The Fontan procedure in adults

Setting-Tertiary adult congenital cardiac referral centre. Design-Retrospective cross sectional analysis. Objectives-To report our 20 year experience with adult Fontan operations, a nd to compare late outcome in patients with single ventricle with definitiv e aortopulmonary or cavopulmonary shunt palliation. Patients and main outcome measures-Patients older than 18 years undergoing Fontan operation between I January 1982 and 31 December 1998 were identifie d. Mortality and late outcome were derived from hospital records. These pat ients were compared with a cohort of 50 adults with single ventricle who ha d not undergone a Fontan operation. Results-61 adults, median age 36 years (range 18-47 years), with a median f ollow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 yea rs. Compared with before the Fontan operation, more patients were in New Yo rk Heart Association (NYHA) functional class I or II at the latest follow u p (80% upsilon 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfuncti on at the latest follow up compared with 5% before Fortran (p < 0.001). Arr hythmia increased with time (10% before Fontan upsilon 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the n on-Fontan group at the latest follow up. Conclusion-The Fontan operation in adults has acceptable early and late mor tality. Functional class, systolic ventricular function, atrioventricular r egurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.