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Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt |
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| Authors: | H S Bernstein P C Ursell M M Brook F C Hanley N H Silverman J Bristow |
| Institution: | (1) Department of Pediatrics, Division of Pediatric Cardiology, University of California San Francisco, Box 0544, 94143 San Francisco, CA, USA;(2) Department of Pathology, University of California San Francisco, 94143 San Francisco, CA, USA;(3) Department of Surgery, Division of Cardiothoracic Surgery, University of California San Francisco, 94143 San Francisco, CA, USA |
| Abstract: | An infant with cyanotic congenital heart disease and polysplenia syndrome developed profound cyanosis within months of undergoing bilateral cavopulmonary anastomoses. Intrapulmonary shunting was diagnosed by contrast echocardiography with peripheral venous and selective pulmonary artery injection. Histopathology revealed abnormal, thin-walled vessels within the interstitium of the lung lobule. These vessels have not been reported previously and are likely to be the anatomic site of arteriovenous shunting. This case demonstrates that pulmonary arteriovenous fistulas (PAVFs) may develop rapidly after cavopulmonary anastomosis in young infants. It also illustrates the use of contrast echocardiography for following PAVF progression in these patients. |
| Keywords: | Pulmonary arteriovenous fistulas Cavopulmonary anastomosis Cyanotic heart disease Contrast echocardiography |
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