Endocardial fibroelastosis of the left ventricle in a patient with Alagille syndrome

We present a case of a 6 week old infant with Alagille syndrome. Cardiological evaluation revealed peripheral pulmonary arterial stenosis and left ventricular endocardial fibroelastosis. While peripheral pulmonary arterial stenosis are typical for the syndrome this is the first case reported with endocardial fibroelastosis. Complex cardiac malformations may worsen the prognosis in Alagille syndrome. They require early diagnosis and therapy.