ARTERIOVENOUS MALFORMATION (AVM) OCCURRING IN ASSOCIATION WITH SOFT TISSUE AND SKELETAL HYPERTROPHY FORMS THE BASIS OF THE PARKES WEBER SYNDROME: a rare congenital angio-osteohypertrophy condition with no obvious racial, gender or geographic preference. Usually in the lower limbs, the AVM can result in a dilated cardiomyopathy due to a high output cardiac state. We describe the case of a 21-year-old woman with an extensive AVM affecting one leg, which included the pelvic vasculature. The case concerns assessment of the current high output "heart failure" and her cardiovascular risk from future pregnancy.
pressure fell to 90/60 mmHg. The nitroprusside infusion was stopped and he was transferred to the operating theatre. Full cardiopulmonary bypass was established through the femoral vessels, he was cooled to 18 8C to protect the brain and spinal cord, and the proximal descending aorta was replaced with a woven Dacron prosthesis during a short period of circulatory arrest. He made a good recovery. COMMENT Massive haemoptysis, de®ned as expectoration of between 200 mL and 1000 mL of blood in 24 hours, is rare and accounts for less than 1.5% of all cases of haemoptysis 1. It may be caused by primary diseases of the lung parenchyma or of the aorta. Historically, tuberculosis and bronchiectasis were the most common primary lung diseases that caused massive haemoptysis but, as their incidence declined, bronchitis and carcinoma of the lung became more important 1. Disruption of the aorta often results in sudden death from exsanguination into the hemithorax. However, in some cases local in¯ammation causes the lung to adhere to the aorta with formation of an aortopulmonary ®stula. The onset of haemoptysis then offers a small window of opportunity to save the patient. Before 1960, mycotic aneurysms caused by tuberculosis, syphilis, and other infections were the leading causes of aortopulmonary ®stula due to aortic disease. Today the predominant causes are atherosclerosis and thoracic aortic surgery 2. Haemoptysis in any patient with a history of thoracic surgery must be treated with the utmost suspicion. Concurrent haematemesis must not distract the clinician from the diagnosis. Blood may appear in the back of the throat without the patient being certain of its origin. While inhaled irritants elicit a cough re¯ex, warm blood may elicit either no response or a gag re¯ex. Consequently, the blood may seem to have been vomited. Matters may be further confused because some blood is swallowed and vomited later. A careful history, examination and plain radiographs should point to the diagnosis. A mediastinal mass on plain chest radiographs demands urgent investigation with crosssectional imaging or angiography and early involvement of cardiothoracic surgeons.
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