A 4-year-old girl was examined for dyspnoea and cyanosis at exercise. An echocardiogram showed a large atrial septal defect in the upper part of the atrial septum. Systolic pulmonary artery pressure was high (70 mmHg) and the inferior vena cava was connected with the left atrium ( Fig. 1 ). A computed tomography scan confirmed this rare anomaly of the inferior vena cava ( Fig. 2 ). Cardiac catheterization showed that the left atrium was posteriorly placed and received all four pulmonary veins, while the right anterior atrium received the right superior vena cava ( Fig. 3 ). Surgical repair was performed successfully. Midterm follow-up showed persistence of moderate pulmonary arterial hypertension.
