Cardiomyopathies and amyloidosis

Cardiomyopathies:Cardiomyopathies include a variety of myocardial disorders that manifest with various structural and functional phenotypes and are frequently genetic. Although some have defined cardiomyopathy to include myocardial disease caused by known cardiovascular causes (such as hypertension, ischemic heart disease, or valvular disease), current major society definitions of cardiomyopathy exclude heart disease secondary to such cardiovascular disorders.Physiologic classification:Cardiomyopathies are classified into the following categories based upon morphology and physiology: Dilated cardiomyopathy (DCM), Hypertrophic cardiomyopathy (HCM), Restrictive cardiomyopathy (RCM).Restrictive cardiomyopathy:Restrictive cardiomyopathy (RCM) is characterized by nondilated ventricles with impaired ventricular filling. Causes of RCM can be classified as familial noninfiltrative, infiltrative(amyloidosis), storage diseases, and others disorders (eg, diabetic cardiomyopathy, scleroderma, endomyocardial fibrosis).Type of amyloidosis:The major subtypes of systemic amyloidosis classified based on the underlying etiologies are as follows: primary(AL) amyloidosis, Secondary (AA) amyloidosis (or reactive amyloidosis), familial amyloidosis (ATTR or hereditary amyloidosis), dialysis-related amyloidosis, and senile systemic amyloidosis (SSA).Cardiac amyloidosis in one of the common infiltrative cardiomyopathies associated with an unfavorable prognosis. The pathophysiology involves the adoption of normal protein into an amyloid state, resulting from cleavage, denaturation, or excess production of abnormal protein, which eventually assume anti parallel beta-pleated sheet configuration forming amyloid fibrils.Clinical manifestation of cardiac amyloidosis:Although systemic involvement of amyloidosis varies according to organ involvement, cardiac manifestations predominantly include symptoms of right-sided heart failure(HF). Symptoms and signs of heart failure include dyspnea and signs of right-sided heart failure including peripheral edema, hepatomegaly, and ascites