Surgical Treatment of Complications after Fontan Operation

Park, Jeong Jun; Hong, Jang Mee; Kim, Yong Jin; Lee, Jeong Ryul; Rho, Joon Ryang

Abstract

BACKGROUND: The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. MATERIAL AND METHOD: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8+/-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1. RESULT: There were 3 early and late deaths respectively. Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1). In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia.
CONCLUSION
Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However,more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class,Although the results were not satisfactory enough in all patients.