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Virtual Pediatric Hospital: Paediapaedia: Pulmonary Arteriovenous Malformation (AVM) Paediapaedia: Chest and Airway Diseases

Pulmonary Arteriovenous Malformation (AVM)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Twenty-five percent have symptoms including cyanosis, dyspnea, and easy fatigability. There may be a machinery murmur, especially on inspiration. Fifty percent have telangiectasias on the mucous membranes and skin - especially in familial hemorrhagic telangiectasis (Osler-Weber Rendu).

Etiology/Pathophysiology:
A congenital malformation resulting in direct intrapulmonary connections between the pulmonary arteries and veins without an intervening capillary bed. This causes a right to left shunt with peripheral arterial oxygen desaturation. Brain abscesses can develop due to lack of pulmonary capillary filtration of microorganisms.

Pathology:
Not applicable

Imaging Findings:
Sixty-five percent are single, 75% are unilateral, and 60% are in the lower lobes usually right beneath the visceral pleura. On CXR a soft tissue mass is seen in the lung that may have tubular densities (vein and artery) connecting it to the hilum. Pulmonary angiography is diagnostic and is used to define the number, size and location of the fistulae.

DDX:

References:
See References Chapter.

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