Extracardiac Fontan conversion, cryoablation, and pacemaker placement for patients with a failed Fontan

We review our experience with Fontan conversion and cryoablation in patients with an atriopulmonary Fontan in low cardiac output from arrhythmia or venous obstruction, including two patients with protein losing enteropathy.

Fifteen patients (mean age 25.0 +/- 8.4 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and December 2004. Twelve patients were in NYHA class III and three were in NYHA class IV. Twelve had clinically important intraatrial reentry tachycardia refractory to medical therapy.

Follow-up was between 2 and 62 months (mean 38.4 +/- 17.7). One death occurred at seven days after surgery due to sepsis and multisystem organ failure. The second death occurred at five days from myocardial depression following surgery. One patient with PLE preoperatively died to malnutrition and sepsis on POD number 52. The second patient with protein losing enteropathy had improved NYHA classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). All surviving patients improved NYHA classification to class I or II. Sustained arrhythmias could not be induced in any patient. One patient had recurrence of intraatrial reentrant tachycardia eleven months postoperatively that required electrical cardioversion and is currently well controlled on one medication. The other patients are not on any antiarrhythmic medical therapy.

Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved NYHA classification. In selected patients, this operation offers improvement in clinical outcome and is an alternative to transplantation. Protein losing enteropathy may not be a contraindication to performing Fontan conversion with cryoablation.