Cardiomyopathies Definition • Primary disease of the myocardium, excluding myocardial dysfunction due to ischemia, chronic valvular disease, hypertensive heart disease and pulmonary heart disease. • Diagnosis by exclusion.
Cardiomyopathies Physiologic Classification • Dilated • Hypertrophic • Restrictive
Cardiomyopathies Echocardiographic Evaluation • Confirm the diagnoses and type. • Define the physiologic consequences.
Dilated Cardiomyopathies Dilated Cardiomyopathy • Characterized by four chamber enlargement. • Impaired systolic function of both ventricles
Dilated Cardiomyopathies Etiology • Idiopathic • Toxins • Metabolic • Peripartum
Dilated Cardiomyopathies Etiology • Infections • Systemic Disease • Inherited disorders
Dilated Cardiomyopathies Toxins • Alcohol • Medications • Cobalt • Snake bite
Dilated Cardiomyopathies Metabolic • Thiamine deficiency • Acromegaly
Dilated Cardiomyopathies Infections • Chagas’ disease • Post viral
Dilated Cardiomyopathies Physiology • Impaired contractility of the LV • Reduced CO • Elevated LV-EDP.
Dilated Cardiomyopathies Symptoms • Low cardiac output • Pulmonary venous congestion • Systemic venous congestion
Dilated Cardiomyopathies Diastolic Dysfunction • LV diastolic dysfunction often coexists with systolic dysfunction but typically is not the predominant feature.
Dilated Cardiomyopathies 2-D Echocardiographic Findings • Enlargement of the 4 chambers • Global decreased in wall motion
Dilated Cardiomyopathies M-mode findings • Increased EPSS • Reduced anteroposterior aortic root motion • B-bump on the AC shoulder
Dilated Cardiomyopathies Doppler • Reduced aortic ejection velocity • Reduced aortic VTI • A slow rate of rise in the velocity of the MR jet (reduced rate of rise in LV pressure in early systole)
Dilated Cardiomyopathies Doppler • Mitral and tricupid regurgitation
Dilated Cardiomyopathies Diastolic Filling Pattern Early - Impaired Relaxation • Prolonged IVRT • Reduced E • Increased A
Dilated Cardiomyopathies Further deterioration in LV function • Increased E velocity (high LA pressure) • Reduced A velocity (elevated LV-EDP) • Pseudonormalization
Dilated Cardiomyopathies LA Filling Pattern • Increased atrial reversal velocity • Increased ratio of antegrade diastolic to systolic flow.
Cardiomyopathies Hypertrophic Cardiomyopathy
Hypertrophic Cardiomyopathies Hypertrophic Cardiomyopathy • Autosomal dominant • Abnormalities in the Beta myosin heavy chain.
Hypertrophic Cardiomyopathies Predominant Features • Asymmetric hypertrophy of the LV • Normal ventricular systolic function • Impaired LV diastolic function • +/- Subaortic dynamic obstruction
Hypertrophic Cardiomyopathies Clinical Features • High risk of sudden death • Symptoms of angina • Exercise intolerance • Syncope • Systolic murmur
Hypertrophic Cardiomyopathies • The apical pattern of hypertrophy is associated with an inverted precordial T waves on the EKG
Hypertrophic Cardiomyopathies The common feature of all of these hypertrophy patterns is normal thickness of the basal posterior LV wall.
Hypertrophic Cardiomyopathies Subaortic obstruction • Caused by apposition of AMVL against the hypertrophied septum. • Dynamic obstruction • Occurs in mid – late systole
Hypertrophic Cardiomyopathies Subaortic obstruction • Maximum LV to aortic pressure gradient occurring in late systole. • Presence and severity of obstruction depends on the loading conditions.
Hypertrophic Cardiomyopathies The obstruction can diminished by maneuvers that: • Increase ventricular volume (increase in preload). • Decrease contractility. • Increase afterload.
Hypertrophic Cardiomyopathies The obstruction can be increased by maneuvers that: • Decrease ventricular volume (decrease in preload) • Increase contractility • Decrease afterload
Hypertrophic Cardiomyopathies Clinical useful maneuvers • Examination during a post premature contraction beat (Increased contraction). • Valsalva (Decreased preload). • Inhalation of amyl nitrate (Decreased afterload and preload)
Hypertrophic Cardiomyopathies These maneuvers lead to • Increased degree of dynamic obstruction • Louder murmur • Increased in Doppler velocity.
Hypertrophic Cardiomyopathies Mitral Regurgitation • The SAM of the leaflets in systole disrupt the normal mitral valve coaptation. • Results in a posteriorly directed MR jet of mild to moderate severity.