Successful repair of double-outlet right ventricle with bilateral conus, 1-transposition of great arteries (S,D,L), and subpulmonary ventricular septal defect

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 71; no. 3; pp. 366 - 370
• Main Authors: Yamaguchi, M, Horikoshi, K, Toriyama, A, Kimura, K, Mito, H
• Format: Journal Article
• Language: English
• Published: United States AATS/WTSA 01.03.1976
• Subjects: Cardiac Catheterization; Child, Preschool; Female; Heart Septal Defects, Ventricular - complications; Heart Septal Defects, Ventricular - diagnosis; Heart Septal Defects, Ventricular - surgery; Heart Ventricles - abnormalities; Humans; Methods; Pericardium - transplantation; Polytetrafluoroethylene; Prostheses and Implants - methods; Transplantation, Autologous; Transposition of Great Vessels - complications; Transposition of Great Vessels - diagnosis; Transposition of Great Vessels - surgery
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/s0022-5223(19)40200-6

Surgical correction was carried out successfully in a severely cyanotic 3-year-old Japanese girl who had a very rare type of double-outlet right ventricle. The malformation was associated with bilateral conus, 1-transposition of the great arteries, and subpulmonary ventricular septal defect without significant pulmonary stenosis in situs solitus. A large amount of subaortic conal musculature which separated the aortic valve from the subpulmonary ventricular septal defect was removed, as was the anterior rim of the ventricular septal defect. A tunnel, constructed with a woven Teflon prosthesis, was inserted in such a manner as to direct blood from the left ventricle through the defect and out to the aorta. The pulmonary outflow tract was reconstructed with a Teflon patch lined with pericardium. The patient's postoperative recovery was uneventful, and she was doing well 3 months postoperatively. To our knowledge, no identical case with a similar type of surgical correction has previously been reported.