Restrictive Cardiomyopathy (Smarty PANCE)

What is restrictive cardiomyopathy (RCM)?

Restrictive cardiomyopathy describes when the heart muscle is restricted, meaning that it becomes stiffer and less compliant. However, the muscles and size of the ventricles stay about the same size or only get slightly enlarged.

What are some common causes of restrictive cardiomyopathy?

Most are idiopathic but other causes include infiltrative diseases such as sarcoid and amyloid, and storage diseases, such as hemochromatosis. Other potential causes include radiation exposure and cancer.

What is the most common type of cardiomyopathy seen in amyloidosis?

Restrictive cardiomyopathy is the most common type of cardiomyopathy seen in amyloidosis.

Clinical restrictive cardiomyopathy? of restrictive cardiomyopathy?

Most patients with RCM present with signs and symptoms of HF (eg, dyspnea with exertion, edema) progressing over months or years.

Is restrictive cardiomyopathy a contraction or dilation problem?

RCM is a dilation problem - The heart is unable to dilate properly in restrictive cardiomyopathy

Is restrictive cardiomyopathy a systolic or diastolic heart failure?

Since filling happens during diastole restrictive cardiomyopathy this is a type of diastolic heart failure

How is restrictive cardiomyopathy diagnosed?

ECG, chest x-ray, and echocardiography are required. If the diagnosis is still in doubt, MRI

What is seen on EKG and chest x-ray in restrictive cardiomyopathy?

- The ECG is usually nonspecifically abnormal, showing ST-segment and T-wave abnormalities and sometimes low voltage.

- On chest x-ray, RCM generally demonstrates cardiomegaly secondary to significant atrial enlargement with or without pulmonary venous congestion and pleural effusions.

What is seen on echocardiography in restrictive cardiomyopathy (RCM)?

While there is no formal echocardiographic definition of RCM, the signs that characterize RCM include the following:

● Normal or near normal left ventricular (LV) systolic function and cavity size

● Marked enlargement of one or both atria

● Abnormal diastolic function

What type of heart sound is heard on cardiac auscultation in restrictive cardiomyopathy due to ventricular noncompliance?

Restrictive cardiomyopathy can cause an S4 heart sound to be heard on cardiac auscultation, due to ventricular noncompliance

What is the treatment of restrictive cardiomyopathy (RCM)?

Try to treat the cause - regardless of the etiology, the treatment of patients with RCM involves managing volume status to reduce pulmonary and central venous congestion and management of end-organ (eg, liver, kidney) dysfunction

What is the definitive cure for restrictive cardiomyopathy?

The cure for restrictive cardiomyopathy is a heart transplant