Restrictive Cardiomyopathy

Overview

Restrictive cardiomyopathy is an abnormal condition of the heart where the ventricles (pumping chambers) are stiff and do not allow the heart to fill with blood normally, leading to increased pressures in the heart. The increased pressures in the heart lead to lung, liver, and leg congestion, which leads to heart failure symptoms described in the symptoms section.

An illustration showing restrictive cardiomyopathy.

Restrictive cardiomyopathy is the rarest form of cardiomyopathy. Causes of restrictive cardiomyopathy can be specific to the heart or related to other medical conditions; genetics also play a role. These causes include:

Fibrosis
Sarcoidosis
Amyloidosis
Hemochromatosis/Iron overload
Lysosomal and other glycogen storage diseases
Radiation therapy
Drugs such as certain chemotherapies

Due to the elevated pressures in the heart ventricles, the atria (upper chambers of the heart) dilate, which leads to complications such as abnormal atrial rhythms (like atrial fibrillation), leaky heart valves, and blood clots in the heart that can cause strokes. Some patients develop a reduction in the ability of their heart to pump blood and get a condition called heart failure with reduced pump function. Other patients can have sudden cardiac death. If symptoms and the disease progress, the patient can be referred for a heart transplant.