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Flashcards in Cardiomyopathies Deck (13)

Dilated Cardiomyopathy

Mostly familial (autosomal dominant) with globoid shape
All chambers are dilated with impairment of contractility
Hypertrophy of myocytes and fibrosis
EF is lower than 40%

Key words: dilated chambers, massive hypertrophy, and interstitial fibrosis


Causes of Dilated Cardiomyopathy

Toxicity: thiamine deficiency, cobalt cardiotoxicity, chemo drugs

Post myocarditis

Peripartum: occurs in the last month before delivery and post 5 months delivery

Iron overload aka iron heart that doesn’t pump very well from hemochromatosis and transfusions

Supraphysiologic stress: catecholamine induced, pheochromocytoma, cocaine, and takotsubo cardiomyopathy


Clinical Feature of Dilated Cardiomyopathy

20-50 years old
CHF, tricuspid/mitral valve regurgitation
Sudden cardiac death


Hypertrophic Cardiomyopathy

Asymmetric septal hypertrophy
Thick septum LV outflow tract obstruction
Diastolic dysfunction

Myofiber disarray is the buzzword – “bananas are going crazy”

Autosomal dominant mutations in sarcomeric protein genes


Clinical Features of Hypertrophic Cardiomyopathy

Can cause sudden cardiac death = need to detect this
Impaired L ventricular diastolic filling*
Harsh systolic ejection murmur


Arrthymogenic Right Ventricular Cardiomyopathy (ARVC)

Yellow of R ventricle = all fat; not just replaced by fat, but thinned and dilated so not functioning well at all

Systolic disorder of RV with recurrent VT and RV origin with focal RV adiposity

Desmosome protein deficiency in myocytes


Clinical Features of ARVC

More common in Italy mostly in males 15-50 years old
Naxos Syndrome: palmar and plantar keratosis with wooly hair

Clinical Presentation: syncope or sudden cardiac death from heart failure arrhythmia


Restrictive Cardiomyopathy

Diastolic disorder
Decrease in ventricular compliance, but normal LV size
Biatrial dilation with mural thrombi
Systolic function is often normal


Restrictive Cardiomyopathy Causes

Infiltrative Diseases:
Radiation fibrosis
Metastatic tumor

Loeffler endomyocarditis: associated with eosinophils and myeloproliferative disorders


Amyloid Restrictive Cardiomyopathy

Amyloid are proteins that are floating around in blood and deposit wherever
Firm, waxy texture with thick ventricles, and endocardial deposits

Buzz word: Apple green birefringence


Sarcoidosis Restrictive Cardiomyopathy

Big nodules
Microscopically is non-caseating granulomas within the myocardium and epicardial fat


Restrictive Cardiomyopathy: Endomyocardial Fibrosis and Fibroelastosis

Endomyocardial fibrosis in Africa and tropical regions – unknown causes of fibrosis (idiopathic)

Endocardial fibroelastosis: babies under 2 months of age; blue glistening color of the heart because cartilaginous material is present



Weeks to years after infective myocarditis
Pathogens: enterovirus, T. cruzi (Chaga’s disease = kissing bug with protozoan parasites), T. gondii (cat litter and pregnant women), and HIV

Lymphocytic= viral infections; show lymphocytic infiltrates