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A 44 year old woman presented with increasing breathlessness and peripheral oedema. Examination revealed central and peripheral cyanosis, pronounced elevation of the venous pressure with tricuspid regurgitation, a palpable right ventricle, and a fixed split second sound with an exaggerated pulmonary component. There was pulsatile hepatomegaly, ascites, and peripheral oedema to the mid thigh.
Oxygen saturation at rest was 65%. Transthoracic echocardiography revealed pronounced dilatation of the right ventricle with a large primum atrial septal defect and a stenotic membrane which partitioned the left atrium. Transoesophageal echocardiography confirmed the membrane separating venous return from all but the left lower pulmonary vein, from the left atrium (arrow indicates orifice between the membrane and the left atrium (LA); LV, left ventricle; RA, right atrium, RV, right ventricle). The orifice in the membrane was < 1 cm in diameter with Doppler features consistent with significant flow obstruction. There was torrential tricuspid regurgitation directed across the large primum atrial septal defect causing mixing of unoxygenated and oxygenated blood. Corrective surgery was contemplated, but before this could be arranged she decompensated and died.