The Fontan procedure in adults

Abstract

Setting-Tertiary adult congenital cardiac referral centre. Design-Retrospective cross sectional analysis. Objectives-To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation. Patients and main outcome measures-Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation. Results-61 adults, median age 36 years (range 18-47 years), with a median follow 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 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up. Conclusion-The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle. (Heart 2001;86:330-335)

Management of the Fontan circulation in the adult patient

“…(86)(87)(88) By adult life the gains in ventricular function are largely lost with around a third of reported patients having moderate or severe ventricular function. (89) There are many plausible theories as to why late ventricular function occurs, with increasing systemic vascular resistance, due to abnormal vascular tone, (90) endothelial dysfunction, (91)(92)(93) and the abnormal resistances encountered by the pulmonary and systemic circulations running in series (6) all probably playing a part. The onset of significant valvular incompetence and the onset of atrial arrhythmias are both poorly tolerated.…”

Treatment of Terminal Heart Failure in Grown Up Congenital Heart Disease.

“…Simptomi su u ovakve populacije vrlo česti s oko 25 % pacijenata nakon Fontanove operacije simptomatično deset godina nakon zadnjeg zahvata 13 , 75 % neoperiranih bolesnika s prirođeno ever, a population of patients who, despite fulfilling the defining criterion for HF possibly better than any other group, i.e. that of having an important 'abnormality of the heart', have remained somewhat overlooked with regard to an appreciation of their HF and its treatment.…”

“…Disingenuous behavior of symptoms in patients with grown up congenital heart disease Symptoms are quite frequent in this population with around 25% of Fontan patients symptomatic 10 years following procedure 13 , 75% of unoperated congenitally corrected TGAs present-…”

Operação de Fontan: reflexões da evolução e perspectivas atuais

“…These complications are less frequent in cavopulmonary connections with extracardiac conduits 8 than in intra-atrial lateral tunnel 7,9 . Other complications such as thromboembolism and protein-losing enteropathy are also directly related to the atriopulmonary technique [4][5][6]10 and also when patients undergo surgery in adulthood 6 . These latter complications were less frequent in the cavopulmonary technique 8,9,11,12 , mainly when the extracardiac conduit 8 was used (Tab.…”

“…Among the complications, those with the highest prevalence and repercussion are heart failure and arrhythmias, mainly in atriopulmonary [4][5][6] techniques, but also in cavopulmonary 7 procedures when performed in adulthood. These complications are less frequent in cavopulmonary connections with extracardiac conduits 8 than in intra-atrial lateral tunnel 7,9 .…”