Diffuse pulmonary arteriovenous malformations - Characteristics and prognosis

Objective: To study the clinical characteristics and prognosis of patients with diffuse pulmonary arteriovenous malformations (AVMs). Design: Retrospective chart review of all patients (n = 16) with diffuse pu lmonary AVMs seen at Yale New Hal en Hospital, Johns Hopkins Hospital, and St. Michael's Hospital, Up-to-date follow-up information was obtained in al l living patients. Results: All patients were severely hypoxic. Neurologic complications (stro ke or brain abscess) had occurred in 70% of patients by the time of diagnos is. During the follow-up period (mean, 6 years), three patients died and tw o others developed new neurologic complications, One of the deaths occurred perioperatively during lung transplantation All patients underwent transca theter embolo-therapy of anp large pulmonary AVMs. A selected group underwe nt pulmonary flow redistribution, a novel technique. Oxygenation did not im prove significantly with embolotherapy of the larger AVMs, but there was a small significant improvement in those patients who underwent pulmonary no iv redistribution. The majority (85%) of the living patients are currently working or studying full-time. Conclusions: Patients with diffuse pulmonary AVMs are at increased risk of neurologic complications. Transcatheter embolotherapy does not significantl y improve the profound hypoxia, but it may reduce the risk of neurologic co mplications, Antibiotic prophylaxis is recommended for bacteremic procedure s to prevent brain abscess, These patients can live for many years and lead productive lives, We do not recommend lung transplantation because surviva l with disease is difficult to predict and we have observed ed a perioperat ive transplant death.