Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Abstract

Background-To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children's Hospital Boston. Methods and Results-A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium-to-pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure-related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; Pϭ0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; Pϭ0.0002

Management of the Fontan circulation in the adult patient

“…Even micro thrombi in the Fontan pathway can, in time, lead to an increase in the pulmonary vascular resistance and large thrombi can occlude the circuit with fatal consequences. (18,19) In addition many patients have a right to left shunt and the risk of a paradoxical embolus is present. For these reasons we routinely anticoagulate our patients with Warfarin with a target INR of 2 to 2.5.…”

“…Yet the majority of these deaths were peri-operative and in survivors, 20 year survival rates were 82%. (19) It is possible that surgical modifications will lead to improvements in early and late survival but that remains unproven. However, the success of the Norwood palliation for HLHS will also impact on these survival figures.…”

Letter by Burchill et al Regarding Article, “Enalapril in Infants With Single Ventricle: Results of a Multicenter Randomized Trial”

“…Systolic heart failure is a late manifestation in patients who have completed their Fontan surgery, associated with high morbidity, mortality, and few effective medical treatment options. 5 Given the high mortality associated with heart transplantation in patients with a Fontan circulation, we share the Pediatric Heart Network's enthusiasm for identifying effective medical treatments in this growing population.…”

Transcatheter Retrograde Device Closure of an Isolated Pulmonary Valve Stump Using a Deflectable Sheath Technique

“…The surgical palliation of children with univentricular hearts without obstruction to pulmonary outflow consists of either Damus-Kaye-Stansel anastomosis of the two great vessels in the setting of potentially obstructed systemic outflow or division of the pulmonary artery leaving the aorta as the sole outflow pathway if there is no potential for subaortic obstruction [1]. If the latter approach is elected, the technique of pulmonary artery division must minimize the potential for thrombus formation in the blind ending pulmonary artery stump and subsequent risk for systemic embolic events [2,3,4].…”