Clinical Outcome of 193 Extracardiac Fontan Patients

Abstract

After 15 years of follow-up, the overall survival, the functional status, and the cardiopulmonary performance of survivors of the extracardiac Fontan procedure compare favorably with other series of patients who underwent the lateral tunnel approach. The incidence of late deaths, obstructions of the cavopulmonary pathway, re-interventions, and arrhythmias is lower than that reported late after other Fontan-type operations.

Transplantation and Mechanical Circulatory Support in Congenital Heart Disease

“…For those whose failure can be attributed to a significant residual anatomic defect, further repair is attractive, especially if the cavopulmonary connection can be "upgraded" to an extracardiac Fontan, given its superior short-and longterm results. [27][28][29][30] For example, in the absence of significant ventricular dysfunction, corrective surgical repair with or without Fontan conversion appeared to confer a low morbidity and mortality in a carefully selected group of patients. 31 If significant ventricular dysfunction is already present, it is questionable that correction of the anatomic defect to recover ventricular function will be an acceptable risk; however, if the defect is in conjunction with an atriopulmonary Fontan (less energy efficient but also uncommon in children in the current era) and atrial arrhythmia is a contributor to the dysfunction, some research would suggest that a Fontan conversion with surgical arrhythmia ablation plus correction of anatomic defects is an alternative to transplantation.…”

Interventional Electrophysiology in Patients With Congenital Heart Disease

“…The incidence of postoperative IART for single-ventricle patients has decreased substantially as a consequence. 77,78 The risk of ventricular arrhythmias also has been reduced in conditions such as tetralogy of Fallot by performing surgery at younger ages with smaller right ventricular incisions and more attention to pulmonary valve competence. 79,80 Therapeutic arrhythmia surgery is used predominantly in the older generation of CHD patients who were repaired with outmoded techniques.…”

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

“…3,4 Several case series have reported surgical mortality rates associated with various approaches. [5][6][7][8][9][10][11][12][13] Valuable data on longer-term actuarial survival are likewise emerging, with a focus on particular surgical techniques 12,14,15 or congenital diagnoses. 16 Causes and distribution of deaths remain poorly defined.…”