Unique variant of Taussig-Bing heart: Double-outlet right ventricle with double ventricular septal defects and double overriding of great arteries |
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Authors: | Elizabeth R. de Oliveira e Silva Michael S. Snyder John E. O'Loughlin Arthur A. Klein Margret S. Magid Dr. Mary Allen Engle Maurice Lev Saroja Bharati |
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Affiliation: | (1) Division of Pediatric Cardiology, The New York Hospital-Cornell University Medical Center, 525 East 68th Street, 10021 New York, New York, USA;(2) Department of Pathology, The New York Hospital-Cornell University Medical Center, 525 East 68th Street, 10021 New York, New York, USA;(3) Congenital Heart and Conduction System Center, The Heart Institute for Children, Christ Hospital and Medical Center, Palos Heights, Illinois, USA |
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Abstract: | Summary A cyanotic, tachypneic newborn was diagnosed to have double-outlet right ventricle of the Taussig-Bing type. Cardiac failure did not respond to medical treatment or surgical palliation. Postmortem examination revealed two ventricular septal defects (VSDs), one a malalignment VSD in the membranous septum and adjacent tissue and the other in the anterosuperior part of the muscular septum. The D-malposed aortic root emerged mainly from the right ventricle, with aortic-mitral continuity. The larger posterolateral pulmonary root arose almost entirely from the right ventricle, confluent with the muscular VSD, and unrelated to the mitral valve. Its right ventricular aspect was obstructed by hypertrophied infundibulum.This unique malformation of the heart functioned as a double-outlet right ventricle of Taussig-Bing type. In addition, however, the malformation had elements of tetralogy of Fallot because of the malaligned VSD and hypertrophied conal musculature (although pulmonary flow was excessive), and also of complete transposition of the great arteries because of the arrangements of the two VSDs, which favored aortic flow from right ventricle and pulmonary blood flow from the left ventricle. Thus, a single heart presented similarities to three anatomic and functional entities. |
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Keywords: | Double-outlet right ventricle Taussig-Bing heart Malaligned ventricular septal defect Double ventricular septal defects D-Malposition of aorta |
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