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Phaeochromocytoma is rare and usually presents as paroxysmal or sustained hypertension; none the less, it can also cause severe acute pulmonary oedema in normotensive individuals.Six patients with phaeochromocytoma presenting in Cornwall and West Devon between 1982 and 1986 bilateral scattered crepitations. The chest x ray showed a slightly enlarged cardiac shadow and pulmonary oedema. The electrocardiogram showed sinus tachycardia, poor R wave progression in the chest leads, and non-specific T wave changes. Full blood count and electrolytes were normal and analysis of arterial blood gases showed Po2 9-8 kPa, Pco2 3-2 kPa, and pH 7-2. She deteriorated rapidly and despite resuscitative measures she died within two hours of admission.At necropsy the heart weighed 285 g (normal 250-400 g) with minimal dilatation of both ventricles. No significant lesions were found in the coronary arteries and histological examination of the heart showed patchy muscle fibre necrosis with phagocytosis. Severe haemorrhagic oedema was found in both lungs, which showed no evidence of acute pulmonary infarction. A tumour 4 cm in diameter was found in the right adrenal gland and histology showed this to be a phaeochromocytoma.PATIENT 2 A 63 year old woman was admitted with a history of chest pain and breathlessness for two hours. The week before she had presented to the local cottage hospital with malaise, pedal oedema, and non-specific chest discomfort. She had had no other significant problems in the past and had never had hypertension. On admission she was pale with sinus tachycardia of 146 beats/min and blood pressure of 110/70 mm Hg. Jugular venous pressure was 3 cm above the sternal angle and auscultation showed a gallop rhythm. She had widespread crepitations in both lung fields. The electrocardiogram showed no evidence of myocardial infarction or left ventricular hypertrophy and the chest x ray was consistent with pulmonary oedema. She was treated with frusemide, amoxycillin, and dobutamine but she did not respond to these measures and died within eight hours of admission.At necropsy the heart weighed 350 g (normal 250-400 g) and was macroscopically normal. Histological examination of the myocardium showed vacuolar and focal necrosis with an inflammatory reaction. The coronary arteries were normal. The airways contained frothy liquid and the lungs were moist on section with microscopic changes consistent with acute pulmonary oedema. The right adrenal gland was replaced by a phaeochromocytoma weighing 70 g with typical histology. PATIENT 3 This 39 year old woman with long standing 234

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A 54-year-old woman with unoperated patent ductus arteriosus developed fatal cardiac tamponade due to a dissecting aneurysm of the pulmonary artery causing haemopericardium. Histology of the pulmonary artery showed the presence of cystic medial necrosis. Previous reports of this condition are reviewed and the role of pulmonary hypertension causing secondary cystic medial necrosis is discussed.