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Osler-Weber-Rendu Disease and Pulmonary Arteriovenous Fistulas (2)

Past medical history was remarkable for rheumatic heart disease as a child. Family history was remarkable for recurrent nosebleeds in her father and two children. Physical examination was notable for diminished breath sounds in the left lung base, fine rales in the right lung base, and a 3/6 mitral regurgitation murmur. Instances of telangiectasia were noted on the lips and buccal mucosa. Digital clubbing was present. Admission laboratory studies were remarkable for a hemoglobin level of 11.2 g/dl and a hematocrit value of 33. A chest x-ray film revealed a large left pleural effusion and multiple rounded densities in the right base with connections to the hilum (Fig 1). Arterial blood gas levels were as follows: pH, 7.50; Pco2, 29 mm Hg; and Po2, 66 mm Hg while the patient was receiving 2 L of 02. A thoracentesis revealed bloody fluid, and the hemothorax was drained with a thoracostomy tube. A selective left pulmonary angiogram was then performed with lead aprons wrapped around the patient s abdomen. It showed a large pulmonary AVF in the left lower lobe abutting the pleura. A shunt study revealed a Po2 while the patient was breathing room air of 52 mm Hg, a Po2 while breathing 100 percent 02 of 151 mm Hg, and a shunt fraction of 23 percent. Figure-1 Figure 1. Posteroanterior chest roentgenogram prior to embolization, showing left pleural effusion and multiple rounded densities with connections to hilum (arrowheads).
Tags: congestive heart failure hemothorax hypoxemia pleural effusion