عنوان مقاله [English]
Restrictive cardiomyopathy defined by increased ventricular wall stiffness and impaired LV filling and heart failure symptoms. This type of cardiomyopathy occurs with lower frequency relative to the dilated and hypertrophic cardiomyopathies. IRCM in 50% is idiopathic and in 50% is secondary to specific clinical disorders especially amyloidosis. Cardiac amyloidosis usually occurs during primary amyloidosis (AL type amyloidosis). Primary amyloidosis is often seen in people with multiple myelomacancer or in the in course of immunocytic dyscrasia. Disease process results from tissue deposition of proteins that have a unique secondary structure. Cardiac amyloidosis is an invariably progressive infiltrative cardiomyopathy that carries a grave prognosis we have presented a case of Cardiac amyloidosis which is a rare clinical disorder.
The Present case was a 46 years old man who admitted for progressive dyspnea and congestive heart failure. Echocardiography revealed increased ventricular wall thickness with small size ventricles, enlarged atria, and a thickened intertribal septum and advanced diastolic dysfunction. Electrocardiography revealed low QRS voltage and normal sinus rhythm. Gingival Biopsy examined by congo Red staining and immunohistochemistry identified specific amyloid proteins. Pathology report confirmed amyloidosis.
In patient complaining of progressive congestive heart failure and suspected to have restrictive cardiomyopathy, cardiac amyloidosis should be born in mind.