We present a case of a one-day-old newborn, without prenatal diagnosis, referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%. Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation, functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus (PDA). Chest X-ray showed severe cardiomegaly and wall to wall heart. Prostaglandin E1 infusion was started once after birth. After few days, clinical conditions progressively worsened because of right heart failure; a first pharmacological approach to close PDA failed and surgery ligation of PDA was necessary to restore anterograde pulmonary flow and heart size.

 

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