PULMONARY ARTERIOVENOUS-FISTULAS

Abstract

A series of 63 patients with pulmonary arteriovenous fistula who were seen from Jan. 1, 1952, through Dec. 31, 1972, was previously reported on. Subsequently, 38 additional patients during the 8.5-yr period from Jan. 1, 1973, through June, 1981, were seen. The series includes 3 patients with hereditary telangiectasia who had bilateral pulmonary arteriovenous fistulas removed at 2 separate thoracotomies. A brief description of 5 additional patients with acquired systemic artery-to-pulmonary artery fistula who underwent miniballoon occlusion of the fistula are also included. These 5 cases were included because this therapeutic catheterization technique may be of particular value in patients with multiple or bilateral pulmonary arteriovenous malformations and may obviate extensive pulmonary resection and repeat thoracotomy. Pulmonary arteriovenous fistula is believed to occur most often in middle-aged women who have associated Rendu-Osler-Weber syndrome, but most of the patients did not have hereditary hemorrhagic telangiectasia. Twenty-three (36.5%) of the 63 patients in a previous Mayo Clinic series and 18 (47%) of the 38 in the present series had associated Rendu-Osler-Weber syndrome. A logical workup of a patient with pulmonary arteriovenous fistula includes, in sequential fashion based on priority, chest roentgenography and tomography, arterial blood gas measurements, contrast echocardiography with indocyanine green dye, angiography and measurement of differential pulmonary venous O2 content.