Cardiomyopathy part three: Restrictive


Restrictive Cardiomyopathies ( Part Three)

This type of cardiomyopathy is defined as the “stiffness†of the ventricle that disallows a significant increase in volume of the ventricle but causes the pressure to rise to abnormally high levels.
These can be categorized in two ways: reduced systolic volumes and reduced diastolic volumes.

These are the rarest types of cardiomyopathies (3% of all cardiomyopathies) but the prognosis is very grave with half of all children dying in the first 2 years without transplantation. However, survival rates after transplantation is very good. Most children require transplantation within 4 years.

Cardiac failure usually occurs rapidly as a result of increased pulmonary vascular resistance and increased pulmonary hypertension. Symptoms include chest pain, ischemia, and syncope and ECG changes.

There are numerous causes for this disease and is often familial (3 of ten children). Genetic mutations of varying types are the most common cause of this disorder. Myocardial disorders caused by endocardial fibrosis, fibroelastosis, and endomyocardial diseases, parasitic infections, and other acquired factors are common. Infiltrative and storage diseases of the endocardium are also contributing factors.

Loffler’s endocarditis and Anderson-Fabry disease may be examples of this. Sarcoidosis is yet another contributing factor.

Onset is quick, and prognosis is poor, with over half of children of adolescents dead within a year.

Imaging techniques include MRI, CT and sonography. Findings usually include ventricular dilatation without hypertrophy. Be watchful of thrombosis.
This disease causes significant ventricular non-compliance which causes a significant rise in ventricular filling pressures that reduces cardiac output.

This defect is often confused with other symptomatic problems such as asthma and chest infections which may delay diagnosis.

Echocardiography generally reveals significant increases in bi-atrial size, severe diastolic dysfunction, and increased end diastolic pressures and pulmonary hypertension.

Transplantation is the recommended solution, but prophylactic treatment is necessary until that time.

Ken Heiden RDCS