cardiomyopathies

By Okash BSN Student

Learning objectives

at the end of this lesson the student will be

able to:
1. Define Cardiomyopathy.
2. List the etiologies of Cardiomyopathies.
3. Describe
the different types of
Cardiomyopathies.
4. Describe the pathogenesis of Cardiomyopathies.
5. Identify the clinical manifestation of Cardiomyopathies.
6. Understand the diagnostic approach of Cardiomyopathies.
7. Understand the management of patients Cardiomyopathies
Definition

 Cardiomyopathies are a group of diseases that

affect the myocardium and are not the result of
hypertension, Valvular, coronary or pericardial
abnormalities.
 Refers to diseases of the heart muscle, where the
walls of the heart chambers become stretched,
thickened or stiff limiting the heart's ability to
pump blood
Etiology

1. Primary cardiomyopathies
 Idiopathic
 Familial
 Eosinophilic endomyocardial diseases
 Endomyocardial Fibrosis
Secondary myocardial involvement
 InfectiveMyocarditis: Viral , bacterial , fungal , protozoal ,
Metazoal , Spirochetal
 Metabolic : Thyrotoxicosis
 Familial
storage diseases: Glycogen storage diseases ,
Hemochromatosis ,
 Deficiencies : Electrolyte , Nutritional ( Vitamin B1
deficiency )
 Connective tissue diseases : SLE , polyarteritis nodosa ,
Rheumatoid arthritis , Systemic sclerosis
 Infiltrationand granulomas ; Amyloidosis ,
sarcoidosis , malignancies
 Neuromuscular : muscular dystrophies ,myotonic
dystrophies
 Sensitivity and toxic reactions :Alcohol , drugs ,
radiation
 Congenital heart diseases
TYPES

 Dilated cardiomyopathy
 Hypertrophic cardiomyopathy
 Restrictive cardiomyopathy
1. DILATED CARDIOMYOPATHIES

 Dilatationand impaired contraction of the left

or both ventricles.
 Left ventricular mass is increased but wall
thickness is normal or reduced
 It develops at any age though common in adults
 More common in males than females
 It occurs in people of any ethnic origin
Etiology of DCM

 Caused by familial/genetic
 viral and/or Autoimmune
 alcoholic/toxic
 or unknown factors (idiopathic)
 secondary to pre-existing cardiovascular
disease.
Morphological features

 Theheart is enlarged and increased in

weight
 Dilatationof all the four chambers making
the heart look globular
 Thin walls of the heart
 Microscopically
there is myocyte loss with
replacement fibrosis in the ventricle
Pathophysiology of dilated
cardiomyopathy
 Dilated cardiomyopathy represents the final
common morphologic outcome of a variety of
biological insults. It is a combination of myocyte
apoptosis and necrosis with increased myocardial
fibrosis, producing reduced mechanical function.
Many causes are a result of direct toxicity (e.g.,
alcohol) or mechanical insults (e.g., chronic
volume overload in mitral valvular regurgitation),
infection (eg myocarditis).
Clinical manifestations

 Syncope may result from arrhythmias

 Systemic embolization, often emanating from
ventricular thrombus, may occur.
 Hypotension , tachycardia
 Cardiomegaly
 Functional mitral or tricuspid regurgitation.
 Findings of CHF : raised JVP and S3 and S4 gallops
Diagnostic work up for DCM

 Chest radiograph: Cardiomegaly and evidence of

pulmonary congestion
 ECG: sinus tachycardia or atrial fibrillation,
ventricular arrhythmias, St segment and T wave
abnormalities
 Echocardiogram: Left ventricular dilatation and
dysfunction (ejection fraction < 40 %)
Hypertrophic Cardiomyopathy

Definition
 Hypertrophic cardiomyopathy is characterized by
left ventricular hypertrophy (myocardial thickness
greater than 1.5 cm), typically of none dilated
chamber, without obvious causes.
 Other etiologies of left ventricular hypertrophy,
such as long-standing hypertension and aortic
stenosis need to be excluded before one can
diagnose HCM.
Genetic Predisposition:
 HCM is the most common genetic cardiovascular
disease. About half of patients with HCM have a
positive family history with autosomal dominant
transmission.
 The prevalence in the general adult population
for people with phenotypic evidence of HCM is
estimated at 1 per 500
Pathophysiology of HCM

 Generally, ventricular hypertrophy involves the proximal

portion of the interventricular septum. As the septum
thickens, it may narrow the outflow tract.
 In addition, systolic anterior motion of the mitral valve
may occur and result in left ventricular outflow tract
obstruction and mitral regurgitation.
 When systolic anterior motion occurs, the mitral valve
leaflets are pulled or dragged anteriorly toward the
ventricular septum, producing the obstruction
 Consequently, the left ventricle has to generate
much higher pressures to overcome the out flow
obstruction and to pump blood to the systemic
circulation.
 Premature closure of the aortic valve may occur
and is caused by the decline in pressure distal to
the left ventricular outflow obstruction
Clinical presentation

 The most common symptom of HCM is dyspnea on

exertion
 Syncope or near syncope
 Palpitations
 chest pain with exertion,
 Bisferiens pulse: rapidly rising carotid pulse
followed by a collapse in the pulse and then a
secondary rise.
 Systolic murmurs.
 Unfortunately, the first clinical manifestation of the
diseases may be sudden cardiac death frequently
occurring in young children and young adults. ,
often during or after physical exertion. HCM is the
most common etiology for sudden cardiac death.
Diagnostic work up for HCM
 Echocardiography is the gold standard for diagnosing
HCM. On transthoracic echocardiography, note the
thickness of the septum; location and pattern of
hypertrophy; site and degree of left ventricular
outflow tract obstruction; presence of systolic anterior
motion of the mitral valve; presence of premature
closure of the aortic valve; and any change in severity
of obstruction with amyl nitrite
 Electrocardiogram often shows left ventricular
hypertrophy.
Restrictive Cardiomyopathy

 Restrictive cardiomyopathy is a disease of the

myocardium that is characterized by restrictive
filling and reduced diastolic volume of either or
both ventricles with normal or near-normal
systolic function.
 The heart muscle is stiff or scarred, or both
 Ventricular filling is impaired because the
ventricles are ‘stiff
Subtypes of RCM

1. Endomyocardial fibrosis – has fibrosis in

the subendocardium.
2. Endocardial fibroelastosis – affects
children, the cardiac chambers are lined
by a diffuse layer of dense white fibrous
tissue, composed mainly of elastic fibres.
Etiology

 Idiopathic
 Familial,
 Systemicdisorders e.g. amyloidosis,
sarcoidosis, carcinoid heart disease,
scleroderma, and anthracycline toxicity
Pathophysiology

 These condition result in impaired ventricular

filling and primarily diastolic heart failure. They
present with a clinical heart failure syndrome that
is frequently indistinguishable from that caused by
systolic dysfunction.
 Atrialfibrillation is poorly tolerated. It simulates
other right side heart failure like cor-pulmanale
and diastolic dysfunction of constricted
pericarditis.
Clinical Features
 Exercise
intolerance and dyspnea are the prominent
symptoms.
 Peripheral edema with predominant ascites
 Enlarged tender and pulsatile liver.
 JVP is elevated and it doesn’t fall normally during
inspiration ( Kussmaul’s sign )
 The heart sounds may be distant but apical impulse is
easily palpable unlike in constrictive pericarditis.
Diagnostic work up for CCM

 Chest x-ray : mild cardiac enlargement

 ECG: low voltage and conduction defects
 Echocardiography: Increases left ventricular
wall thickness, normal or mildly reduced
systolic function
MANAGEMENT OF CARDIOMYOPATHIES

Dilated cardiomyopathy:
 Standard therapy of heart failure
 Cardiactransplantation provides a median
10-year survival and is effective palliation
in appropriately selected individuals
Hypertrophic cardiomyopathy:
 Competitive sport and probably strenuous exercise
should be avoided
 Dehydration should be avoided and diuretics should
be used with caution.
 ß-blockersare considered first-line therapy while
the second line therapy is Calcium channel blockers
 Digoxin should be avoided in HCM patients,
particularly in those with resting or latent
obstruction, because of its positive inotropic effect.
 Patients
with HCM should receive
prophylactic antibiotics for endocarditis
prevention before dental or invasive
procedures.
 PREVENTION: First degree relatives of
patients with HCM should be screened with
ECHO.
REFERENCES

 Harsh-Mohan-Textbook-of-Pathology-6th-Edition (chapter
16)
 kaplan-pathology-lecture-notes-2016 (chapter 13 pg 111)
ISBN: 978-1-5062-0045-3
 USMLE step 1 pathology notes, cardiac pathology (pg 105)
 Kasper L., Braunwald E., Harrison’s principles of Internal
medicine, Cardiomyopathies 16th Edition
THE END