Fatal Congenital Heart Disease in a Postpartum Woman

The image represents the post-mortem heart of a 28 years-old female patient, diagnosed in childhood with complete common atrioventricular canal defect. At time of diagnosis, the family refused surgery. Despite being advised against pregnancy, she became pregnant. On presentation to hospital, she was cyanotic, with clubbed fingers, hemodynamically instable, in sinus rhythm, with Eisenmenger syndrome and respiratory failure partially responsive to oxygen. During pregnancy, owing to systemic vasodilatation, the right-to-left shunt is increased, with more severe cyanosis and low cardiac output [1]. Echocardiography revealed complete common atrioventricular canal defect, a single atrioventricular valve with severe regurgitation, right ventricular hypertrophy, pulmonary artery dilatation, severe pulmonary hypertension and hypoplastic left ventricle. She gave birth vaginally to a healthy boy. Three days later, the patient died. The autopsy revealed hepatomegaly, greatly hypertrophied right ventricle with a purplish clot ascending the dilated pulmonary arteries and hypoplastic left ventricle with narrowed chamber. A single valve was observed between the atria and ventricles, making all four heart chambers communicate, also insufficiently developed interventricular septum and its congenital absence in the cranial third. These morphological changes define complete common atrioventricular canal defect, with right ventricular dominance, a rare and impressive malformation, mandatory to be solved in early childhood [2,3].