Endocardial fibroelastosis of the right ventricle in the newborn Presenting the clinical picture of the hypoplastic right heart syndrome

A 12 hour old infant with extensive right ventricular endocardial fibroelastosis presenting with cyanosis and all the clinical features of the hypoplastic right heart syndrome is reported. Anatomically, there were no other associated cardiac defects. Endocardial fibroelastosis of the right ventricle, therefore, must be recognized as another cause of cyanosis in the newborn which may present with all the clinical manifestations of the hypoplastic right heart syndrome.

Although many different pathologic entities can produce the hypoplastic right heart syndrome, clinical and hemodynamic features are similar. Since the major physiologic derangement is inadequate blood flow to the lungs in all conditions included in this syndrome, a palliative shunt procedure to increase pulmonary blood flow is indicated in symptomatic patients, regardless of the exact anatomic diagnosis.