Pulmonary arteriovenous fistulas

Area of Science:

• Cardiology
• Pulmonary Medicine
• Interventional Radiology

Background:

• Pulmonary arteriovenous fistulas (PAVF) are abnormal connections between pulmonary arteries and veins.
• While often associated with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome), many patients lack this condition.
• Previous institutional experience documented 63 PAVF cases from 1952-1972.

Purpose of the Study:

• To report on an additional 38 patients with PAVF seen from 1973-1981.
• To describe the use of miniballoon occlusion for acquired systemic artery-to-pulmonary artery fistulas.
• To highlight the diagnostic workup for PAVF.

Main Methods:

• Review of 38 consecutive PAVF patients (1973-1981) and 5 patients with acquired fistulas.
• Miniballoon occlusion technique described for acquired fistulas.
• Sequential diagnostic workup including chest imaging, arterial blood gases, contrast echocardiography, angiography, and differential pulmonary venous oxygen content measurement.

Main Results:

• The 38 new patients represent an 8.5-year follow-up period.
• 18 (47%) of the 38 patients had Rendu-Osler-Weber syndrome.
• Miniballoon occlusion was used for acquired fistulas, potentially avoiding extensive surgery.

Conclusions:

• PAVF diagnosis relies on a prioritized sequence of investigations.
• Miniballoon occlusion offers a less invasive therapeutic option for specific PAVF types.
• The prevalence of Rendu-Osler-Weber syndrome in PAVF patients varies.