Marfan syndrome (MS) is a connective tissue disease involving the cardiovascular, ocular, and the musculoskeletal systems. MS has variable phenotypic expression and is most often diagnosed in adult life. Infantile-onset MS is rare and is associated with severe cardiovascular manifestations; there is an extremely high mortality during the first 2 years of life. We present a case of a child with severe infantile MS who, during the course of infancy and early childhood, developed aortic root dilatation and polyvalvar insufficiency requiring subsequent successful replacement of the aortic root and of all cardiac valves. To our knowledge, this is the first reported case of quadrivalvar replacement in the pediatric age group.

The origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation. We report a baby who presented with an echocardiographically diagnosed perimembranous ventricular septal defect and normal left ventricular (LV) function. Later on the boy developed failure to thrive and increasing tachypnea. At the age of 5 weeks the ECG showed that LV strain and echocardiographic LV function had worsened (FS 18%). Echocardiography and heart catheterization showed that all coronary arteries originated from the pulmonary trunk. Intraoperative inspection revealed a single ostium for the right and left coronary artery in the nonfacing sinus of the pulmonary trunk. A tube was constructed connecting the coronary artery to the ascending aorta. Coronary perfusion was sufficient and the sinus rhythm was restored. However, in the early postoperative period there was a sudden deterioration of cardiac output followed by cardiac arrest. Reanimation was not successful.

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