Cardiomyopathies

• Group of entities that affect the myocardium

• Controversial classifications
 Primary cardiomyopathy
WHO Classification
anatomy & physiology of the LV
1. Dilated
• Enlarged
• Systolic dysfunction
2. Hypertrophic
• Thickened
• Diastolic dysfunction
3. Restrictive
• Diastolic dysfunction
4. Arrhythmogenic RV dysplasia
• Fibrofatty replacement
5. Unclassified
• Fibroelastosis
• LV noncompaction

Circ 93:841, 1996

 Secondary Cardiomyopathy: Specific
Etiologies
• Ischemic Ischemic: thinned, scarred tissue

• Valvular
• Hypertensive
• Inflammatory
• Metabolic
• Inherited
• Toxic reactions
• Peripartum
Dilated (congestive) cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive (infiltrative) cardiomyopathy

 Dilated Cardiomyopathy

•Dilation and impaired contraction of ventricles:

•Reduced systolic function with or without heart failure
•Characterized by myocyte damage
•Multiple etiologies with similar resultant pathophysiology

•Majority of cases are idiopathic

•incidence of idiopathic dilated CM 5-8/100,000
•incidence likely higher due to mild, asymptomatic cases
•3X more prevalent among males and African-Americans
 DCM: Etiology
▪Ischemic
▪Valvular
▪Hypertensive
▪Familial
▪Idiopathic
▪Inflammatory
Infectious
Viral – picornovirus, Cox B, CMV, HIV
Ricketsial - Lyme Disease
Parasitic - Chagas’ Disease, Toxoplasmosis
Non-infectious
Collagen Vascular Disease (SLE, RA)
Peripartum
▪Toxic
Alcohol, Anthracyclins (adriamycin), Cocaine
▪Metabolic
Endocrine –thyroid dz, pheochromocytoma, DM, acromegaly,
▪Nutritional
Thiamine, selenium, carnitine
▪Neuromuscular (Duchene’s Muscular Dystrophy--x-linked)
 Clinical Findings
Biventricular Congestive Heart Failure

-Low forward Cardiac Output

-fatigue, lightheadedness, hypotension

-Pulmonary Congestion
-Dyspnea,
-orthopnea, & PND

-Systemic Congestion
-Edema
-Ascites
-Weight gain
 Physical Exam
Decreased C.O.
Tachycardia
↓ BP and pulse pressure
cool extremities (vasoconstriction)
Pulsus Alternans (end-stage)
Pulmonary venous congestion:
rales
pleural effusions
Cardiac:
laterally displaced apex
S3
mitral regurgitation murmur
Systemic congestion
↑ JVD
hepatomegaly
ascites
peripheral edema
 Diagnostic Studies

CXR -enlarged cardiac silhouette,

vascular redistribution interstitial edema,
pleural effusions
ECG –normal
tachycardia, atrial and ventricular
enlargement, LBBB, RBBB, Q-waves

Echocardiography
LV size, wall thickness and function
valve diseases
Cardiac Catheterization
hemodynamics
LVEF
angiography
Endomyocardial Biopsy
 Treatment of DCM

• Standard HF therapy
– ACE inhibitor
– B-blocker
– Diuretic
– Aldosterone antagonist
– Digoxin
– CCB should generally be avoided
– Na+ restriction helpful
 Hypertrophic CM

• Asymmetrical wall hypertrophy, mostly septal ,

lead to LV outflow tract is narrowed during
systole
 Hypertrophic CM

• HOCM is inherited as autosomal

dominant trait and is caused by mutations
in a number of genes
 Hypertrophic CM

• Most frequent symptoms are:

dyspnea
chest pain
syncope (often post-exertion)

• Ventricular arrhythmias are common and SCD

may occur, especially in athletes after extreme
exertion
Physical Exam of HCM

• Bisferiens carotid pulse

• triple apical impulse
• loud S4
• Ejection systolic murmur over left sternal
border
Diagnostic Studies HCM

• ECG - Left ventricular hypertrophy

• CXR - usually normal, ascending aorta is
dilated
• ECHO - diagnostic, reveals asymmetric
LVH, small & hypercontractile LV, thick
septum
Hypertrophic Cardiomyopathy
 Treatment of HCM

• Beta blockers are initial drugs of choice

• Slower heart rate allows more time for diastolic
filling
• CCB have also been effective
• Surgical myomectomy or alcohol ablation
 Restrictive CM

• Myocardial disease characterized by impaired diastolic

filling with preserved contractile function.
• Usually caused by myocardial infiltration or fibrosis
• Amyloidosis is most common
Amyloid infiltration
Clinical Findings of RCM

• Low cardiac output

• Pulmonary venous congestion
• Systemic venous congestion

• Must be distinguished from constrictive

pericarditis.
Treatment & Prognosis RCM

• Little useful therapy exists

• Diuretics can help
• Digoxin may worsen
• Cardiac transplantation
• Overall prognosis very poor