A case of restrictive cardiomyopathy presenting in fetal life with an isolated pericardial effusion

We describe the evolution during pregnancy of a case of restrictive cardiomyopathy which first presented at 22 weeks' gestation with a large pericardial effusion. Measurements of cardiac function were normal and remained near normal until late in the third trimester, when pulsed and tissue Doppler data suggested impairment in ventricular relaxation. This disease progressed in postnatal life to symptomatic restrictive cardiomyopathy by 2 years of age necessitating cardiac transplant. To our knowledge, this is the first time this unusual association has been reported.