Korean J Thorac Cardiovasc Surg.  2003 Jul;36(7):510-513.

Aortic Root Replacement in Patient of Right Ventricular Hypoplasia with Annuloaortic Ectasia, Ventricular Septal Defect and Aortic Regurgitation: Report of 1 case

• Baek MJ
• Na CY
• Oh SS
• Kim WH
• Whang SW
• Lee C
• Chang Y
• Jo WM
• Kim JH
• Seo HJ
• Kang SS
• Moon HS
• Park YK
• Kim CW

Abstract

Annuloaortic ectasia, aortic regurgitation (AR), and ventricular septal defect (VSD) in patients with right ventricular hypoplasia is a very rare condition. We report a patient who underwent aortic root replacement with a composite graft for annuloaortic ectasia associated with VSD and AR in right ventricular hypoplasia. The patient was a 19 year-old male. Transthoraic echocardiogram and cardiac catheterization revealed a perimembranous VSD (2 cm in diameter), severe AR, annuloaortic ectasia, bipartite right ventricle with hypoplasia, and hypoplastic tricuspid valve. Operative findings showed that free margins of the right and noncoronary cusps were markedly elongated, thickened, and retracted, and commissure between the right coronary cusp and the noncoronary cusp was fused and calcified. VSD was closed with an autologous pericardial patch and composite graft aortic root replacement using direct coronary button reimplantation was performed, and the hypertrophic muscle of the right ventricular outflow tract was resected. The patient had transient weaning failure of cardiopulmonary bypass and was discharged at the postoperative 14 days without any problems.

MeSH Terms

Aortic Valve Insufficiency*
Cardiac Catheterization
Cardiac Catheters
Cardiopulmonary Bypass
Dilatation, Pathologic*
Heart Septal Defects, Ventricular*
Heart Ventricles
Humans
Male
Replantation
Transplants
Tricuspid Valve
Weaning
Young Adult