Outcome of symptomatic patients undergoing extracardiac Fontan conversion and cryoablation

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 2 patients with protein-losing enteropathy.

Ten patients (mean age 21.1 +/- 7.0 years) underwent extracardiac Fontan conversion, cryoablation, and pacemaker placement between November 1999 and April 2002 (13.1 +/- 4.1 years after the original atriopulmonary connection). Eight patients were in New York Heart Association class III and 2 were in New York Heart Association class IV. Nine patients had clinically important intra-atrial reentry tachycardia refractory to medical therapy.

Follow-up was between 3.1 and 32.6 months (16.8 +/- 9). One death occurred at 7 days after surgery due to sepsis and multisystem organ failure. The second death occurred at 48 days from complications of protein-losing enteropathy. The second patient with protein-losing enteropathy had improved New York Heart Association classification, cessation of albumin transfusions, and a normal stool alpha antitrypsin level (down from 4.1 mg/g preoperatively). Five patients improved to New York Heart Association class I and 3 patients to New York Heart Association class II. Sustained arrhythmias could not be induced in any patient. Seven patients are on no antiarrhythmics. One patient had recurrence of intra-atrial reentrant tachycardia 11 months postoperatively, which required electrical cardioversion; this patient's symptoms are currently well controlled on 1 medication.

Extracardiac Fontan, cryoablation, and pacemaker placement reduced atrial arrhythmias and improved New York Heart Association classification in all surviving patients. 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.