Unique variant of Taussig-Bing heart: double-outlet right ventricle with double ventricular septal defects and double overriding of great arteries.
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.
de Oliveira e Silva
E R
ER
Division of Pediatric Cardiology, New York Hospital-Cornell University Medical Center, New York.
Snyder
M S
MS
O'Loughlin
J E
JE
Klein
A A
AA
Magid
M S
MS
Engle
M A
MA
Lev
M
M
Bharati
S
S
D004310:Double Outlet Right Ventricle / Q000175:diagnosis* / Q000473:pathology; D006344:Heart Septal Defects, Atrial / Q000175:diagnosis* / Q000473:pathology; D006345:Heart Septal Defects, Ventricular / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male