, Gentile F, Jneid H, Krieger EV, Mack M,
McLeod C, O’Gara PT, Rigolin VH, Sundt TM 3rd, Thompson A, Toly C. 2020 ACC/AHA
Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A
Report of the American College of Cardiology/American Heart Association Joint Committee on
Clinical Practice Guidelines. Circulation. 2021 Feb 2;143 (5):e35–e71. https://doi.org/10.1161/
CIR.0000000000000932. Epub 2020 Dec 17. Erratum in: Circulation. 2021 Feb 2;143 (5):e228.
Erratum in: Circulation. 2021 Mar 9;143 (10):e784. PMID: 33332149)
pathways for patients include surgical intervention, transcatheter intervention, and

medical/palliative therapy. In some cases, particularly when valvular heart disease
severity has not yet met criteria for definitive therapy, patients will remain under
surveillance with periodic clinical evaluations, assessment of symptoms and echo-
cardiograms. While an intervention may be technically feasible, treatment plans
must consider both short-term risks and long-term benefits. Determination of goals
of care and balanced treatment decisions are best served by the foundation of a
comprehensive evaluation pathway and a collaborative MDT (Figs. 1.2 and 1.3).
1.3 Acquired Valvular Heart Disease Pathologies
1.3.1 Aortic Stenosis
Aortic stenosis (AS) is the most frequent VHD in the western world [4, 5]. AS is an
active disease process characterized by thickening and impaired movement of the
valve leaflets, potential development of left ventricular (LV) dysfunction, and hemo-
dynamics changes, which in combination lead to clinical symptoms [6]. AS restricts
the blood flow from the left ventricle to the aorta and may also cause backward
failure with increased filling pressures on the left side of the heart.
Surgical Mitral Valve Repair

SAVR TAVI or Replacement TEER
Technical or anatomic
Prior mediastinal radiation Aorto-iliac occlusive Prior sternotomy Multivalve disease

Ascending aortic calcification disease precluding Prior mediastinal radiation Valve morphology (eg, thickening,
(porcelain aorta may be transgemoral approach Ascending aortic calcification perforations, clefts, calcification,
prohibitive) Aortic arch atheroscle- (porcelain aorta may be and stenosis)
rosis (protuberant prohibitive) Prior mitral valve surgery
lesions)
Severe MR or TR
Low-lying coronary
arteries
Basal septal
hypertrophy
Valve morphology
(eg, bicuspid or uni-
cuspid valve)
Extensive LV outflow
tract calcification
Comorbidities
Severe COPD or home oxgyen Severe COPD or home Severe COPD or home oxgyen Severe COPD or home oxgyen
therapy oxgyen therapy therapy therapy
Pulmonary hypertension Pulmonary hypertension Pulmonary hypertension Pulmonary hypertension
Severe RV dysfunction Severe RV dysfunction Severe RV dysfunction Severe RV dysfunction
Hepatic dysfunction Hepatic dysfunction Hepatic dysfunction Hepatic dysfunction
Frailty* Frailty* Frailty* Frailty*
Futility
STS score >15 STS score >15 STS score >15 STS score >15
Life expectancy <1 y Life expectancy <1 y Life expectancy <1 y Life expectancy <1 y
Poor candidate for Poor candidate for Poor candidate for Poor candidate for rehabilitation
rehabilitation rehabilitation rehabilitation
*Validated frailty scores include the Katz Activities of Daily Living Score (10,34,35).
COPD indicates chronic obstructive pulmonary disease; MR, mitral regurfitation; RV, right ventricular; surgical aortic valve replacement; STS, Society of Thoracic Surgeons;
TAVI, transcatheter aortic valve implantation; TEER, transcatheter edge-to-edge repair; and TR, tricuspid regurgitation.
Fig. 1.3 Examples of procedure-specific risk factors for interventions not incorporated into exist-
ing risk scores. (Reprinted with permission of Otto CM, Nishimura RA, Bonow RO, Carabello BA,
Erwin JP 3rd, Gentile F, Jneid H, Krieger EV, Mack M, McLeod C, O’Gara PT, Rigolin VH, Sundt
TM 3rd, Thompson A, Toly C. 2020 ACC/AHA Guideline for the Management of Patients With
Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/
American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021
Feb 2;143 (5):e35–e71. https://doi.org/10.1161/CIR.0000000000000932. Epub 2020 Dec 17.
Erratum in: Circulation. 2021 Feb 2;143 (5):e228. Erratum in: Circulation. 2021 Mar 9;143
(10):e784. PMID: 33332149)
There is usually a prolonged asymptomatic phase followed by the development

of symptoms as narrowing of the valve becomes more severe. The onset of severe
symptoms such as angina, syncope and heart failure represent a critical juncture in
the disease’s course with the need for timely evaluation for appropriate therapeutic
options. Although many patients may present with profound symptoms, it is not
uncommon for symptoms to be more subtle such as fatigue or a gradual decrease in
exercise tolerance. Patients may dismiss these symptoms as a normal function of
aging. The documentation of a detailed history is critical for a complete assessment
of the impact of aortic stenosis. Given the progressive nature of aortic stenosis,
patients are commonly followed over time with serial physical exams and
echocardiograms.
1.3.1.1 Age and Gender

AS is common in the elderly population, with higher prevalence among men [7, 8].
Among people older than 65 years, studies estimate that between 1 and 3% have
moderate to severe AS and among those over 75 years of age, the corresponding
number is up to 12% [7]. The rate is expected to increase worldwide as the popula-
tion ages [1]. However, better cardiovascular health with modification of risk factors
is thought to be associated with a lower rate of development of calcific valve disease
in the future [5, 9].
1.3.1.2 Etiology and Risk Factors

AS is the result of calcific aortic valve disease (CAVD), that begins with aortic scle-
rosis, which is asymptomatic, to the development of symptoms when the stenosis
becomes severe. The risk factors for AS include a congenital bicuspid aortic valve
and older age. Hypertension has been associated with CAVD and is a strong risk
factor for AS in several cohort studies [7]. Cigarette smoking has also been associ-
ated with CAVD in several studies [8].
Dyslipidemia has been linked with AS in observational studies but testing of
lipid-lowering therapies in preventing disease progression does not affect the pro-
gression of AS [10]. Severe valve calcification and AS have been demonstrated
among individuals with familial hypercholesterolemia [7]. Advanced chronic kid-
ney disease (CKD) has been associated with rapid and progressive calcification of
the cardiovascular system, including the valve structures, with a less consistent link
when CKD is mild [7, 8]. The process of valve calcification is thought to share the
same regulating factors and signaling pathways as seen in osteoporosis and bone
metabolism with the process of calcium deposition [8].
The joint effect of having one or more of the risk factors has only been investi-
gated in a few studies, but the number of risk factors has been demonstrated to be
strongly associated with the presence of AS [7].
1.3.1.3 Comorbid Conditions

Hypertension (HTN) frequently accompanies aortic stenosis [8]. Both HTN and low
systolic and pulse pressures have been associated with higher rates of mortality [8].
Although statin therapy is not indicated for progression of AS, patients may benefit
from medical therapy in cardiovascular risk prevention [5]. Approximately half of
patients with AS have coexisting coronary artery disease, for which coronary angi-
ography is frequently recommended during the evaluation for an aortic valve
replacement [7]. Atrial fibrillation (AF) may occur due to the progression of aortic
stenosis [7, 8]. Anticoagulation management of AF with AS continues to evolve.
Multi-society international guidelines have recommendations for oral anticoagu-
lants [1, 4]. Mitral regurgitation (MR) frequently coexists with severe AS [11]. The
optimal management of these patients is still unclear. Careful clinical evaluation
and surveillance are recommended to identify the patients who might benefit from
a double valve intervention, or if MR should be left untreated [11].
1.3.2 Aortic Regurgitation
Aortic regurgitation (AR) is characterized by regurgitation of blood from the aorta

and into the left ventricle due to impaired aortic valve function [12]. Overall, AR is
caused by abnormalities of the aortic valve leaflets, the aortic root or the ascending
aorta [13]. AR may occur as an acute or a chronic valvular disease, and different
management strategies are required in clinical practice.

The prevalence of AR increases with advancing age, appearing in individuals at
40–60 years [12]. Moderate or severe aortic regurgitation was found in 2% in a US
population of 75 years of age or older [13]. AR has a higher prevalence in men and
has been observed in 13% of men and 8.5% of women in a population-based cohort
study [14].

Acute AR is most often caused by infective endocarditis (the valve leaflets may be
damaged or interference with aortic cusp coaptation by a valvular vegetation), aortic
dissection or a traumatic rupture [12]. The expanding adoption of transcatheter aor-
tic valve replacement (TAVR) and balloon aortic valvuloplasty (BAV) has revealed
more clinical AR cases as the techniques can cause both acute and chronic AR [15].
Chronic AR is often caused by calcific degeneration of the aortic valve, associated
with aging and BAV [13]. Systemic hypertension and hyperlipidemia are also asso-
ciated with chronic AR [12].
A congenital bicuspid aortic valve may result in an inadequate coaptation of the
aortic valve, and this is a common cause for chronic AR [13]. Rheumatic disease
can cause chronic AR through infiltration of the aortic cusps with fibrous tissues
[15]. AR also occurs in conjunction with diseases of the aorta without direct involve-
ment of the aortic valve, such as degenerative aneurysms, aortic dissection or
Marfan syndrome causing secondary AR via annulus dilatation [13].

Hypertension is often present in patients with chronic asymptomatic AR and treat-
ment is recommended to prevent AR progression [1]. AR is present in up to 75% of
older patients with AS, although usually in a mild disease stage [13]. Prevention of
disease progression requires medical monitoring, focusing on symptom develop-
ment and LV dysfunction [12].
1.3.3 Mitral Regurgitation
Mitral regurgitation may be chronic or acute. Primary, or degenerative, mitral regur-

gitation involves the leaflets and chordae tendineae. Secondary, or functional, mitral
regurgitation is caused by changes to left ventricular function or geometry.
Mitral regurgitation (MR) is characterized by a retrograde flow of blood from the

left ventricle, into the left atrium through the dysfunctional mitral valve. It is essen-
tial to make a distinction between primary (degenerative) and secondary (func-
tional) MR as this will dictate the treatment strategy [16].

MR is the second most common valvular abnormality worldwide, affecting more
than 2% of the total population, and the prevalence increases with age [17]. There
are no significant gender difference in prevalence of MR [14], however, there are
more men than women with severe MR who undergo mitral valve surgery/interven-
tion [1].

Primary MR may be caused by degenerative disease of the leaflets or chords, most
often with mitral valve prolapse (MVP) [16]. In primary MVP, advancing age is the
driving factor causing the disease progression. Infective endocarditis (IE) and rheu-
matic heart disease may involve the mitral valve and cause regurgitation [16].
Secondary MR may be caused by ischemic or nonischemic conditions and is
associated with left ventricular dilatation and systolic dysfunction [18]. Congestive
heart failure (CHF) is associated with the stages of mild, moderate and severe MR
[18]. AF is found to cause increased size of the valve annulus, resulting in functional
MR [17]. Hypertrophic cardiomyopathy (HCM), defined as severe left ventricular
hypertrophy, can also lead to MR [16].
MR can also be categorized as an acute or chronic condition [1]. Disruption of
different parts of the mitral valve apparatus, such as spontaneous chordal rupture,
leaflet perforation associated with IE, or papillary muscle rupture in the setting of
an acute ST segment elevation myocardial infarction, may cause acute MR and
require urgent intervention [16].
It is important to distinguish between the chronic primary (degenerative) and the
chronic secondary (functional) MR. Considerations must be given to both patients’
symptoms, LV size and function, pulmonary pressures and MR severity [18].

Patients with chronic primary MR are often elderly and frail, have multiple comor-
bidities and are at high surgical risk [1]. The decision to recommend medical man-
agement versus surgical intervention of MR depends on severity, chronicity,
comorbidities, and etiology [17].
1.3.4 Mitral Stenosis
Mitral stenosis (MS) causes hemodynamic obstruction of the mitral valve inflow
[19]. This obstruction is caused by a modification in thickness, shape and mobility
of the mitral leaflets. Worldwide, MS is mainly associated with rheumatic heart
disease; the incidence has decreased significantly in the past decades in industrial-
ized countries in parallel with better accessibility of medical services and availabil-
ity of antibiotics [19]. The incidence of non-rheumatic calcific MS is increasing in
the elderly population in high-income countries [1].
Mild exertional dyspnea is the most common initial symptom. As the degree
of stenosis increases, dyspnea, paroxysmal nocturnal dyspnea and fatigue may
be present. Severe mitral stenosis is associated with symptoms of heart failure
with mild exertion and evidence of right-sided heart failure with advanced
disease.

The clinical presentation of MS varies. In regions with low disease prevalence, MS
occurs often in older patients, presenting decades after an episode of rheumatic
fever (age 50–70 years) [20]. In regions with high disease prevalence, rheumatic
MS presents at a younger age (from teen years to 30 years) [1]. The prevalence of
mitral annular calcification is proportional to age and is greater than 25% after
75 years of age. Rheumatic MS occurs more frequently in women who account for
about 80% of cases, than in men. Women are also more frequently affected by non-
rheumatic MS [19].

Mitral stenosis most commonly occurs as a sequela of rheumatic fever. Degenerative
mitral stenosis may also be related to atherosclerotic heart disease, advanced age,
and uncontrolled hypertension.
MS is mainly caused by chronic rheumatic valvular disease and by calcific
degeneration. A small proportion is caused by previous oncological therapies [1].
Calcific MS is a consequence of mitral annular calcification (MAC) and degenera-
tion, involving the leaflets [20]. MAC is associated with AF and other arrhythmias,
stroke and mortality [20]. Chronic kidney disease impacts the progression of calci-
fication significantly [19]. Diabetes is likewise associated with mitral annular
degeneration [1].

Older patients with MS are often frail and have multiple comorbidities. Treatment
strategies should consider appropriate risk stratification [1] and likelihood to
benefit.
1.3.5 Tricuspid Regurgitation
Tricuspid regurgitation (TR) is the most common disorder of the tricuspid valve
[21]. The majority of TR is functional and secondary to other disease processes, and
not related to primary tricuspid leaflet pathology [21]. Mild TR is usually benign,
whereas moderate or severe TR may lead to irreversible myocardial damage and
potentially adverse outcomes [22].

Age and gender influence progression from mild to more significant degrees of
TR. In men and women aged 70 years and older, the prevalence of moderate to
severe TR is 1.5% and 5.6% respectively [14]. The Framingham Heart Study
revealed the presence of trace to more than moderate TR to be 82% in men and
85.7% in women [14].

TR occurs frequently as a consequence of tricuspid annular dilatation and right
ventricular remodeling due to high right-sided chronic pressure and/or volume over-
load [23]. Chronic severe TR can lead to right-sided heart failure and potentially to
low cardiac output [21].
Functional TR is related to pulmonary venous hypertension and caused by a
significant left-sided heart disease, pulmonary hypertension or pulmonary artery
hypertension [21]. Tricuspid annular dilatation is also caused by chronic AF, myo-
cardial infarction or dilated cardiomyopathy [22].
Primary tricuspid disease may be caused by congenital heart disease (e.g.,
Ebstein anomaly), rheumatic or carcinoid disease, IE, trauma or tricuspid valve pro-
lapse [22]. Leads from a permanent pacemaker or internal cardiac defibrillator may
cause valve injury in the tricuspid apparatus.
At the time of a left-sided valve surgery, if left uncorrected, a mild or moderate
degree of secondary TR may progress over time and result in reduced long-term
functional outcome and survival in approximately 25% of patients [1].

Tricuspid valve interventions may be required in symptomatic patients and have
increased during the last decade. Isolated surgical replacement of functional TR has
been associated with high operative morbidity and mortality for both genders [23].
Improved risk stratification and timely intervention is required [22]. Transcatheter
options for the minimally invasive management of TR are rapidly evolving to facili-
tate both valve repair and replacement.
In summary, the complex clinical presentation of many patients with acquired
valvular heart disease requires the attention of an astute and collaborative multidis-
ciplinary team, a comprehensive evaluation pathway, and the incorporation of
shared decision-making processes to support patient and family education, expecta-
tions and goals of care. These components provide the foundation for balanced
treatment decisions and optimal outcomes.
1.4 Key Takeaways
• A comprehensive and multidisciplinary approach to the individual patient trajec-

tory facilitates balanced treatment decisions and positive outcomes.
• Patients with acquired VHD present with complex clinical profiles associated
with age, comorbid burden, frailty and disability, and/or multi-valve disease.
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Anatomy and Pathophysiology
of Valvular Heart Disease 2
Elizabeth M. Perpetua and Dmitry B. Levin
2.1 Objectives
1. Discuss the standards of practice for nursing, advanced practice providers, and
allied health professionals as they pertain to valvular anatomy and
pathophysiology.
2. Understand the various etiologies of valvular heart disease and their pathoana-
tomical consequences.
3. Conceptualize how the pathoanatomy of valvular heart disease results in patho-
physiologic consequences.
4. Review the progression of valvular heart disease, the pathophysiologic effects on
organ systems, and the dynamic interaction with related cardiovascular condi-
tions and comorbidities.
5. Recognize physical and hemodynamic assessment findings in patients with val-
vular heart disease before, during, after, and without intervention.
E. M. Perpetua (*)
Empath Health Services, Seattle, WA, USA
School of Health Sciences, School of Nursing, Seattle Pacific University, Seattle, WA, USA
e-mail: perpetuae@spu.edu
D. B. Levin
Division of Cardiology, Department of Medicine, University of Washington,
Seattle, WA, USA
e-mail: dlevin@uw.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 15

M. C. Hawkey, S. B. Lauck (eds.), Valvular Heart Disease,
https://doi.org/10.1007/978-3-030-86233-6_2
16 E. M. Perpetua and D. B. Levin
2.2 Background
Valvular heart disease (VHD) is defined as a pathologic condition of any of the four
heart valves disrupting of the unidirectional flow of blood. Various disease states
cause pathoanatomic changes and pathophysiologic dysfunction of these critical
structures. These changes disrupt the unidirectional flow of blood during the cardiac
cycle. In valve stenosis, the valve leaflets do not open normally, limiting forward
blood flow. With valve insufficiency or regurgitation, the valve leaflets do not close
normally and blood may flow backward instead of forward. There may be mixed
VHD, in which there is both stenosis and insufficiency of the heart valve. Dysfunction
of more than one heart valve may also occur, known as concomitant VHD. As VHD
progresses, pathoanatomical changes of the affected valve(s) and other cardiac
structures thereby result in abnormal valve hemodynamics, cardiovascular hemody-
namics, clinical manifestations, and physical assessment findings. In severe VHD,
there are hemodynamic consequences that ultimately result in symptoms.
The prevalence of VHD is growing. In the general adult population is approxi-
mately 2.5% in the U.S. [1] The global prevalence of VHD is much higher, largely
owing to the burden of rheumatic heart disease in developing countries although its
rate is decreasing overall [2]. After age 65, the prevalence of VHD increases to
about 1 in 10 patients [1] due to degenerative changes to valve anatomy associated
with aging. The rapid growth of an aging population, estimated to increase from
962.3 to 2080.5 million worldwide between 2017 and 2050, positions VHD as a
public health problem. Most if not all healthcare workers across settings and spe-
cialties will encounter a patient with VHD [2].
The care of patients with VHD requires knowledge, skills, and standards of prac-
tice founded on deep understanding of normal anatomy, physiology, and patho-
physiology [3, 4] (Table 2.1). Nurses and allied health professionals must know
Table 2.1 Foundation of knowledge, skills, and practice standards for nurses and allied health
professionals
Knowledge
Knows the indications for imaging in valvular heart disease
Knows the indications for referral of patients with advanced or complex valvular heart
disease to a multidisciplinary heart team
Knows the indications for catheter and surgical based interventions for valvular heart
disease
Knows the differences in etiology, evaluation, and management of primary versus secondary
mitral regurgitation
Knows the pathophysiology and management for bicuspid aortic valve disease and
associated aortopathies
Knows the advantages and disadvantages of transcatheter and surgical options for patients
with valvular heart disease, including replacement with mechanical or biological prostheses
and valve repair
Knows prosthetic valve complications
Knows the recommendations for antithrombotic therapy for patients with valvular heart
disease and prosthetic heart valves
2 Anatomy and Pathophysiology of Valvular Heart Disease 17
Table 2.1 (continued)

Skills
Skill to assess and collaboratively manage patients with valvular heart disease
Skill to understand and as appropriate refine the therapeutic plan of care for patients with
valvular heart disease, based on goals of care, laboratory tests, and diagnostic test results
Skill to manage patients with valvular heart disease with complex comorbid conditions such
as atrial fibrillation, pulmonary diabetes mellitus, and renal disease
Skill to assist in surgical or percutaneous interventions
Skill to manage perioperative patients following surgical and percutaneous valvular heart
disease procedures
Skill to recognize and as appropriate manage cardiac arrhythmias and perioperative
complications in patients with valvular heart disease and after valvular heart procedures
Skill to recognize and as appropriate manage complications of prosthetic heart valves
Standards of practice
Collects comprehensive data pertinent to the patient’s health or the situation
Analyzes the assessment data to determine the diagnoses or the issues
Identifies expected outcomes for a plan individualized to the patient or the situation
Develops a plan that prescribes strategies and alternatives to attain expected outcomes
Implements the identified plan
Coordinates care delivery
Employs strategies to promote health and a safe environment
The advanced practice clinician may provide consultation to influence the identified plan,
enhance the abilities of others, and effect change
The advanced practice clinician may use prescriptive authority, procedures, referrals,
treatments, and therapies based on education, certification, credentialing, and scope of
practice in accordance with state and federal laws and regulations
Evaluates progress toward attainment of outcomes
Practices ethically
Attains knowledge and competence that reflects current practice
Integrates evidence and research findings into practice
Contributes to quality practice
Communicates effectively in a variety of formats in all areas of practice
Demonstrates leadership in the practice setting and the profession
Collaborates with patient, family, and others in the conduct of professional practice
Evaluates her or his own nursing practice in relation to professional practice standards and
guidelines, relevant statutes, rules and regulations
Utilizes appropriate resources to plan and provide services that are safe, effective, and
financially responsible
Practices in an environmentally safe and healthy manner
etiologies, disease progression, and organ system consequences of VHD [3, 4]. The
pathophysiologic implications of VHD may then be more readily conceptualized
and applied for therapeutic management, definitive intervention, and programmatic
approaches to care delivery across the continuum and the patient lifespan. Advanced
competencies may include navigating and influencing the arenas of healthcare qual-
ity and research, health economics, complex health systems, and health policy of
VHD. To promote learning, this chapter is structured in three parts: (1) normal valve
anatomy and physiology; (2) overarching etiologies and pathogenesis of valvular

heart disease; (3) the specific etiology, pathoanatomy (mechanisms), clinical mani-
festations and presentation associated with stenosis, insufficiency/regurgitation, and
mixed and/or concomitant valve disease. As groundwork for subsequent chapters on
evaluation, diagnostic imaging, and intervention, a vital array anatomic/pathoana-
tomic specimens is featured to enhance the multidimensional understanding of
these complex structures.
2.3 Anatomy and Physiology
To grasp what is abnormal or pathologic, there must first be comprehension of struc-

turally normal heart valves (Fig. 2.1) and their function in the cardiac cycle (Fig. 2.2)
[5]. At the histological level, heart valves are comprised of a highly organized extra-
cellular matrix (ECM) and valve interstitial cells (VIC) surrounded by an endothe-
lial cell layer. The ECM of the valves is stratified into elastin, proteoglycan, and
collagen rich layers, each of which has distinct biomechanical characteristics to the
leaflets and the valve complex. Signaling pathways perform vital functions in val-
vulogenesis and in the lifespan maintenance of valve structure and function [6].
At the macro-level, the valves are attached to the fibrous skeleton [7]. There are
unique areas of fibrous continuity. The aortic valve and pulmonic valve are known
as semilunar valves. The semilunar valves separate the ventricles from the great
arteries and allow blood to flow to the systemic and pulmonary circulation respec-
tively. The mitral valve and tricuspid valve, or atrioventricular valves, separate the
atria from the ventricles and allow blood to flow from the atria to the ventricles. This
section details the normal anatomy and physiology of the four heart valves.
Pulmonic
a Great
cardiac vein
Left
coronary
Anterior
valve
artery Aortic valve
Left
ventricle
Right
b
Anterior
coronary
artery
Pulmonic
Circumflex
Left coronary artery valve
artery
Left ventricle
Aortic valve
Circumflex artery Right coronary
artery
Mitral valve Right marginal

artery
Great cardiac Tricuspid valve
vein
Coronary sinus
Posterior
Mitral
valve
Tricuspid
valve
Coronary
sinus
Posterior
Fig. 2.1 Cardiac valves. Seen in the same plane through a transverse section with the atria
removed. (a) “Teapot” view of the heart. (b) Schematic representation of the heart valves from
superior view. Note that in panel B the non-coronary cusp of the aortic valve is referred to as (P),
posterior. A anterior, P posterior, S septal, L left. (Used with permission from Center for
CardioVascular Innovation, University of Washington)
Fig. 2.2 Cardiac cycle. (Reprinted with permission from Flamm KL, Granger M, Gawlik K, et al.
Evidence-Based Assessment of the Heart and Circulatory System. In: Gawlik KS, Melnyk BM,
Teall AM, eds. Springer Publishing Company; 2016:103–160)
2.3.1 Normal Anatomy
2.3.1.1 Semilunar Valves

The semilunar pulmonary and aortic valves are each comprised of three cup-shaped
cusps of approximately equal size that attach at their base to the fibrous skeleton.
The valve cusps are convex from below, with thickened nodules at the center of the
free margins. The cusps are comprised of fibrous connective tissue lined with endo-
thelium. The endothelial lining on the nonventricular side of the valves closely
resembles and merges with that of the intima of the arteries beyond the valves. The
semilunar valve cusps are thicker than the atrioventricular valve cusps.
The aortic and pulmonic semilunar valves are nearly perpendicular to each other
in the closed position. The pulmonic valve is anterior and superior to the other three
cardiac valves. When closed, the semilunar valve cusps contact each other at the
nodules and along the crescent arcs (lunulae), below the free margins. During sys-
tole, the cusps are thrust upward as blood flows from an area of greater pressure in
the ventricle to an area of lesser pressure in the aorta or the pulmonary artery.
Deceleration of blood in the aorta during late systole on small circular currents of
blood in the sinuses of Valsalva helps passively to close the semilunar valve cusps.
Backflow into the ventricles during diastole is prevented because of the cusps’
fibrous strength, their close approximation, and their shape.
Pulmonary Valve
The pulmonary valve (Figs. 2.3 and 2.4) is approximately 8.5 cm in circumference.
The pulmonic valve cusps are termed right anterior (right), left anterior (anterior),
and posterior (left). As mentioned, the pulmonic valve is typically considered part of
the right ventricular outflow tract. Dysfunction of this valve is usually due to congeni-
tal heart disease thus pulmonic valve disease is minimally discussed in this chapter.
Aortic Valve
The aortic valve (Fig. 2.5) is approximately 7.5 cm in circumference. The valve is
histologically formed by three layers. On the ventricular side of the leaflet is the first
a b
Fig. 2.3 Pulmonary valve. (a) Superior view of the pulmonary and aortic valves. (b) Split view of
the pulmonary valve. Right ventricular has been dissected open and pulmonary valve cut between
right and anterior leaflets. LV left ventricle, RV right ventricle, RA right atrium, LA left atrium.
(Used with permission from Center for CardioVascular Innovation, University of Washington)
ventricularis, comprised of elastin-rich fibers arranged radially and perpendicular to

the leaflet margin. On the aortic side of the leaflet is the fibrosa, comprised of fibro-
blasts and collagen fibers organized circumferentially and parallel to the leaflet mar-
gin. The third layer is a loose, connective tissue layer at the base of the leaflet known
Fig. 2.4 Pulmonary valve. Specimen demonstrates three cusps of the pulmonic valve – right (R),
left (L) and anterior (A). In addition it shows location of right ventricular outflow track (RVOT)
which has space between pulmonic and tricuspid valves (TV). Unlike left side of the heart where
left ventricular outflow track (LVOT) is comprised of septum and part of mitral valve (MV)
Fig. 2.5 Aortic valve. Normal aortic valve (AV). Healthy aortic valve leaflets are thin and rela-
tively see-through. (a) View looking down at the valve in transverse or short axis, from above
(aortic) view. Three cusps are represented as R—right coronary cusp, L—left coronary cusp and
N—non-coronary cusp. (b) View from above - aortic (AO). (c) View from below – left ventricular
(LV). (Used with permission from Center for CardioVascular Innovation, University of Washington)
as the spongiosa. This layer, seated between the fibrosa and ventricularis, is com-
prised of fibroblasts, mesenchymal cells, and a mucopolysaccharide matrix.
Together these three layers offer the tensile, pliable strength necessary to support
decades of normal AV function [8].
The sinuses of Valsalva are pouch-like structures immediately behind each semi-
lunar cusp. The coronary arteries branch from the aorta from two of the pouches or
sinuses of Valsalva. The aortic cusps are designated by the name of the associated
coronary ostia: right coronary (right or anterior) aortic cusp, left coronary (left or
left posterior) aortic cusp, and non-coronary (posterior or right posterior) aortic
cusp. At the proximal aspect of the left coronary cusp and right coronary cusps are
the coronary artery ostia which give rise to the left main coronary artery and right
coronary artery respectively. The aortic cusps are thicker than the pulmonic cusps.
The sinotubular junction is the area of confluence of the sinuses of Valsalva and the
ascending aorta.
2.3.1.2 Atrioventricular Valves

The atrioventricular tricuspid and bicuspid (mitral) valve complexes are comprised
of six components that function as a unit: the atria, the valve rings, or annuli fibrosi
of the fibrous skeleton, the valve cusps or leaflets, the chordae tendineae, the papil-
lary muscles, and the ventricular walls. The mitral and tricuspid valve cusps are
comprised of fibrous connective tissue covered by endothelium. The valve cusps
attach to the fibrous skeleton valve rings. Fibrous cords called chordae tendineae
connect the free valve margins and ventricular surfaces of the valve cusps to papil-
lary muscles and ventricular walls. Each papillary muscle or muscle group controls
the adjacent sides of two cusps, resisting valve prolapse during systole. The papil-
lary muscles are bundles of trabeculae carneae oriented parallel to the ventricular
walls, extending from the walls to the chordae tendineae. The chordae tendineae
provide many cross-connections from one papillary muscle to the valve cusps or
from trabeculae carneae in the ventricular wall directly to valves.
Tricuspid Valve
The tricuspid valve (Fig. 2.6) is named for its three cusps or leaflets. In the adult, the
circumference of the tricuspid orifice is approximately 11 cm, or capable of admit-
ting three fingers. The tricuspid valve annulus is a non-planar shape. The cusps
resemble curtain-like, billowing flaps. The combined surface area of the valve cusps
is larger than the surface area of the valvular orifice. There are commonly three tri-
cuspid valve cusps: the large anterior, the septal, and the posterior (inferior) tricus-
pid valve leaflets. There are usually two principal right ventricular papillary muscles,
the anterior and the posterior (inferior), and a smaller set of septal (accessory) papil-
lary muscles attached to the ventricular septum.
Mitral Valve
The bicuspid valve is named for a bishop’s hat, or miter; hence the structure is
called the “mitral” valve (Figs. 2.7 and 2.8). The circumference of the mitral
orifice is approximately 9 cm, or capable of admitting two fingers. The mitral
Fig. 2.6 Tricuspid valve. Images demonstrate the architecture of TV. Left side images shows view
of TV from a right ventricular (RV) dissection. Right image shows TV from a superior (Right
atrial—RA) view. Tricuspid valve is consistent of three leaflets – septal (S), posterior (P) and ante-
rior (A) all of different sizes. Similar to mitral valve (MV), TV leaflets are connected via chords to
papillary muscles in RV. SVC – superior vena cave, IVC – inferior vena cava, CS – coronary sinus.
(Used with permission from Center for CardioVascular Innovation, University of Washington)
Fig. 2.7 Mitral valve,

posterior view. Mitral
valve (MV) View from
posterior looking at an
anterior mitral valve leaflet
(AML). Leaflet is
connected to posteromedial
(PMPM) and antero-lateral
papillary (ALPM) muscles
via chordae tendineae. LA
left atrium, LV left
ventricle. (Used with
permission from Center for
CardioVascular Innovation,
University of Washington)
a Noncoronary
sinus
Aortomitral
curtain b Anterior Left atrium
leaflet
Atriovalvular
junction
Left coronary Posteromedial Aortomitral Annulus
sinus trigone curtain
Anterolateral Bundle Aorta Atrioventricular
trigone of His junction
Circumflex
Posterior
Left leaflet
ventricular
outflow Left
tract ventricle
Septum
Papillary
muslces
Right ventricle
Coronary
sinus
Left atrium
c
Posteromedial
commissure
A3
Anterolateral A1
commissure A2
P1 P3
P2
Fig. 2.8 Schematic representation of the mitral valve. (a) En face or surgeon’s view of the mitral
valve from the left atrium. (b) Left ventricular outflow tract (LVOT) view including the left atrium,
left ventricle and the LVOT. (c) En face or surgeon’s view of the mitral valve; leaflet segments are
labeled using Carpentier’s classification
valve annulus is a non-planar saddle shape, which decreases the strain on the
posterior leaflet during systolic valve closure. From the atrial view when the
valve is closed, the line where the leaflets meet looks like a smile. The areas
where the two leaflets (commissures) insert into the annulus are named the
anterolateral commissure and posteromedial commissure. The smaller, less
mobile posterior cusp is situated posterolaterally, behind, and to the left of the
aortic opening. The posterior mitral leaflet has three indentations or scallops,
which care thusly named in succession from the lateral to the medial commissure
using Carpentier’s classification: P1 (adjacent to the anterolateral commissure),
P2 (central scallop), and P3 (adjacent to the posteromedial commissure) [9]. The
larger, tongue-shaped, highly mobile anterior mitral leaflet extends from the
anterolateral papillary muscle to the ventricular septum. This cusp does not have
indentations; however, its segments are named for the parallel scallops of the
anterior leaflet: A1, A2, A3.
The left ventricle most commonly has two major papillary muscles: the postero-
medial papillary muscle attached to the diaphragmatic ventricular wall and the
anterolateral papillary muscle attached to the sternocostal ventricular wall. Chordae
tendineae from each papillary muscle go to both mitral cusps. The rough zone is
where the chordae tendinae insert into the mitral leaflets. The coaptation zone is free
edge of the leaflet, which is the thicker zone. The coaptation zone is where the valve
leaflets meet in systole.
2.3.2 Normal Physiology
The four valves of the heart ensure the unidirectional flow of blood. During diastole,
the AV valves open passively when pressure in the atria exceeds that in the ventri-
cles. The papillary muscles are relaxed. The valve cusps part and project into the
ventricle, forming a funnel and thus promoting blood flow into the ventricles. Each
cusp of the semilunar valves bears a nodule in the midpoint of its free edge, flanked
by a thin connective tissue area called the lunula. Toward the end of diastole, the
deceleration of blood flowing into the ventricles, movement of blood and increasing
pressures in the ventricle compared with lessening pressures in the atria causes the
nodules and lunulae to meet centrally, closing the valve. Filling of the coronary
arteries occurs during diastole (when ventricular walls are relaxed). During systole,
the free edges of the valve cusps are prevented from being everted into the atria by
contraction of the papillary muscles and tension in the chordae tendineae. Thus, in
the normal heart, blood is prevented from flowing backward into the atria despite
the high systolic ventricular pressures. The normally functioning aortic valve closes
during mid- to end-diastole as left ventricular filling and left atrial contraction takes
place. The aortic valve opens at the end of isovolumetric contraction and remains
open throughout systole or left ventricular ejection of blood through the ascending
aorta to the systemic circulation (Fig. 2.2).
2.4 Etiologies and Pathogenesis of Acquired VHD
Valvular heart disease may be classified as acquired or congenital. Congenital

VHD develops before birth whereas acquired VHD develops later in life. Acquired
VHD may then be further categorized as rheumatic or non-rheumatic (Fig. 2.9).
Rheumatic VHD (RVHD) is a consequence of streptococcal infection which may
Congenital VHD Calcific
Rheumatic
Acquired VHD Degenerative
Non-rheumatic
Functional
Endocarditis/
Inflammatory
Fig. 2.9 Etiologic categories of valvular heart disease

result in rheumatic fever and subsequent inflammatory changes and scarring of

cardiac tissue. Non-rheumatic VHD (NRVHD) generally encompass degenera-
tive (myxomatous and calcific), functional, and infectious (endocarditis) etiolo-
gies and pathogenesis [10]. The acquired NRVHD category also includes patients
who have undergone heart valve surgery, which comprises nearly one-third of
those referred for the management of VHD. The focus of this chapter is
acquired VHD.
2.4.1 Rheumatic heart disease
2.4.1.1 Epidemiology
Currently RHD affects nearly 40 million people and claims nearly 300,000 lives
each year. In industrialized high-income countries the incidence of rheumatic fever
has declined to less than 1 in 100,000 but remains higher than 100 in 100,000 in less
developed low and middle-income countries [11]. Between 1990 and 2017, how-
ever, the global incidence of rheumatic heart disease (RHD) has decreased by 8.67%
[2]. Measures to reduce RHD particularly in LICs and MICs include antibiotics to
treat and prevent streptococcal infection, decreased crowding, improved housing
and sanitation, and access to healthcare. There has been a 54% decrease in the age-
standardized death rate for RHD and a 53% decrease in global age-standardized
disability adjusted life years rate (sum of years of life lost due to premature mortal-
ity and the years lived with a disability) [2]. Despite these improvements, RHD
remains the number one cause of global cardiovascular death in children and young
adults [2]. RHD is a persistent, pervasive global health problem without a cure or
vaccine [12].
2.4.1.2 Pathogenesis
Rheumatic heart disease is a consequence of rheumatic fever, an autoimmune disor-
der resulting from untreated or undertreated Group A β-hemolytic streptococcal
infection. It is thought that during an initial or recurrent upper respiratory infection,
lymphatic channels from the tonsils transmit group A Streptococci to the heart.
Rheumatic fever causes inflammation of the skin, tissue, and heart valves; the latter
results in RVHD [13, 14].
Acute rheumatic fever involves diffusive exudative and proliferative reactions in
these tissues. Active AHD may present with cardiac failure and require surgery,
with the potential for rheumatic fever to recur. Initially based upon expert opinion
not clinical trials, the Jones criteria for diagnosis of rheumatic fever were introduced
in 1944 and have been iterated to date. Major diagnostic criteria include carditis,
erythema marginatum, subcutaneous nodules, and polyarthritis. Minor diagnostic
criteria include arthralgia, fever, and elevated C-reactive protein. The relationship
between diagnostic criteria and rate and severity of RHD recurrence is unclear.
Recovery from myocarditis and cardiac failure can occur however RVHD can
remain [13, 15].
2.4.1.3 Presentation
In almost all RVHD cases the mitral valve is affected although the aortic valve may
also be involved. Regurgitation is seen in the early stages and stenosis in the later
stages [16]. During the early phase of RVHD, echocardiography has detected small
verrucous nodules caused by thrombi along the lines of coaptation [15, 17]. These
nodulous lesions alone do not result in pathoanatomic changes. Long-term inflam-
mation can occur after isolated or recurrent episodes of rheumatic fever, ultimately
leading to valve dysfunction in genetically predisposed patients. Leaflets are usually
minimally fibrosed and pliable in three-quarters of patients under age 30, however
after age 40 scars and ridges are observed in two-thirds of patients [15, 17]. Different
morphologic changes may lead to different presentations. Chordal shortening is
dominant in 90% of patients with mitral stenosis, but only occurs in 3% of patients
with pure mitral regurgitation [16]. Annular dilatation is found in 90% of patients
with pure rheumatic mitral regurgitation, but only in 30% in cases of pure mitral
stenosis. In late progression, fusion and chordal shortening is seen along with com-
missural fusion and endothelial surface erosion. Fibrosis and inflammation are
major findings however lipids may trigger calcification in genetically predisposed
cases [15].
The typical physical assessment signs of RVHD are murmurs of aortic and mitral
insufficiency. A high-pitched, blowing, pansystolic murmur may be noted when the
mitral valve is affected. A short, low-pitched mid-diastolic murmur known as a
Carey Coombs murmur may be noted at the apex. The Carey Coombs murmur may
be due to swelling and stiffening of mitral valve leaflets, increased gradient across
the valve, and decreased left ventricular compliance.
2.4.2 Non-rheumatic Valve Disease
2.4.2.1 Infective Endocarditis

Infective endocarditis includes acute and subacute bacterial endocarditis, and non-
bacterial endocarditis caused by viruses, fungi, and other microorganisms. The
insidious and changing nature of the etiologic agent(s) often means that by the time
a patient presents, disease is already severe. Both rheumatic and congenital valve
disease are predisposing factors to endocarditis.
Epidemiology
Endocarditis has increased since 1990, reaching an estimated 1.09 million incident
cases, 1.72 million disability adjusted life years, and 66,300 deaths in 2019. Age-
standardized rates show an incidence of 13.8 per 100,000 and death rate of 0.9 per
100,000 [18]. The increase in deaths are attributed to virulent, more widespread
organisms including Staphylococci, and complex infections in patients at extreme
risk for cardiac surgery [18].
The etiologic agent depends upon the patient history, valve anatomy, and the
portal of entry [19, 20]. Acute endocarditis usually affects normal heart valves and
is most commonly caused by Staphylococcus aureus. Brucella and listeria strains

may also be the cause. Staphylococcus aureus is the leading cause of endocarditis in
children. Native valve endocarditis usually involves the mitral valve; 20% of cases
are seen in mitral valve prolapse. The aortic valve may also be affected especially if
there is congenital heart disease such as a bicuspid aortic valve or Marfan syn-
drome [18].
Subacute endocarditis is most commonly caused by streptococcal bacteria. The
most common pathogens in subacute bacterial endocarditis include streptococci,
enterococci, coagulase-negative staphylococci, and the HACEK group of organisms
(Haemophilus species, Actinobacillus actinomycetemcomitans, Cardiobacterium
hominis, Eikenella species, and Kingella kingae). Viridans-type streptococci (alpha-
hemolytic streptococci) follow as a close second, usually prompted by a recent den-
tal procedure. Coagulase-negative staphylococcus is often the etiologic agent in the
presence of an indwelling central venous catheter. Staphylococcus aureus (includ-
ing methicillin resistant S aureus) and Staphylococcus epidermis are the predomi-
nant etiologic agents in early prosthetic valve endocarditis while Streptococci
usually cause late prosthetic valve endocarditis. Following open-heart surgery and
particularly if immunosuppressed, fungal organisms may be an issue. With intrave-
nous drug use, Gram-negative organisms are rarely the cause. Rather, Staphylococcus
aureus is the most common cause, followed by Pseudomonas aeruginosa or Serratia
marcescens [21].
Pathogenesis
Three processes drive the pathogenesis of infective endocarditis. First, the heart
valve must be primed for bacterial adherence. Circulating bacteria do not readily
adhere to normal endothelial surfaces. Alterations in endothelial surfaces due to
trauma, inflammation, disruption of the ECM, deposition of platelets and fibrin may
render the valve more susceptible to infection. In addition to trauma, other risk or
priming factors include diabetes, steroid or immunosuppressant use, advanced age,
and pacemaker intervention [19]. Second, circulating bacteria, particularly certain
strains as described, adhere to the fibrin-platelet matrix. Thirdly, antibodies may
block adherence but bacteria may be resistant to the complement cascade and leu-
kocyte phagocytosis. Some streptococci for example prompt valve cells to produce
thromboplastin, which triggers the deposition and growth of a fibrin-platelet clot
over bacterial colonies, or vegetations [22].
Presentation
Symptoms may be minimal to non-existent early in the disease. A prolonged persis-
tent fever that lasts for several months may be the sole sign. Onset may also be acute
and severe with a high fever. Nonspecific symptoms such as headache, nausea, vom-
iting, chills, fatigue, myalgia, and arthralgia may also be reported. A new or chang-
ing heart murmur may be associated with heart failure. Fibrotic changes and
vegetations may cause stenosis and/or insufficiency of the heart valves, usually the
mitral valve, and thrombus formation may result in emboli. Abscesses may form
further damaging the lining of the heart and heart valves.
Staphylococcal disease is associated with neurologic presentations ranging from

altered cognition and focal neural signs to embolic strokes, cerebral abscesses, hem-
orrhage, and increased intracranial pressure. Myocardial abscesses may present as
heart block due to cardiac conduction system damage, or even purulent pericarditis
due to an abscess rupturing into the pericardium. Late in the course skin manifesta-
tions including Roth spots (bleeding in the back of the eye), Janeway lesions (small
painless spots of the palms and soles of the feet), Osler nodes (pain nodules at the
fingertips), splinter hemorrhages, and petechiae may be seen. These lesions are
indicative of vasculitis from circulating antigen-antibody complexes [21]. A defini-
tive diagnosis of infectious endocarditis is made using Modified Duke Criteria [19]
(Table 2.2).
Treatment is out of the scope of this chapter. In terms of prevention, it is noted
that prophylactic antibiotic recommendations have changed dramatically. In 1955
Table 2.2 Definition of infectious endocarditis according to the modified Duke criteria
Definite IE
Pathological criteria:
• Microorganisms demonstrated by culture or histological examination of a vegetation, a
vegetation that has embolized, or an intracardiac abscess specimen; or pathological
lesions; vegetation or intracardiac abscess confirmed by histological examination showing
active endocarditis
Clinical criteria:
• Two major criteria OR
• One major criterion and three minor criteria OR
• Five minor criteria
Possible IE
• One major criteria and one minor criterion OR
• Three minor criteria
Description of diagnostic criteria
Major criteria
• Blood culture positive for IE
• Typical microorganisms consistent with IE from two separate blood cultures: Viridans
streptococci, Streptococcus bovis, HACEK group, Staphylococcus aureus; or community-
acquired enterococci in the absence of a primary focus, or microorganisms consistent with
IE from persistently positive blood cultures defined as follows: At least two positive
cultures of blood samples drawn >12 h apart or all three or a majority of ≥4 separate
cultures of blood (with first and last sample drawn at least 1 h apart)
• Single positive blood culture for Coxiella burnetii or anti–phase 1 IgG antibody titer
≥1:800
• Evidence of endocardial involvement
• Echocardiogram positive for IE (TEE recommended for patients with prosthetic valves,
rated at least possible IE by clinical criteria, or complicated IE [paravalvular abscess];
TTE as first test in other patients) defined as follows: Oscillating intracardiac mass on
valve or supporting structures, in the path of regurgitant jets, or on implanted material in
the absence of an alternative anatomic explanation; abscess; or new partial dehiscence of
prosthetic valve or new valvular regurgitation (worsening or changing or preexisting
murmur not sufficient)
Table 2.2 (continued)

Minor criteria
• Predisposition, predisposing heart condition, or IDU
• Fever, temperature >38 °C
• Vascular phenomena, major arterial emboli, septic pulmonary infarcts, mycotic aneurysm,
intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions
• Immunological phenomena: Glomerulonephritis, Osler nodes, Roth spots, and rheumatoid
factor
• Microbiological evidence: Positive blood culture but does not meet a major criterion
(excludes single positive cultures for coagulase-negative staphylococci and organisms that
do not cause endocarditis) or serological evidence of active infection with organism
consistent with IE
• Echocardiographic minor criteria have been eliminated
Adapted from Baddour Larry M, Wilson Walter R, Bayer Arnold S, et al. Infective
Endocarditis. Circulation. 2005/06/142005;111(23):e394–e434. https://doi.org/10.1161/
CIRCULATIONAHA.105.165564
HACEK indicates Haemophilus species, Aggregatibacter species, Cardiobacterium hominis,
Eikenella corrodens, and Kingella species, IDU injection drug use, IE infective endocarditis, IgG
immunoglobulin G, TEE transesophageal echocardiography, TTE transthoracic echocardiography
the American Heart Association recommended penicillin prophylaxis before dental

procedures for patients with congenital heart disease and rheumatic heart disease.
The lack of research evidence supporting this practice coupled with antibiotic resis-
tance led the United States and Europe to limit the use of antibiotic prophylaxis as
of 2009. While the United Kingdom does not recommend antibiotic prophylaxis
preceding dental procedures, this practice persists in the U.S. for patients with pros-
thetic cardiac valves including transcatheter implanted prostheses and homografts;
prosthetic material used for cardiac valve repair (e.g., annuloplasty rings, chords, or
clips); previous infectious endocarditis; unrepaired cyanotic congenital heart dis-
ease or repaired congenital heart disease with residual shunts or regurgitation adja-
cent to prosthetic material; cardiac transplant with valve regurgitation due to a
structurally abnormal valve (Class IIa recommendations) [23].
2.4.2.2 Degenerative Valve Disease
Epidemiology
Degenerative valve disease historically encompassed both aortic and mitral valve
disease. Evidence has demonstrated that degenerative mitral valve disease is patho-
physiologically distinct from calcific aortic valve disease [8]. Affecting approxi-
mately 2% of the population [24, 25], the prevalence of degenerative mitral valve
disease was 18.1 million with 35,700 deaths globally in 2017 [26]. The most com-
mon degenerative mitral valve disease is mitral valve prolapse [18]. Overall, women
are disproportionately affected. Disability adjusted life years are higher in women
than men for all age groups; women also have higher mortality rates and lower rates
of surgery [27].
Pathogenesis
Degenerative valve disease generally encompasses two types of basic histopatho-
logical processes: myxomatous or fibrotic change [6]. Myxomatous degeneration is
characterized by proteoglycan accumulation, degradation of collagen, and fragmen-
tation of elastin. These pathoanatomic changes result in prolapse and regurgitation
due to a “floppy” valve. Fibrotic degeneration is characterized by collagen accumu-
lation, degradation of proteglycan, and elastin fragmentation. These pathoanatomic
changes results in restriction of leaflet motion and valve stenosis, or a “stiff” valve.
Sclerosis or hardening may be seen in the early stages; valve calcification is a late
finding of progressive fibrosis and advanced disease [6, 28]. At the cellular level,
valvular intracellular (VIC) activation and extracellular matrix (ECM) remodeling
is seen and the normally stratified leaflet structure is disrupted [29]. Left untreated,
degenerative mitral valve disease results in atrial fibrillation and heart failure. The
hemodynamic, echocardiographic, and physical presentation of degenerative valve
disease are further discussed in later sections.
2.4.2.3 Calcific Valve Disease
Epidemiology
Calcific aortic stenosis is the predominant calcific valve disease globally, usually
owing to aging affecting more than 1000 per 100,000 of the population over age
75 [18, 25]. Approximately 1 in 8 people over age 75 are estimated to have
moderate-to-severe aortic stenosis [30]. The mortality rate is 50% at 2 years for
once diagnosed with symptomatic severe aortic stenosis [31]. In high-income
countries, it is the third leading cause of cardiovascular death. In 2017, the preva-
lence of calcific aortic valve disease was 12.6 million with 102,700 deaths [26].
Between 1990 and 2017, the number of disability adjusted life years increased for
calcific aortic valve disease increased by 101% [26]. Along with high morbidity,
mortality, and healthcare costs, patients with aortic valve stenosis also have a high
rate of survival after definitive intervention. Diagnosis and timely intervention are
of prime importance.
Pathogenesis
Spanning from sclerosis to stenosis, calcific valve disease, namely of the aortic
valve, is not simply a degenerative disease. Histopathological and clinical data have
emerged to suggest calcific valve disease is much like atherosclerosis, involving the
deposition of lipoproteins, chronic inflammation, and active calcification akin to
bone formation if an osteoblast phenotype is present [8, 32] (Fig. 2.10). Osseous
metaplasia has been observed on calcified aortic valves excised in surgery. Patients
with these calcified valves have significantly greater prevalence of atherosclerotic
vascular diseases including coronary artery disease, peripheral arterial disease,
hypercholesterolemia, and hypertension than patients without valve calcification
[33]. Calcific aortic valve disease and its hemodynamic, echocardiographic, and
physical presentation are further discussed in later sections.
Fig. 2.10 Disease progression in calcific AS, illustrating changes in aortic valve histological fea-
tures, leaflet opening in systole, and Doppler velocities. In (a), the histology of the early lesion is
characterized by a subendothelial accumulation of oxidized low-density lipoprotein (LDL), pro-
duction of angiotensin (Ang) II, and inflammation with T lymphocytes and macrophages. Disease
progression occurs by several mechanisms, including local production of proteins, such as osteo-
pontin, osteocalcin, and bone morphogenetic protein 2 (BMP-2), which mediate tissue calcifica-
tion; activation of inflammatory signaling pathways, including tumor necrosis factor alpha (TNF
α), tumor growth factor beta (TGF-β), the complement system, C-reactive protein, and
interleukin-1β; and changes in tissue matrix, including the accumulation of tenascin C, and up-
regulation of matrix metalloproteinase 2 and alkaline phosphatase activity. In addition, leaflet
fibroblasts undergo phenotypic transformation into osteoblasts, regulated by the Wnt3–Lrp5–β-
catenin signaling pathway. Microscopic accumulations of extracellular calcification (Ca2+) are
present early in the disease process, with progressive calcification as the disease progresses and
areas of frank bone formation in end-stage disease. The corresponding changes in aortic valve
anatomy are viewed from the aortic side with the valve open in systole (b) and in Doppler aortic-jet
velocity (c). (Used with permission from ESC CardioMed (3 ed). Edited by A. John Camm,
Thomas F. Lüscher, Gerald Maurer, and Patrick W. Serruys. European Society of Cardiology
Oxford University PressPrint. Publication Date: Dec 2018 Print ISBN-13: 9780198784906
Published online: Jul 2018 https://doi.org/10.1093/med/9780198784906.001.0001)
2.5 Aortic Valve Disease
2.5.1 Aortic Stenosis
2.5.1.1 Etiology and Pathoanatomy

Disease processes that result in aortic valve stenosis can be classified as congenital
or acquired. The acquired group largely consists of calcific (formerly “degenera-
tive”) and post-inflammatory (or rheumatic) disease. Aortic stenosis is a gradually
progressive disease that may have a long asymptomatic phase often lasting for
decades that is then followed by a symptomatic phase with severe narrowing of the
aortic valve orifice. Upon onset of symptoms, survival declines rapidly. The age at
which aortic stenosis becomes symptomatic is determined by the underlying cause.
Aortic stenosis occurring from 1 to 30 years of age usually represents congenital
aortic stenosis. Aortic stenosis presenting at the ages of 40–60 years is primarily
rheumatic in origin or secondary to calcific aortic stenosis in a congenitally bicuspid
aortic valve. In patients past the age of 60–70 years, calcific degenerative stenosis is
the most prevalent cause [10, 34]. Aortic stenosis is characterized by a fibrocalcific
transformation of the valve leaflets, resulting in progressive narrowing of the aortic
valve opening.
2.5.1.2 Pathophysiology
Aortic stenosis is accompanied by a hypertrophic response of the left ventricle,
which may result in dyspnea, heart failure, syncope, angina, and ultimately death
(Fig. 2.11). Over the past decade, evidence has emerged suggesting that the patho-
physiology of aortic stenosis occurs in two phases. The initiation phase is character-
ized by endothelial damage, lipid infiltration, and inflammation mimicking
Onset of severe symptoms

100
Angina
80 Syncope
Failure
Latent period
Survival (%)
60 (increasing obstruction,
0 2 4 6
myocardial overload) Average survival (years)
40
20 Average death
(age)
0
0 10 20 30 40 50 60 70 80
Age (years)
Fig. 2.11 Natural history of aortic stenosis. Natural history of aortic stenosis. Survival is excellent
during the prolonged asymptomatic phase. After the development of symptoms, mortality exceeds
90% within a few years. Presenting symptoms are distinctly associated with survival. (Adapted
from Ross & Braunwald and used with permission from Carabello BA, Paulus WJ. Aortic stenosis.
Lancet. 2009 Mar 14;373(9667):956–66. https://doi.org/10.1016/S0140-6736(09)60211-7. Epub
2009 Feb 21. PMID: 19232707)
atherosclerosis. The second phase is propagation, in which valve fibrosis and calci-
fication (similar to skeletal bone) occurs in a proliferative cycle. Valve injury drives
cyclical calcium formation and disease progression. As the valve cusps become less
mobile, the valve orifice decreases in size, increasing the pressure gradient across
the valve. Flow becomes increasingly turbulent during systole which is audible on
auscultation. Increased ventricular systolic pressure is necessary to eject blood
across the stenosed valve. The left ventricle must pump harder to overcome valvular
resistance. The high left ventricular-aorta pressure gradient drives blood into the
aorta, initially maintaining cardiac output. Over time the increased left ventricular
afterload results in compensatory concentric left ventricular hypertrophy. The left
atrium dilates and hypertrophies as a result of the pressure overload; the stiff left
ventricle and high left ventricular pressure impairs left ventricular filling. This dia-
stolic dysfunction decreases cardiac output; thus, cardiac output becomes increas-
ingly dependent upon atrial filling of the left ventricle. The onset of
symptoms—angina, syncope, and heart failure—represent the inflection point for
decline in the disease’s course [35].
2.5.1.3 Clinical Manifestations

Although initially adaptive in aortic stenosis and helps to preserve ejection perfor-
mance, left ventricular hypertrophy impairs coronary bloodflow reserve, reduces
diastolic function, and is associated with increased mortality [35, 36]. Patients with
heart failure with reduced EF (HFrEF) have significant myocardial dysfunction and
LV performance is more sensitive to changes in afterload conditions than in patients
with normal EF. As aortic stenosis becomes more severe, ventricular systolic func-
tion may also decline, resulting in congestive heart failure. With higher pressure
gradients, blood from the left ventricle is ejected back into the left atrium, which
worsens any underlying mitral regurgitation [34].
Unlike the systemic circulation, coronary perfusion of the myocardium occurs
primarily during diastole, and oxygen extraction is near maximum. Myocardial
oxygen demand is increased secondary to increased left ventricular wall stress and
muscle mass. Thus, the sole compensatory strategy is to increase coronary blood
flow to meet the increased myocardial oxygen demand. The thickness of the left
ventricle due to compensatory hypertrophy, however, challenges epicardial coro-
nary arteries to perfuse the endocardium. Myocardial oxygen delivery is decreased
as a result of decreased coronary perfusion pressure. Angina may occur in the
absence of coronary artery disease because of this described imbalance in myocar-
dial oxygen supply and demand [37].
When aortic stenosis is critical, cardiac output is fixed. There is no further com-
pensation that can increase the flow or reduce the pressure gradient across the aortic
valve. Thus, another ominous symptom of aortic stenosis is syncope. The exact
mechanism of syncope in aortic stenosis remains unclear but syncope is usually
exercise-induced in the face of fixed cardiac output. Because of this fixed cardiac
output, syncope or near syncope may also occur during orthostatic blood pressure
Another random document with
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English ships, and, I trust, have no difficulty in making our
way. Simon Sablot is in the secret, and will have the
animals all ready."
"And when shall we set out?" I asked anxiously.
"To-morrow night, my little one. I must go once more to

Avranches to bestow in safety the money belonging to our
consistory, which thou knowest is in my hands."
"Could not Simon take the money to Avranches?" asked

my mother.
"And thus run the risk while I was escaping? Nay, my

Margaret, that is not spoken like thyself. But, in truth, my
risk would be much less than his. Thou knowest I have
made many errands thither of late, concerning the houses
which are being repaired in the market-place. No one will
think it at all strange."
My mother shook her head, but both she and I knew

that, once my father's mind was made up on a point of
duty, there was no more to be said.
The day passed quietly and sadly enough, for we all felt
it was probably the last day we should spend in the dear old
house. Our preparations were all completed, even to filling
the panniers of the spare donkey with the dried fruits and
other matters which were to form our ostensible errand to
Honfleur. As my father said, he had laid by a considerable
amount of wealth in diamonds and other jewels, which,
being of small bulk, could be easily concealed about our
persons. We had also about three hundred Louis in gold,
which was divided between us. We dared take but very few
clothes, and as for books or any treasures of that sort, they
were of course quite out of the question.
I think none of us slept that night. I am sure I did not.
It seemed to me as if I could not endure to lose sight for a
moment of the things and places I was so soon to leave
forever. At daylight my father called us all together, and for
the last time we joined in prayer about that family altar
which was so soon to be laid in ruins, never to be builded in
that place again.
But why should I say so? Never is a long day. Perhaps

some time, in the councils of heaven, that altar may be
once more erected.
We took our breakfast together very silently, and then

my father kissed us all and mounted his horse to go to
Avranches, taking Andrew with him. My mother called all
the servants and paid them their wages, with a little present
into the bargain. I believe the good souls had an idea of
what was going to happen, though none of them said a
word. It was a weary day, for we had done everything we
could think of by way of precaution, and the time hung
heavy on our hands. My father was to have returned by
three o'clock, but the hour struck and he did not come.
Alas, never again!
I had gone down to the gate for the tenth time to look
for them, when, as I opened the little wicket, I met Pierre
Le Febre face to face.
"Thank the holy archangel," said he breathlessly. "I was

wondering how I should get speech of you, mademoiselle.
But let me come in, for I have somewhat to say."
I let him into the courtyard, and called my mother to

hear Pierre's tale.
"I was standing by the great gate of the hospital, as

they call it," said he. "I had sold my fish to the Sisters, and
was waiting for my money when the wicket suddenly
opened and Lucille Sablot looked out. Ah, madame, how
changed! But, as I said, she looked out, and, seeing no one,
she put this little packet into any hand."
"'Quick, Pierre, if ever you cared for me,' said she. 'This
for Mamselle Vevette, and make haste. Life and death are in
thy steps. Tell Vevette I dared not write, but she will know
what this means by the English name.' Then she drew in
her head, and I heard some one scolding her within for
looking out of bounds."
Breathlessly I opened the paper. There was nothing in it

but a grosse mouche, what in English we call a bluebottle.
"A fly," said I. "Fly! That is what it means, maman.

Lucille has sent us a warning. She knows of some danger
that threatens us immediately. What shall we do?"
"Oh, if your father were but here!" said my mother,

wringing her hands convulsively.
"There he comes," said I, and at that moment

appeared, not my father, but Andrew, riding across the
fields at break-neck speed, his horse covered with foam. He
sprang to the ground, flinging his reins loose anyhow.
"Armand! My husband!" said my mother. "Where is he?"
"To the tower first, aunt, then I will tell you all. Pierre, if
ever he or I did you a good turn, do me one now!" said
Andrew sharply. "I do not ask you to risk yourself, but let
me have your boat. The wind is fair. We must run for Jersey
as soon as it is a little later. Go, and get it ready."
"My boat does not go without me, monsieur," said

Pierre. "I can bring it back, and if I am out two or three
days I am kept by the wind. You can never manage it alone;
you do not know the channels, and I do."
"As you will; but have it ready by ten this night. It will
be very dark, but so much the better. Run, now. Come,
aunt, for Heaven's sake, for your child's sake."
For maman stood like a marble statue.
"I will not move till you tell me news of Armand," said
she.
"He is with God," answered Andrew, with a convulsed

face. "His last words were, 'Tell Margaret to escape, for my
sake, and the child's. We shall meet again.'"
"True, we shall meet again. It is but a short parting,"

said my mother musingly.
Then, as Andrew stamped his foot with impatience, she

seemed to rouse herself.
"I am ready, my dear son. What shall we do?"
"Go, you and Vevette, and put on your peasant dresses,

and secure the money and jewels, while I warn the
servants. I want them to find an empty nest. Stay in your
room till I come."
We obeyed at once. My mother was pale as ashes, but

calm, and even cheerful. As to myself. I believe I retained
only one rational thought at that moment—to do as I was
bid. We changed our dresses and made our other
arrangements with the speed of thought, but we had hardly
finished before the noise of voices and clapping of doors
told that the alarm had been given. In another moment
Andrew appeared.
"I have told them that the mob are coming, and that
their ladies have already escaped. I have bid them take to
the woods for the night. Come, now! Leave everything in all
the confusion possible to look like a hasty flight. It will all
the better throw them off the scent."
We entered the secret passage, and closing it securely

after us we sought the upper floor of the tower—not,
however, the uppermost one, but the second.
"Do you know the way, Andrew?" I asked. "My father

said these floors were not safe."
"They are safe enough for us, but our enemies will not
find them very safe," was Andrew's response. "Step lightly,
and follow me exactly."
We went around the side of the room to a cupboard

with shelves, masking a door so entirely that no one would
have known it was there. This door opened into a second
and much smaller room, which again opened upon the
staircase up which I had led the preacher.
"We can take breath now," said he. "We need not seek
the vaults till we hear them approaching, and not then
unless they come into this tower."
"They will come," said I. "Remember the staircase from

the gallery."
"Let them," was Andrew's grim reply. "There are a few

secrets about this place which even you do not know,
Vevette."
As he spoke he stooped down, drew out two large iron

bolts and laid them on the floor.
"The trap is set and baited," said he; "now let the rats
walk in whenever they please."
"But how—how was it," I asked in a whisper, for my

mother never said a word. The fact that my father was dead
seemed enough for her.
"We had hardly reached Avranches when we heard the

uproar in the market-place," returned Andrew. "At first we
did not think of the cause, but as soon as we caught sight
of the place we saw what was going on. They were pulling
down the houses of the Protestants, and dragging out the
women and the little children."
Andrew shuddered and covered his face. "I saw one

man in a friar's gown take two little baby girls in his arms
and try to carry them out of the press, but they were torn
from him. Then they caught sight of us, and one cried out,
'There is the arch heretic. There is the man who shelters
the preachers.' And a volley of stones flew about our ears.
We turned to fly, as there was clearly nothing else to be
done, but a man named Michaud—I don't know whether you
know him—"
"My father saved him from the galleys," said I.
"Well, he raised his arquebus and deliberately fired at

my uncle, wounding him in the breast. He did not fall nor
lose his presence of mind, and by lanes and by-ways we
gained the wood. Then he sank to the ground, and I saw
that he was dying.
"'Lose no time with me,' said he faintly. 'Hasten home at

once. Did we not hear them cry, "To the tower!" Remember
the secret passage. Hide as long as you can, if you cannot
get away. Go not by the road, but across the heath. Why do
you stay?'
"But I did not leave him till he had breathed his last.
Then I drew his body aside into the bushes, and hastened
hither."
"And do you think they will come?" I asked, as soon as I

could speak.
"I most surely do," he answered. "The hope of plunder

would bring the rascals, of whom there are abundance. The
priest sets on the zealots and others join because they are
afraid of being suspected of favoring the cause."
We sat in silence for what seemed a very long time, till

the great clock struck eight. At that very moment we heard
a shout and the trampling of many feet, while a strong glare
shone through the little grated casement of the room.
"There they are," said Andrew, stepping to the window.

I followed him and looked out. On they came, a mob of
ruffians and abandoned women, with many, too, of whom I
should have hoped better things. Heading the press was
one of the curés of Avranches, a man whose openly
dissolute life was a scandal to his own people. There were
also two or three friars, among them the one who had
visited us the day before.
"Ah, the traitor!" said I. "My father's old companion in

arms, and but yesterday eating his bread."
"I believe you do him injustice," said my mother, in as

calm a tone as if she were speaking of the most ordinary
matter. "He has come in the hope of rendering us some
service. Poor, miserable, deluded people!"
"I would I had some charges of grapeshot for these

poor people," said Andrew. "They would go farther to dispel
their illusions than a deal of reasoning. Anything but hiding
like rats in a hole. But we have no choice. Not a word or
sound, for your lives. But what is here?"
It was something which in my excited state almost sent

are off into a hysterical laugh—namely, my great, long-
haired, white cat Blanchon, which had followed us into the
tower, and now mounted upon the window-seat was
growling savagely at the intruders. He was an odd creature,
very fond of his friends, but formidable to his enemies, and
he had this peculiarity, that he never mewed. A strange yell,
which sounded like that of a human being in the wildest
rage, when he flew upon his enemies, and a loud purr were
all the noises he ever made.
"Let him be. He will do no harm," said I. "He never

makes any noise. What shall we do now?" as the mob made
their onslaught on the gates with a savage yell which made
me shudder.
"Keep quiet," was the reply. "We are safe enough unless
they set fire to the tower."
In another moment the gate yielded, and the people

poured in. Before one could speak they were all over the
house, calling to each other and venting their rage at
finding no one by breaking and destroying all before them.
"To the old tower, comrades!" finally cried a voice.

"There is the hiding-place."
I suppose numbers gave the people courage, for I am

certain not one of them would have dared invade the
domain of the white chevalier alone. We heard the rush up
the stairs and then the battering down of the door. Then
there was a short pause.
"Come on," cried the same voice, which I now
recognized as that of Michaud, our old gamekeeper, whom
my father had saved only to be murdered by him. "Come
on. Who cares for ghost or devil?"
There was a rush into the room, then a cry from those
nearest the door.
"Take care! The floor!"
But it was too late. The loosened boards gave way, and
down went a dozen men, Michaud among them, through a
yawning gulf clear to the ground floor.
"Back! Back! The tower is falling!" was the cry, while the
shrieks of the men below added to the confusion. The tower
was at once deserted, and we presently heard sounds which
told us that the fallen men were being rescued from amid
the ruins of the floor.
"To the cellars!" cried now the voice of Pierre Le Febre.

"Let us taste the old chevalier's wine and brandy."
"Good, Pierre!" said Andrew. "Once let them get among

the casks and bottles, and we are safe."
"If Pierre does not get among them himself," said I.
"I do not believe he will, and in any case we have the

boat. But it is time we were stirring. Aunt, can you walk?"
"Oh, yes! I can do anything you wish," answered my

mother, in the same calm way. She seemed to have all her
wits about her, but she did not speak unless we spoke to
her.
"Come, then," and he opened the door of the secret
passage into which pussy led the way, majestically waving
his tail and looking back as if to say, "Come on, and fear
nothing! You are under my protection."
I remember smiling, in all my grief and anxiety, at his

air of patronage.
I went first, after I had lighted the lantern, then came

my mother, and lastly Andrew.
We heard only distant and muffled sounds, and judged

that the people were busied in the cellar, where was stored
not only wine and liquor, but abundance of old cider, strong
as brandy itself.
We had just reached the level of the chapel and were

about passing the door which led into it, when Blanchon the
cat stopped, growling fiercely. In another moment a light
shone through the opened door. The next Blanchon sprang
forward with his wild, unearthly yell of onset, and flung
himself into the face of a man who had just put his head
through the opening. There was a scream of quite another
character, and the man fled stumbling and falling on his way
out, while Blanchon came back to us with the loud purr,
which was his way of expressing complacency.
"Good cat," said Andrew. "That man won't find his way
back in a hurry, but some one else may. Hold up the light,
Vevette."
I held up the light while Andrew pulled to the door and

with a stone smashed the spring-lock.
"Nobody will open that, even if any one dares try," said
he. "Now for all the haste we can make."
I caught up Blanchon and carried him, to which he
made no objection. We were soon in the open air, and
walking quickly down the course of the stream which had
scooped out the valley, we found ourselves in the little
hamlet. It seemed to be deserted. Not a man was to be
seen, nor a light, save in Isabeau's cottage. The night had
grown wild and stormy, but it was not very dark. And we
could see the mast of the boat, which lay at the end of the
little pier.
"Now if Pierre has been true," said Andrew, and at that

moment we heard his voice.
"Monsieur and madame, is that you! All is ready; but we

shall have a wild night."
"Never mind, so long as the wind is fair," returned

Andrew, in the same whisper. "I would rather face the sea
than the devils we have left behind."
We were assisted into the boat. I holding fast to my cat,

and set sail. I can give little account of the voyage. I know
it was a rough and tempestuous one, and that we were
many times in the greatest danger from the rocks and
counter currents which make navigation in those parts so
difficult.
Andrew had the helm most of the time, while Pierre,

whose smuggling and other lawless exploits had made him
well acquainted with the channel, directed our course. My
mother sat quite still under the half-deck of the boat, and I
dozed by fits, with Blanchon in my lap, who now and then
uttered a peevish growl, as he vainly tried to lick himself
dry.
"There comes the morning at last," said Le Febre

joyously; "and here is the blessed St. Aubin's bay spread
out before us, if we can but get into it. I would we had a
better pilot than myself."
"Yonder comes a boat which has been out all night,"

said Andrew. And he stood up and hailed her in English:
"Boat ahoy!"
"Hilloa!" came back, as the stranger rapidly overhauled

us. "Who are you?"
"English," was the answer. "We have ladies on board.

Where are you bound?"
"To St. Aubin's," was the reply. "Follow us, and you will
do well enough."
"Good!" said Andrew to my mother. "We shall land close

at home. And now that we are comparatively safe, tell me,
Pierre, did I not hear your voice at the tower last night?"
"You did, monsieur," was the reply. "I had a mind to see
what was going on, for I knew I would get back in time, and
without being missed. It was I who put the rascals up to
break into the cellars. The priest tried to draw them away
after him to search the old chapel, but he did not know his
men so well as I did. Then, when I saw them well engaged,
I took to my heels and reached the pier before you, not
having so far to go, or knowing the way better. But where
were you when the floors fell? I trembled for you then."
"We were safe enough, and not far off," was the reply.
"Was any one much hurt?"
"Yes; Michaud will die, and a good riddance too. There

were some broken heads and bones; I don't know how
many. But, monsieur, what could have been in the chapel
which handled the priest so terribly. I found him in the court
blinded in both eyes and his face torn to pieces as by a wild
beast, and he said something sprang at him in the old
chapel. Could it have been that devil of a white chevalier,
think you? Could a ghost handle a man like that?"
"I do not know whether or no ghosts can scratch,"

answered Andrew gravely; "but the one who attacked the
priest has been a passenger with us."
And he raised my cloak and showed Blanchon, who had

abandoned the attempt to keep himself dry, and lay a wet
and sulky heap in my lap.
Pierre's face fell.
"A white cat," said he. "If I had known we had a white
cat on board, I should have given up in despair a dozen
times. However, all is well that ends well," he added,
brightening up; "and here we come sure enough."
"And yonder is your cousin's house, Vevette," said

Andrew, pointing to a comfortable-looking mansion not far-
away. "We shall soon be under a roof once more."
The family of the fisherman whose boat had preceded

us were gathered at the landing to see us come in, and loud
were their exclamations of wonder and pity as my mother
and myself were assisted from our cramped position in the
bottom of the boat to the landing-place.
By one of the boys Andrew sent a message up to the

house, and in what seemed a wonderfully short time we
were surrounded and conveyed to the mansion Andrew had
pointed out, by a troop of excited boys and girls, under the
leadership of an elderly considerate manservant. Here we
were warmly welcomed, kissed, fed with hot soup and
mulled wine, and finally put to bed in the most fluffy of
feather-beds, my mother and myself in adjoining rooms.
Maman was still in the same curiously passive state, but not
unconscious.
"Go to rest, my Vevette," she said, kissing me as I hung

over her. "Have no fears for me. I shall do well. Thank God
that you are in safety. Ah, if thy father were but here!" And
for the first time, she burst into tears.
"That is well, my love," said my oldest cousin, to whom

I looked in anxiety. "These tears will relieve your mother,
and she will sleep, and all the better if she knows you are at
rest. Go, my child."
I was used to obey, and my kind motherly cousin

inspired confidence by her very tone. I undressed, put on
the dry warm flannels provided for me, and crept into the
bed, on which Blanchon was already established.
Oh, the delicious depths of that English bed! I thought I

should lie awake to listen to the sounds from the next room,
but I was worn-out, and fell asleep before my head was
fairly on the pillow.
CHAPTER IX.
IN JERSEY.
I SLEPT till afternoon, and when I waked I could not at

first tell where I was, everything about me was so utterly
different from anything I had been used to. My bed was
surrounded by light curtains of blue and white checked
linen, and through these at the foot I could see that the
hangings of the latticed window were of the same. The bed
was covered with a white spread worked with a curious
pattern in colored crewels. Everything was very quiet, but I
could hear the distant hum of a spinning-wheel, and the
singing of a robin outside my window.
I lay quietly a long time, half asleep and dreaming, half

bewildered, feeling as if I had died and wakened into a new
world, of which all I knew was that it was safe and friendly.
At last I raised myself, put aside the curtain, and looked
out.
The room was small, very little larger than the one I
had inhabited—oh, how long ago—but it was very different.
The window was not a mere slit almost lost in the thickness
of the wall, but a peaceful lattice, broad and low, into
which, late as it was, looked a cluster of noisette roses. The
floor was of boards instead of tiles, and covered here and
there with rugs, evidently of home construction. A little
table stood at the head of the bed, on which were placed a
bright brass candlestick, a Bible and prayer-book, and a
little cup of flowers, and a shelf on the wall held a slender
row of volumes. On an arm-chair near the bed was laid a
change of clean linen, and beside it a mourning frock.
The sight of that black frock brought back to my mind

all that had passed in the last twenty-four hours. I had been
through so much, and the need of action had been so
instant, that I had had no time, as it were, to feel what I
had lost, but now it came upon me in one moment. My
father was dead—murdered by the very man whom he had
saved from the effects of what he believed to be a false
accusation. His body lay unburied at this moment, a prey to
wild animals or more savage men. My mother and myself
were exiles in a strange land, never again to see the home
where I had grown-up, and where I had lived so happily, in
spite of uncertainty and danger.
"Oh, if my father were but here, I would not care for

anything else!" I sobbed, and covering my head I wept till I
was exhausted, and once more I fell asleep.
I was waked by some one who came very softly into the
room bearing a shaded light, and I started up in alarm.
"What has happened?" I asked, only half awake. "Have

I been asleep? Has not my father come home?"
"It is I, my love—Cousin Marianne," said the new-comer

in a soft, ladylike voice. "Do not be frightened. All is safe.
Your mother is awake, and I thought perhaps you would like
to rise and take some refreshment with her."
"Is it very late?" I asked, still bewildered. "Has neither

my father nor Andrew come back?"
"Recollect yourself, dear child," said my cousin, setting
down her light and coming to the bedside. "Do you not
remember what has happened?"
"Oh, yes, I remember!" said I, and my tears flowed

again.
My cousin sat down on the bedside, laid my head on her

shoulder, and wept with me for a while. Then she began
gently to soothe and hush me, and by degrees I grew
composed, so that when she again proposed to me to try to
rise, I was quite ready to comply. She assisted me to dress,
but looked a little displeased when she saw the black gown.
"That was thoughtless of Katherine," said she. "We are

wearing mourning ourselves, but she might have got out a
colored frock for to-day."
"It does not signify," said I. "I must put on black, of

course. How is my mother, madame?"
"She seems well in health, and very quiet and

composed," was the answer; "but I have persuaded her to
remain in her room, for I am sure she must need rest after
the events of yesterday and last night."
"Yesterday!" I exclaimed. "Is it possible that it was only

yesterday morning that I saw my father and Andrew set out
from our gate to go to Avranches?"
"So I understand from Andrew," was the reply. "I dare

say it seems an age to you. My love, how curly your hair
is."
"It curls worse than usual because it has been wet,"

said I, almost laughing at the odd transition. "Maman says
it is real Corbet hair."
"Yes, you are like your mother's family, all but the
complexion. Here is a fresh cap for you. They say that in
London young ladies do not wear caps, but I cannot think
that a modest custom. There, now, you look like an English
maiden, and a very sweet one," said the dear old lady,
kissing me, and then holding me off and regarding me with
great satisfaction, much as if I had been a doll she had just
dressed.
"Now I will let you go in to your mother, as I dare say

she would rather see you alone just at first. The next door
to this on the right hand, remember. I will go down and
send up your supper presently, and you must try to make
dear mamma eat something."
And Cousin Marianne glided away with that peculiar

swift, short step of hers, which never seemed to make any
noise even on a tiled flour. I never saw any one else move
in the same way or get over so much ground in the same
time.
It was with a feeling of awe that I opened my mother's

door. She was up and dressed, and lay back in a great chair,
with her little worn prayer-book in her hand. I now
remembered seeing her slip it into her bosom when we
changed our dresses in such a hurry. She held out her arms
to me, and I fell into them weeping; but she did not weep,
and I never saw her shed a tear but once afterward.
Seeing how calm she was, I tried to quiet myself, and

succeeded.
Then she read to me that prayer in the Litany which

begins, "O God, Merciful Father," and then for a while we
were silent.
"Do you feel quite well, my Vevette?" she asked at last.

"Yes, dear maman, only tired," I answered truly; for
though my head was a little inclined to be giddy, and I had
an odd feeling of bewilderment, as though I were some one
beside myself, I had no pain. "Why do you ask?"
"Your eyes are heavy, and your cheeks more flushed

than usual; that is all."
"And you, maman?"
"I am quite well, my love, only weary, as you say. Have

you seen any of the family?"
"No, maman; only that kind, gentle old lady. She called
herself my Cousin Marianne. Who is she?"
"She is your cousin, as she said—the sister of Mr.

George Corbet, the rector of this parish, and whose
household she has governed since his wife died. A better
woman never lived, nor one on whom advancing years
made less impression. We have fallen among kind friends in
our exile, my Vevette, and we must take care to show that
we appreciate their kindness. You will find your cousins'
ways quite different from anything you have been used to;
but do not fall into the common error of thinking that
therefore those ways must be wrong. Even if they should
laugh at you, take it in good part and laugh with them."
"I do not feel as if I should ever have the heart to laugh

again," said I, sighing.
"Ah, my dear one, you are young, and youth is elastic.

Your father would not wish to have all your life wrapped in
gloom because he hath been so early and so easily removed
to his eternal rest. But oh, my child, if you are ever tempted
to sin against your own soul by denying your religion,
remember it was for that your father laid down his life."
"I will never deny my religion!" said I almost
indignantly.
"I trust not; but no one knows how he may be tempted.

There are other inducements besides that of escaping
persecution. The smiles of the world are far more
dangerous to natures like yours than its frowns, and more
than one of our religion has given up to blandishments and
to ambition what he would never have yielded to the rack.
Your father was attacked on this side many a time, with
promises of high command, of court favor, and kingly grace,
but he never yielded an inch—no, not, as I believe, in his
inmost thoughts. Remember it, my Vevette, and let his
example be, next to your duty to Heaven, the guiding light
of your life."
The entrance of Cousin Marianne, followed by a neat

maid bearing a tray of good things, interrupted our
conversation. With that gentle, noiseless quickness, which
was one of her characteristics, she spread a little table with
a clean white cloth and arranged thereon the tempting
dishes she had caused to be prepared. She also set out two
cups and saucers of delicate china-ware—such as David had
once brought to my mother from Dieppe.
A signal dismissed the maid, who, however, presently

returned carrying a small silver coffee-pot—the first one I
had ever seen; for though coffee had come into quite
common use in London and Paris, it had not yet penetrated
to Normandy.
"I have made you a small pot of coffee, cousin," said

she. "My brother learned to like it in London, and though I
do not approve of its constant use, yet tempered with
cream it is refreshing and wholesome when one is weak or
tired. Now I shall leave you to wait upon yourselves, and do

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Valvular Heart Disease: A Guide for

Cardiovascular Nurses and Allied

Chest X-ray Interpretation for Radiographers, Nurses

The Ophthalmic Study Guide For Nurses and Health

Each Woman’s Menopause: An Evidence Based Resource: For

Elsevier's 2024 Intravenous Medications: A Handbook for

Self Care for Allied Health Professionals From

Telepsychiatry and Health Technologies A Guide for

Braunwald's Heart Disease - Part 2 - A Textbook of

Gahart's 2021 Intravenous Medications: A Handbook for

Valvular Heart Disease

ISBN 978-3-030-86232-9 ISBN 978-3-030-86233-6 (eBook)

Part I Understanding Valvular Heart Disease

Part II Valvular Heart Disease Program Structure

Part III Assessing Valvular Heart Disease

Part IV Valvular Heart Disease Treatment Options

9 Transcatheter Treatment Options for Acquired Valvular

Part V Nursing Care for Patients with Valvular Heart Disease

Valvular Heart Disease

Experts Needed: Opportunities for Clinical Leadership

A Unique Resource for a New Field of Practice

A Team of International “Practice-Close” Leaders

In assembling our team of co-authors, we purposefully sought the collaboration of

Britt Borregaard, PhD works as a Clinical Nurse and an Associate Professor at

Sarah E. Clarke, DNP, ACNP-BC is a Senior Principal Program Development

Kimberly Guibone, DNP, ACNP-BC, FACC is the Structural Heart Clinical

Marian C. Hawkey, RN has an extensive nursing background in cardiac and criti-

Dmitry B. Levin, BA is the Associate Director of the Center of CardioVascular

Jennifer Munoz, MSN, APN-C received her Bachelor of Science Degree in

Casey Panebianco, DNP, APN-C has been a board-certified Advanced Practice

Elizabeth M. Perpetua, DNP, ANP-BC, ACNP-BC, FACC is a first-generation

Martina Kelly Speight, MSN, RN, FNP is a board-certified Nurse Practitioner in

1. Provide a broad overview of the population of patients with acquired valvular

1.2  hallenges Associated with the Care of Patients

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 3

Stage Definition Description

A At risk Patients with risk factors for development of VHD

B Progressive Patients with progressive VHD (mild to moderate severity and

C1: Asymptomatic patients with severe VHD in whom the LV

C2: Asymptomatic patients with severe VHD with

D Symptomatic Patients who have developed symptoms as a result of VHD

decisions. Shared-decision making is an essential component of the overall decision-­

Low-Risk SAVR Low-Risk Surgical Mitral Valve

pathways for patients include surgical intervention, transcatheter intervention, and

1.3 Acquired Valvular Heart Disease Pathologies

1.3.1 Aortic Stenosis

Surgical Mitral Valve Repair

 Prior mediastinal radiation  Aorto-iliac occlusive  Prior sternotomy  Multivalve disease

There is usually a prolonged asymptomatic phase followed by the development

1.3.1.1 Age and Gender

1.3.1.2 Etiology and Risk Factors

1.3.1.3 Comorbid Conditions

1.3.2 Aortic Regurgitation

Aortic regurgitation (AR) is characterized by regurgitation of blood from the aorta

1.3.2.1 Age and Gender

1.3.2.2 Etiology and Risk Factors

1.3.2.3 Comorbid Conditions

1.3.3 Mitral Regurgitation

Mitral regurgitation may be chronic or acute. Primary, or degenerative, mitral regur-

Mitral regurgitation (MR) is characterized by a retrograde flow of blood from the

1.3.3.1 Age and Gender

1.3.3.2 Etiology and Risk Factors

1.3.3.3 Comorbid Conditions

Part I Understanding Valvular Heart Disease

Part II Valvular Heart Disease Program Structure

Part III Assessing Valvular Heart Disease

Part IV Valvular Heart Disease Treatment Options

9 Transcatheter Treatment Options for Acquired Valvular

Part V Nursing Care for Patients with Valvular Heart Disease

Valvular Heart Disease

Experts Needed: Opportunities for Clinical Leadership

A Unique Resource for a New Field of Practice

A Team of International “Practice-Close” Leaders

1.2 hallenges Associated with the Care of Patients

decisions. Shared-decision making is an essential component of the overall decision-

1.3 Acquired Valvular Heart Disease Pathologies

1.3.1 Aortic Stenosis

Prior mediastinal radiation Aorto-iliac occlusive Prior sternotomy Multivalve disease

1.3.1.1 Age and Gender

1.3.1.2 Etiology and Risk Factors

1.3.1.3 Comorbid Conditions

1.3.2 Aortic Regurgitation

1.3.2.1 Age and Gender

1.3.2.2 Etiology and Risk Factors

1.3.2.3 Comorbid Conditions

1.3.3 Mitral Regurgitation

1.3.3.1 Age and Gender

1.3.3.2 Etiology and Risk Factors

1.3.3.3 Comorbid Conditions

1.3.4 Mitral Stenosis

1.3.4.1 Age and Gender

1.3.4.2 Etiology and Risk Factors

1.3.4.3 Comorbid Conditions

1.3.5 Tricuspid Regurgitation

1.3.5.1 Age and Gender

1.3.5.2 Etiology and Risk Factors

1.3.5.3 Comorbid Conditions

1.4 Key Takeaways

2.3 Anatomy and Physiology

2.3.1 Normal Anatomy

2.3.1.1 Semilunar Valves

2.3.1.2 Atrioventricular Valves

2.3.2 Normal Physiology

2.4 Etiologies and Pathogenesis of Acquired VHD

2.4.1 Rheumatic heart disease

2.4.2 Non-rheumatic Valve Disease

2.4.2.1 Infective Endocarditis

2.4.2.2 Degenerative Valve Disease

2.4.2.3 Calcific Valve Disease

2.5 Aortic Valve Disease

2.5.1 Aortic Stenosis

2.5.1.1 Etiology and Pathoanatomy

2.5.1.3 Clinical Manifestations