OBJECTIVES: The purpose of this study was to examine the outcome after the Fontan operation in patients with congenitally corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis (ccTGA-VSD-PS). METHODS: Patient- and procedure-related variables were analysed in 23 patients with ccTGA-VSD-PS operated between April 2003 and April 2015. RESULTS: The mean age was 14.07 ± 6.38 years (range 4-23, median 11 years), with 82% patients being male (19/23). Dextrocardia was present in 52% (12/23) of patients and left superior vena cava was present in 26% (6/23) of patients. Most patients underwent extracardiac Fontan (n = 18), whereas in 5 patients lateral tunnel Fontan was performed. All patients received polytetrafluoroethylene grafts of size 18-22 mm for extracardiac Fontan. In 8 patients, conduits were fenestrated to reduce the intraconduit pressure. The mean hospital stay was 15.7 ± 11.24 days (5-60, median 14 days). The most common cause for prolonged hospital stay was pleural effusion in 5 patients (21.7%). One 7-year old patient developed conduit thrombosis, intracranial bleed, seizures and died. The mean follow-up was 46.4.4 ± 32.2 months (range 8-142, median 42 months) and was available for 21 patients (91.3%). There was 1 mid-term non-cardiac death after 3 years of operation. Of the total, 85.7% (18/21) patients in follow-up are in NYHA class I, whereas 3 patients are in class II. The actuarial event-free survival rate was 81.8 ± 13.2% at 10 years. CONCLUSIONS: In ccTGA-VSD-PS patients with non-routable VSD and in those with difficult options for biventricular repair, the Fontan approach provides satisfactory mid-term palliation.
OBJECTIVES: The purpose of this study was to examine the outcome after the Fontan operation in patients with congenitally corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis (ccTGA-VSD-PS). METHODS:Patient- and procedure-related variables were analysed in 23 patients with ccTGA-VSD-PS operated between April 2003 and April 2015. RESULTS: The mean age was 14.07 ± 6.38 years (range 4-23, median 11 years), with 82% patients being male (19/23). Dextrocardia was present in 52% (12/23) of patients and left superior vena cava was present in 26% (6/23) of patients. Most patients underwent extracardiac Fontan (n = 18), whereas in 5 patients lateral tunnel Fontan was performed. All patients received polytetrafluoroethylene grafts of size 18-22 mm for extracardiac Fontan. In 8 patients, conduits were fenestrated to reduce the intraconduit pressure. The mean hospital stay was 15.7 ± 11.24 days (5-60, median 14 days). The most common cause for prolonged hospital stay was pleural effusion in 5 patients (21.7%). One 7-year old patient developed conduit thrombosis, intracranial bleed, seizures and died. The mean follow-up was 46.4.4 ± 32.2 months (range 8-142, median 42 months) and was available for 21 patients (91.3%). There was 1 mid-term non-cardiac death after 3 years of operation. Of the total, 85.7% (18/21) patients in follow-up are in NYHA class I, whereas 3 patients are in class II. The actuarial event-free survival rate was 81.8 ± 13.2% at 10 years. CONCLUSIONS: In ccTGA-VSD-PS patients with non-routable VSD and in those with difficult options for biventricular repair, the Fontan approach provides satisfactory mid-term palliation.