[Pulmonary arterio-venous aneurysm and fistula (author's transl)]

Pulmonary arteriovenous aneurysms and fistulae (PAF) can appear in people with or without hereditary hemorrhagic telangiectases (HHT). There is a 3 to 6% chance to find PAF in members of a family with HHT and 17% chance to find PAF in a patient himself with HHT. The authors present the cases of 6 patients with PAF, 13 to 64 years old, all treated surgically. In this group there are as many males as females. Three patients with HHT had nose bleeding and hemangiomas in their family history. Three patients had dyspnoea on exertion and insufficient oxygen saturation. Two patients had neurological symptoms; four had extracardiac murmur. Location was 3 times in the inferior lobe, twice in the superior and once in the middle pulmonary lobe. Surgical treatment can be indicated for a clinical or roentgenological progression, for an important right-to-left shunt or for existing or anticipated complications such as hemorrhages, thrombosis with embolism and central nervous system disorders with cerebral abscess. For these reasons, surgical excision is also advisable in asymptomatic patients, where diffuse bilateral lesions are to be expected, that recur frequently. The authors performed one wedge resection, three segmental resections and two lobectomies.