Pulmonary arteriovenous fistulas. Case presentations and clinical recognition

This report describes characteristic features and clinical presentation of six young patients (ranging in age from five to 25 years) with various congenital forms of pulmonary arteriovenous fistulas as well as one 21-year old patient with traumatic intrapulmonary arteriovenous fistula. The former six patients were cyanotic, had clubbing of the digits and exertional dyspnea; two, additionally, had telangiectasia. All patients underwent cardiac catheterization and pulmonary angiography. None had pulmonary hypertension. Angiographically, four had diffuse arteriovenous fistulas in both lungs (Figures 1 and 3 to 5) and two had arteriovenous fistulas localized in one pulmonary segment. Four patients were additionally studied with radionuclide angiography and pulmonary perfusion scintigraphy. In three of these, substantial right-to-left shunt in the region of the lungs was detected; the diagnosis could not be established in only one of the latter with a small traumatic intrapulmonary arteriovenous shunt of less than 10%. While noninvasive methods provide adjunctive diagnostic support, pulmonary angiography is still required to assess, with certainty, the nature and localization of the lesion. In cyanotic patients with no evidence of cardiac disease, differential diagnostic consideration should be given to pulmonary arteriovenous fistulas.