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Erschienen in:
01.10.2007 | Images in Pediatric Cardiology
Idiopathic Bilaterally Diffuse Arteriovenous Fistulas Causing Severe Central Cyanosis
Erschienen in: Pediatric Cardiology | Ausgabe 5/2007
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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.
Fig. 1
Saline contrast transthoracic echocardiogram showing microbubbles filling the left atrium following systemic venous saline injection, suggesting right-to-left shunting of blood. LA, left atrtium; LV, left ventricle; RA, right atrium; RV, right ventricle.
Fig. 2
Right (A) and left (B) pulmonary artery angiograms (posterior–anterior projection) showing the “reticular pattern” of multiple telangiectasias.
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