Pulmonary arteriovenous malformations: Epidemiology, etiology, and pathology in adults
- James R Gossage, MD
James R Gossage, MD
- Professor of Medicine
- Augusta University
Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between pulmonary arteries and veins . Alternative names include pulmonary arteriovenous fistulae, pulmonary arteriovenous aneurysms, cavernous angiomas of the lung, and pulmonary telangiectases . PAVMs are uncommon in the general population, but they are an important consideration in the differential diagnosis of common pulmonary problems, including hypoxemia, pulmonary nodules, and hemoptysis. In addition, they are a common occurrence in patients with hereditary hemorrhagic telangiectasia (HHT, also called Osler-Weber-Rendu syndrome).
The epidemiology, etiology, pathology, clinical features, and natural course of PAVMs are reviewed here. The diagnosis and treatment of PAVMs are discussed separately. (See "Pulmonary arteriovenous malformations: Clinical features and diagnostic evaluation in adults" and "Therapeutic approach to adult patients with pulmonary arteriovenous malformations".)
General population — Pulmonary arteriovenous malformations (PAVMs) are uncommon in the general population. This was illustrated by a study of 15,000 consecutive autopsies, in which only three cases of PAVMs were detected . Three additional case series from the Mayo Clinic suggested a similar low rate of PAVMs in the general population reporting 194 cases of PAVMs over 45 years (ie, 4.3 cases per year) [3-5].
PAVMs are 1.5 to 2 times as common among women, compared with men .
In patients that have PAVMs, they are commonly multiple, and vary in their number, distribution, and size. As an example, in one retrospective study of 350 patients with PAVMs, 36 percent of patients had multiple lesions and 25 percent had bilateral disease . More than half of the lesions were in the lower lung fields and almost 80 percent involved the pleura. In another study of 112 patients who underwent embolization of PAVM, 67 percent had multiple PAVMs and 92 percent were in the lower lobes . Other retrospective series indicate that PAVMs are typically 1 to 5 cm in size (but occasionally exceed 10 cm) and that 7 to 11 percent of patients have diffuse microvascular PAVMs that may occur in combination with radiographically visible PAVMs [5,9,10]. PAVMs tend to increase in size over time and rarely regress spontaneously.
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