Idiopathic Bilaterally Diffuse Arteriovenous Fistulas Causing Severe Central Cyanosis

Excerpt

We present an extremely rare case of diffuse bilateral pulmonary arteriovenous fistulas (PAVF) of unknown etiology. The patient presented at 15 years of age with increasing cyanosis and chest pain. He had central cyanosis and clubbing, Transcutaneous oxygen saturation was 50%. Transthoracic echocardiography showed no evidence of an intracardiac defect; however, an intravenous bolus of agitated saline demonstrated immediate return of microbubbles to the left atrium suggesting the possible presence of intrapulmonary arteriovenous fistulas (Fig. 1). Cardiac catheterization showed a vascular net with diffuse small arteriovenous fistulas in the right and left lungs (Fig. 2). No evidence of Rendu–Osler–Weber syndrome or other systemic diseases causing PAVFs could be demonstrated.

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