Restrictive/Infiltrative Cardiomyopathy
Overview
Plain-Language Overview
Restrictive/Infiltrative Cardiomyopathy is a condition that affects the heart muscle, making it abnormally stiff. This stiffness prevents the heart from filling properly with blood, which can reduce the amount of blood pumped to the rest of the body. The main body system involved is the cardiovascular system, specifically the heart's ventricles. People with this condition often experience symptoms like shortness of breath, fatigue, and swelling due to fluid buildup. The heart's ability to relax and fill during the resting phase is impaired, leading to problems with blood flow and overall heart function. This condition can be caused by abnormal substances building up in the heart muscle or scarring from other diseases.
Clinical Definition
Restrictive/Infiltrative Cardiomyopathy is characterized by impaired ventricular filling due to increased myocardial stiffness without significant ventricular dilation. The core pathology involves fibrosis, amyloid deposition, or infiltration by abnormal substances such as in amyloidosis, sarcoidosis, or hemochromatosis. This leads to diastolic dysfunction with preserved systolic function in early stages. The condition results in elevated filling pressures and symptoms of congestive heart failure. It is distinguished from other cardiomyopathies by the restrictive physiology and often normal or mildly increased ventricular wall thickness. The clinical significance lies in its progressive nature and poor prognosis if untreated, often requiring specific diagnostic and therapeutic approaches.
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