The fontan procedure : Contemporary techniques have improved long-term outcomes

• Published in: Circulation (New York, N.Y.) N.Y.), 2007-09, Vol.116 (11), p.I157-I164
• Main Authors: D'UDEKEM, Yves, IYENGAR, Ajay J, COCHRANE, Andrew D, GRIGG, Leeanne E, RAMSAY, James M, WHEATON, Gavin R, PENNY, Dan J, BRIZARD, Christian P
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Child; Child, Preschool; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; Female; Follow-Up Studies; Fontan Procedure - methods; Fontan Procedure - mortality; Fontan Procedure - trends; Hospital Mortality - trends; Humans; Male; Medical sciences; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Survival Rate; Time; Treatment Outcome; Vascular surgery: aorta, extremities, vena cava. Surgery of the lymphatic vessels
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.106.676445

To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection. Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12+/-6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed. The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.