Objective: To study the clinical characteristics and
prognosis of patients with diffuse pulmonary arteriovenous
Design: Retrospective chart
review of all patients (n = 16) with diffuse pulmonary AVMs seen at
Yale New Haven Hospital, Johns Hopkins Hospital, and St. Michael’s
Hospital. Up-to-date follow-up information was obtained in all living
Results: All patients were
severely hypoxic. Neurologic complications (stroke or brain abscess)
had occurred in 70% of patients by the time of diagnosis. During the
follow-up period (mean, 6 years), three patients died and two others
developed new neurologic complications. One of the deaths occurred
perioperatively during lung transplantation. All patients underwent
transcatheter embolotherapy of any large pulmonary AVMs. A selected
group underwent pulmonary flow redistribution, a novel technique.
Oxygenation did not improve significantly with embolotherapy of the
larger AVMs, but there was a small significant improvement in those
patients who underwent pulmonary flow redistribution. The majority
(85%) of the living patients are currently working or studying
Conclusions: Patients with diffuse
pulmonary AVMs are at increased risk of neurologic complications.
Transcatheter embolotherapy does not significantly improve the profound
hypoxia, but it may reduce the risk of neurologic complications.
Antibiotic prophylaxis is recommended for bacteremic procedures to
prevent brain abscess. These patients can live for many years and lead
productive lives. We do not recommend lung transplantation because
survival with disease is difficult to predict and we have observed a
perioperative transplant death.