A 24-year-old Indian man died in 1977 after a long illness that began in 1961 (aged 8 years) with a severe gastrointestinal haemorrhage. Laparotomy showed large oesophageal varices that were oversewn. The spleen was enlarged but the liver looked normal. After a further gastrointestinal bleed in 1966 he underwent splenic venography, which showed a small, poorly opacifying portal vein and extensive oesophageal varices. Splenectomy and lienorenal anastomosis were performed. Chest radiography was normal. He remained well until 1972 when he developed pneumonia. Cardiac catheterisation at this time showed a mean pulmonary arterial pressure of 64 mmHg, a mean wedge pressure of 5 mmHg, and a pulmonary vascular resistance of 826 dyne s cm-5. Pulmonary angiography showed aneurysmal dilatation of the pulmonary trunk and pruning of the peripheral vessels but no sign of thromboembolism. Hepatic wedge pressure was 3 mmHg. Results of spirometry, liver function tests, and a liver biopsy were normal. He was treated with anticoagulants but his exercise tolerance gradually decreased, and in 1976 he developed signs of right heart failure from which he died in July 1977.