Korean J Thorac Cardiovasc Surg.  2009 Feb;42(1):9-13.

The Surgical Management of Hypoplastic Left Heart Syndrome andthe Results of a Fontan Operation

• Chung ES
• Kim WH
• Jeon JH
• Choi CH
• Lee CH
• Lee YT

Abstract

BACKGROUND: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. MATERIAL AND METHOD: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was 17.3+/-10.8 days (range: 9~36 days) at the 1st staged operation, 8.9+/-7.1 months (4.6~23.3 months) at the 2nd staged operation (the Bidirectional Glenn procedure) and 32.4+/-9.8 months at the final staged operation (the Fontan procedure). During the 2nd staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. RESULT: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was 19.6+/-14.9 months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I).
CONCLUSION
All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.

Keyword

Congenital heart disease; Hypoplastic left heart syndrome; Norwood procedure; Fontan procedure

MeSH Terms

Aspirin
Echocardiography
Follow-Up Studies
Fontan Procedure
Hemodynamics
Humans
Hypoplastic Left Heart Syndrome
Medical Records
Norwood Procedures
Polytetrafluoroethylene
Retrospective Studies
Tricuspid Valve
Tricuspid Valve Insufficiency
Ventricular Function
Aspirin
Polytetrafluoroethylene