Valvular Heart Disease and

the Cardiac Exam

2009
 Overview
 Clinical syndromes
 Overview of cardiac murmurs and maneuvers
 Left sided valvular lesions
– Aortic stenosis and sclerosis
– Mitral stenosis
 Rheumatic fever prophylaxis
– Acute and chronic aortic regurgitation
– Acute and chronic mitral regurgitation
 Right sided valvular lesions
– Tricuspid valve disease
 Prosthetic valves
 Endocarditis prophylaxis
 Questions
 General Appearance
 Marfan Syndrome
– Tall, long extremities
– Associated with: aortic root
dilitation, MV prolapse
 Acromegaly
– Large stature, coarse facial
features, “spade” hands
– Associated with: Cardiac
hypertrophy
 Turner Syndrome
– Web neck, hypertelorism,
short stature
– Associated with: Aortic
coarctation, pulmonary
stenosis
 Pickwickian Syndrome
– Severe obesity, somnolence
– Associated with: Pulmonary
hypertension
 Fredreich ataxia
– Lurching gait, hammertoe, pes
cavus
– Associated with: hypertrophic
cardiomyopathy
 Duchenne type muscular
dystrophy
– Pseudohypertrophy of the
calves
– Cardiomyopathy
 Ankylosing spondylitis
– Straight back syndrome, stiff
(“poker”) spine
– Associated with: AI, CHB
(rare)
 Lentigines (LEOPARD
syndrome)
– Brown skin macules that do
not increase with sunlight
– Associated with: HOCM, PS
“Spade” hands in acromegaly
 General Appearance- 2
 Hereditary hemorrhagic
telangiectasia (Osler-
Weber-Rendu)
– Small capillary hemangiomas
on the face or mouth
– Associated with: Pulmonary
arteriovenous fistula
 Lupus
– Butterfly rash on face,
Raynaud phenomenon- hands,
Livedo reticularis
– Associated with: Verrucous
endocarditis, Myocarditis,
Pericarditis
 Pheochromocytoma
– Pale diaphoretic skin,
neurofibromatosis- café-au-lait
spots
– Associated with:
Catecholamine-induced
secondary dilated CM
 Sarcoidosis
– Cutaneous nodules, erythema
nodosum
– Associated with: Secondary
cardiomyopathy, heart block
 Tuberous Sclerosis
– Angiofibromas (face; adenoma
sebaceum)
– Associated with:
Rhabdomyoma
 Myxedema
– Coarse, dry skin, thinning of
lateral eyebrows, hoarseness
of voice
– Associated with: Pericardial
effusion, LV dysfunction
 Grading the Intensity of Cardiac
Murmurs
 Grade 1
– Murmur heard with stethoscope, but not at first
 Grade 2
– Faint murmur heard with stethoscope on chest wall
 Grade 3
– Murmur hears with stethoscope on chest wall, louder than grade
2 but without a thrill
 Grade 4
– Murmur associated with a thrill
 Grade 5
– Murmur heard with just the rim held against the chest
 Grade 6
– Murmur heard with the stethoscope held away and in from the
chest wall
 Cardiac Murmurs

 Most mid systolic murmurs of grade 2/6 intensity

or less are benign
– Associated with physiologic increases in blood
velocity:
 Pregnancy
 Elderly
 In contrast, the following murmurs are usually
pathologic:
– Systolic murmurs grade 3/6 or greater in intensity
– Continuous murmurs
– Any diastolic murmur
Maneuver
Normal respiration
Passive leg elevation
Stand to squat
Squat to stand
Valsalva
Isometric handgrip exercise
Inhaled amyl nitrate
Hemodynamic Effect
Transient ↑ in venous filling
during inspiration
↑ venous return (transient
↑ in LV size and preload)
↑ venous return (transient
↑ in LV size and preload)
↓ venous return (transient ↓
in LV size and preload)
↓ venous return (transient ↓
in LV size, preload, and
relative systemic
hypotension)
↑ afterload
↓ afterload
Murmur Effect
↑ right-sided murmurs
↑ right-sided murmurs,
↓murmur of HOCM and MVP
↑ right-sided murmurs,
↓murmur of HOCM and MVP
↑ murmur of HOCM, moves
midsystolic click of MVP
closer to S1 and ↑ MVP
murmur, ↓ AS murmur
↑ murmur of HOCM, moves
midsystolic click of MVP
closer to S1, and ↓ murmur
of MVP
↑ murmur of MR and VSD,
↓the murmur of HOCM, ↓AS
murmur
↓ murmur of MR and VSD,
no change in AS murmur
 Diagnostic Testing
 ECHOCARDIOGRAM
 Exercise testing
– To assess the clinical severity of valvular heart disease
 Those with inconsistent resting hemodynamics
 Equivocal history of symptoms
– Exercise testing in AS patients
 Should be ended promptly if:
– Cardiac symptoms provoked
– Decrease or minimal increase (<20 mmHg) in blood pressure
 Prior history of angina, congestive heart failure, or exertional
syncope absolute contraindications to exercise testing
 Cardiac catheterization
– Usually not needed for primary evaluation
 Aortic Stenosis
 Most common cause is calcific degeneration
– Active disease process with risk factors of male sex, smoking,
HTN, DM, older age, hypercholesterolemia
 2% of the general population have bicuspid aortic valves
– Symptomatic or severe AS occurs earlier (age 40-60 years)
 AS less commonly from rheumatic heart disease
valvulitis
– Invariably MV involved first
– Associated AV involvement in <1/2 patients
 AV sclerosis
– Valve thickening without obstruction
– Present in >20% of people >65 years
– Associated with 50% increased risk of MI and CV death
 Progression of Aortic Sclerosis

 Hemodynamic progression usually slow

– Average rate of increase in aortic jet velocity of 0.3
m/s per year
– Increase in mean transaortic gradient of 7 mmHg
– Decrease in AVA of 0.1 cm2 per year
 Severe AS
– Aortic jet velocity > 4 m/s
– Mean transvalvular pressure gradient > 50 mmHg
– AVA < 1.0 cm2
 Pathophysiology of Aortic Stenosis

 Obstruction of LV outflow increases intracavitary

systolic pressures and leads to LV pressure
overload
 Initial compensatory mechanism is myocardial
hypertrophy with preservation of systolic
function
 Diastolic function impaired as a consequence of
increased wall thickness and abnormal
myocardial relaxation
 Increased wall stress and afterload causes
eventual decrease in ejection fraction
 Pseudostenosis
 Occurs in patients with impaired systolic function
and aortic stenosis
– Unable to generate transvalvular gradient
 Careful diagnostic testing with dobutamine
infusion protocols can aid in differentiating
between true AS and pseudostenosis
 If the calculated AVA increases with
augmentation of cardiac output, then
pseudostenosis present
 If AVA does not increase with dobutamine, then
obstruction fixed and true AS present
Clinical Presentation of Aortic Stenosis
 Cardinal symptoms:
– Angina
 Occurs in >50% of patients, not sensitive due to prevalence of CAD
– Syncope
– CHF
 Sudden cardiac death rare, <1% per year
 In earlier stages, AS presentation more subtle
– Dyspnea
– Decreased exercise tolerance
 Rarely, AS diagnosed in the setting of GI bleeding
– Heyde’s syndrome
 Bleeding caused by AVM
 Concurrent AS occurs at prevalence rate of 15-25%
 Associated with an acquired von Willebrand syndrome due to
disruption of vW multimers through a diseased AV
 Management of Aortic Stenosis
 Prognosis in asymptomatic disease excellent
 Conservative approach with monitoring for symptoms
recommended
 When severe stenosis present-
– 38% of asymptomatic patients develop symptoms within 2 years
– 79% are symptomatic within 3 years
 Once symptoms occur, AVR needed
 LV dysfunction and severe AS have increased
perioperative mortality with AVR
– But outcomes still favorable with surgery
 Nitroprusside may transiently improve cardiac function
as a bridge to valve replacement
– Does not supplant AVR in symptomatic patients
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
 Aortic Valve Replacement
 Prophylatic AVR in asymptomatic patients not routinely
performed due to surgical risks
– Thromboembolism, bleeding associated with anticoagulation,
prosthetic valve dysfunction, and endocarditis
– Occurs at a rate of 2-3% annually
– Only should be considered:
 If other cardiac surgery (such as CABG) planned
 Severe LVH or systolic dysfunction
 Women contemplating pregnancy
 Patients remote from health care
 Surgical valve replacement with operative morbidity and
mortality of 10%
 Percutaneous balloon aortic valvotomy rarely used
 Mitral Stenosis
 Usually associated with history of
rheumatic fever
 >40% of cases of RHD result in mitral
stenosis
– Women affected more than men (2:1)
 Presentation 20-40 years after the initial
episode of rheumatic fever
– If infected at a young age, latent period is a
few years
 Clinical Presentation of Mitral Stenosis
 Significant MS leads to ↑LA pressure and pulm HTN
 Symptoms include dyspnea with ↑ cardiac demand
– Exercise
– Pregnancy
 Survival excellent with asymptomatic or minimally
symptomatic patients
– >80% survival at 10 years
 Survival in symptomatic patients much worse
– 10 year survival drops to 15% or lower (if pulm HTN present)
 Findings consistent with severe MS:
– Transvalvular diastolic pressure gradient >10 mmHg
– MVA <1.0 cm2
– Severe pulmonary hypertension (>60 mmHg)
 Management of Mitral Stenosis

 Atrial fibrillation
– Prevalence >30% in symptomatic patients and
associated with poorer long term outcome
– Warfarin indicated:
 In patients with AF and MS
 Patients without history of AF but with MS and embolic CVA
– In patients with prior history of AF who have mitral
valve surgery, decreased postoperative AF observed if
MAZE performed concominantly
 Mitral Valve Repair
 Percutaneous valvotomy
– Therapeutic intervention of choice if:
 LAA thrombus excluded
 MR less than moderate
 Valvular characteristics favorable
– Pliable leaflets, minimal commisural fusion, minimal valvular or
subvalvular calcification
– Pulmonary HTN not contraindication to valvotomy
– Major complications include: severe MR (1-8%),
systemic embolization (1-3%), and tamponade (1-
2%)
 Periprocedural mortality- 1%
 Surgical commissurotomy or MVR can be
performed in unfavorable anatomy
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
 Rheumatic Fever Prophylaxis
 Primary prophylaxis
– If living in an endemic area, with pharyngitis and a
+test for group A strep or positive throat culture
– Given once, may be repeated as needed:
 PCN G 1.2 million U IM or PCN V 500 mg TID x 10d
 Azithromycin 500 mg on day 1, 250 mg daily for 4d

 Secondary prophylaxis
– PCN G 1.2 million units IM every 4 weeks or PCN V
250 mg PO BID or erythromycin 250 mg BID
 RHD without carditis- At least 5 years or until >21 y of
age
 RHD with carditis, no valvular HD- At least 10 y or well
into adulthood
 RHD with carditis and valvular HD- At least 10 years
from last episode or until patient is older than 40 years
 Acute Aortic Regurgitation
 Causes of acute aortic regurgitation:
– Aortic dissection
– Valve distruction from endocarditis
– Traumatic rupture
 Classic physical exam findings may be absent in the
acute presentation
– Diastolic murmur may not be present due to sudden increase of
LVEDP
 TTE, along with TEE, cath, CT or MRI may be used for
diagnosis
 Surgical AV repair or replacement should be performed
emergently
 Afterload reducing medications and inotropes may help
to acutely stabilize the patient
 IABP contraindicated
 Acute Mitral Regurgitation
 Most often occurs in:
– Chordae tendineae rupture due to myxomatous valve
disease or endocarditis
– Myocardial infarction with papillary muscle
dysfunction or rupture
 Symptoms almost always occur
– Dyspnea and pulmonary edema
 Systolic function may occur normal or
hyperdynamic
 IABP or afterload reducing drugs to temporize
 Surgical intervention for treatment
 Chronic Valvular Regurgitation
 Cardiac chamber size and function have time to
compensate for dysfunction
– May allow patients to remain asymptomatic for a long time
 Both preload and afterload increases
 Once increase in cardiac output insufficient→ systolic
function declines → pulmonary HTN may develop and
symptoms develop
 LV enlargement and progressive systolic dysfunction are
associated with significant morbidity and mortality
 Serial echocardiography and evaluation by a cardiologist
is indicated
 Chronic Aortic Regurgitation
 Occurs most often in bicuspid AV
 Other causes include ascending aortic aneurysm and Marfan’s
Disease
 Risk factors for poorer outcome:
– Age
– Cardiac symptoms
– Atrial fibrillation
– LV enlargement
– Lower LVEF
 Asymptomatic patients with normal LV size and function do not
require prophylatic surgery
 Surgery should be considered if:
– LVESD > 55 mm
– Ejection fraction <60%
– Symptoms develop
 Oral afterload reduction (nifedipine or ACE-I) may slow rate of LV
dilation
Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51
 Chronic Mitral Regurgitation

 Often caused by myxomatous disease or MVP

– Myxomatous mitral valve disease with progressive MR
associated with poor long term outcome
 Higher risk of arrhythmias and sudden cardiac death
– Mitral valve prolapse occurs in ~2% of the general
population
 Consists of the buckling of the mid portion of the valve
leaflets into the LA
 Usually asymptomatic, but may be associated with
palpitations or chest discomfort
 Prognosis usually benign
 Antibiotic prophylaxis now not indicated
 Chronic Mitral Regurgitation
 Other causes include secondary or acquired
leaflet dysfunction
– Endocarditis
– Rheumatic heart disease
– Annular tethering from LV dilation
– Tethering of the chordal apparatus from ischemic
heart disease
– Rare cause: Fenfluramine and phentermine, also
associated with AI
 Compensatory increase in LV chamber size
initially allows for increase in total stroke volume
and restoration or total forward cardiac output
 Treatment of Chronic Mitral
Regurgitation
 Mitral valve repair preferred over mitral valve
replacement
– Avoids risk of anticoagulation
– Preservation of subvalvular apparatus
 Better postoperative LV function and long term survival
 When MR occurs in volume overloaded states,
afterload reduction can be beneficial
– Dilated CM
– CAD
 Revascularization may improve dysfunction of
the papillary muscle
 Biventricular pacing may improve LV geometry
 Timing of Intervention for Left-Sided Valvular Conditions
Aortic Stenosis
Intervention:
AVR
IF:
Patient is symptomatic
(NYHA class II or greater,
angina due to AS, or
syncope)
OR
Patient has symptomatic
severe AS and needs other
cardiothoracic surgery (i.e.
CABG)
OTHERWISE
Depending on the severity
of AS, at least annual
clinical evaluation with TTE
to monitor for symptom
onset
Mitral Stenosis
Intervention:
Percutaneous valvotomy if
anatomy amenable and
<moderate MR, and no
LAA clot. Otherwise, open
commissurotomy or MVR
IF:
Patient has moderate or
more severe MS (MVA <
1.5 cm2)
OR
Pulmonary hypertrension at
rest (PAP > 60 mmHg)
OR
Abnormal hemodynamic
response to exercise:
PAP > 60 mmHg
Mean gradient > 15 mmHg
OTHERWISE
Clinical evaluation at least
annually, depending on the
severity of the mitral
stenosis
Chronic Severe AR Chronic Severe MR
Intervention: Intervention:
Surgical AVR with aortic Surgical mitral valve repair
root replacement if needed if anatomy amenable.
Otherwise, MVR
IF: IF:
Patient is symptomatic Patient is symptomatic
(NYHA class II or greater) (NYHA class II or greater)
OR OR
EF <60% EF <60%
OR OR
ESD > 55 mm ESD > 45 mm
OR OR
Abnormal hemodynamic Pulmonary hypertension or
response to exercise atrial fibrillation
PAP increase by 25 mmHg
OTHERWISE OTHERWISE
Repeat TTE at least yearly, Repeat TTE yearly, repeat
repeat clinical evaluation at clinical evaluation
least biannually depending biannually
on the severity of the LV
dilitiation
 Tricuspid Valve Disease
 Tricuspid stenosis is rare
– Associated with rheumatic heart disease
 TR usually occurs secondary to:
– Pulmonary hypertension
– RV chamber enlargement with annular dilatation
– Endocarditis (associated with IV drug use)
– Injury following pacer lead placement
 Other secondary causes: carcinoid, radiation therapy,
anorectic drug use, and trauma
 Primary causes: Marfan’s syndrome and congenital
disorders such as Ebstein’s anomaly and AV canal
malformation
 Echo is diagnostic in most cases
 Tricuspid Regurgitation
 Severe tricuspid regurgitation is difficult to treat
and carries a poor overall clinical outcome
 Symptoms are manifestations of systemic
venous congestion
– Ascites
– Pedal edema
 Surgical intervention usually considered if other
cardiac surgery planned
 Surgical options include valvular annuloplasty or
replacement
– If replacement planned, bioprosthetic valve preferred
 Prosthetic Valves- Mechanical
 Three types:
– Ball-cage valve
– Single tilting disk valve
– Bileaflet valve
 Durable but require life long anticoagulation
 For operative procedures, warfarin typically is
discontinued for 48-72 hours and restarted
postop as soon as possible, except for:
– Mechanical mitral prosthesis
– Atrial fibrillation
– Prior thromboembolic events
Ball-cage valve

Single tilting disk valve

Bileaflet valve
 Prosthetic Valves- Biological
 Biological Valves
– Composed of autologous or xenograft biological
material mounted on stents and a sewing ring
– Warfarin therapy not required due to lower
thromboembolic potential
– Valve durability less when compared to mechanical
valves
– Newer stentless valves with increased longevity
Anticoagulation Guidelines for
Mechanical Valves

Bonow et al. J Am Coll Cardiol 2006; 47: 2141-51

 Prosthetic Valve Complications
 Common complications include:
– Structural valve deterioration
– Valve thrombosis
– Embolism
– Bleeding
– Endocarditis
 Endocarditis prophylaxis required for patients with all types of
prosthetic valves
 Suspect valve dehiscence or dysfunction in:
– Acute CHF in the immediate postop period
– New cardiac symptoms
– Embolic phenomena
– Hemolytic anemia
– New murmurs
 TEE is the diagnostic procedure of choice
 Postop TTE should be done 2-3 months after surgery
 Valve Thrombosis
 Incidence with mechanical prosthesis of 2-4 % per year
 Suspect in patients with new murmur, change in
cardiopulmonary symptoms, or an embolic event
 Diagnosis based on clinical presentation, TTE/TEE, and
fluroscopy
 In small thrombus, treatment with heparin may be
adequate
 Optimal treatment for left sided thrombosis is emergency
surgery
 Consider thrombolytic therapy for right sided thrombosis
or if surgery cannot be performed with left sided disease
Endocarditis Prophylaxis

2007 AHA Prevention of Infective Endocarditis Guidelines