Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

Abstract

Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease

“…The Fontan revision carries an operative mortality rate of 5% to 25% in reported series. 636,640 If PLE is due to Fontan pathway obstruction, successful revision of the Fontan communication may be curative. PLE often requires heart transplantation, 640 although the PLE does not always resolve.…”

Enteropatia perdedora de proteínas após cirurgia de Fontan

“…Thirty-one patients reoperated after the Fontan procedure were recently reported by Marceletti et al 34 , most of them due to arrhythmia and heart failure. Three of these patients had PLE, two of whom improved after extracardiac insertion of a polytetrafluoroethylene (PTFE) valveless tube connecting the inferior vena cava to the right pulmonary artery, associated to the reduction of the right atrium.…”

“…This study highlighted the importance of some variables associated with PLE and became an important reference for the Fontan operation 12 . The incidence of PLE following the Fontan operation has been reported as 2.5 to 10% 23,[31][32][33][34] , but always in studies involving a small number of patients. In a large multicenter study published in 1998 and involving 3,029 patients, PLE was diagnosed in 114 cases (3.7%).…”

Management of the Fontan circulation in the adult patient

“…Once demonstrated the management is difficult, routine anticoagulation with low molecular weight heparin and oral anticoagulation for those not routinely anticoagulated may be sufficient. Case reports have described successful outcomes with catheter delivered thrombolysis (52) and even surgical embolectomy (53,54) and Fontan conversion surgery, (55,56) however, a large occlusive thrombus is frequently fatal, regardless of management strategy. (53,57) (58,59) These narrowed parts of the Fontan circuit would typically be addressed in the catheter laboratory with balloon dilatation or stent implantation in the older child and adult.…”

“…(151) In carefully selected patients, the operation is associated with a low operative mortality. (151,152) …”