A 22 year-old male patient was admitted with supracardiac, nonobstructive, total anomalous pulmonary venous connection and large atrial septal defect. Cardiac catheterization revealed severe pulmonary hypertension due to pulmonary overflow (Qp/Qs 6, pulmonary vascular resistance = 1.6 Woods/m²). Complete repair was performed through median sternotomy and cardiopulmonary bypass. The patient was discharged from hospital on the sixth postoperative day with no complications, using converting enzyme inhibitor and aspirin. At six-month follow-up he is currently asymptomatic, with preserved biventricular function and no echocardiographic signs of pulmonary hypertension.
Heart defects, congenital; Hypertension, pulmonary; Pulmonary veins