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Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas: Discussion (1)

Our case documents hypoxemia and intrapleural rupture of an AVF in a young woman in her 24th week of pregnancy. She had been asymptomatic prior to pregnancy, suggesting a causal relationship. Complicating her illness was the presence of rheumatic mitral valve disease and congestive heart failure. Hemothorax is a rare but potentially fatal complication of pulmonary AVFs. There have been 16 reported cases of rupture into the pleural space, including our own. Of the 14 in which outcome was reported, sudden death occurred in five. In addition, there are two reports of hemothorax due to the rupture of pleural telangiectasia.' Interestingly, 11 of 13 cases in which the location of the hemothorax was mentioned were left-sided—the reason for this finding is unclear. Pregnancy has been reported to cause deterioration in patients with pulmonary AVFs. Hoffman and Rabens reported the case of a 25-year-old woman who developed two radiographically apparent AVFs during pregnancy after having a normal chest x-ray film documented prior to pregnancy. Swinburne et al described a 21-year-old woman with multiple pulmonary AVFs who experienced worsening dyspnea and hypoxemia during pregnancy. A cesarean section at 35 weeks’ gestation was necessary.
Tags: congestive heart failure hemothorax hypoxemia pleural effusion