Outcomes of 1½- or 2-ventricle conversion for patients initially treated with single-ventricle palliation

Abstract

Objective: As outcomes for the Fontan procedure have improved, it has become more difficult to select between a single-ventricle repair or biventricular repair for patients with complex anatomy and 2 ventricles. However, late complications after the Fontan procedure remain a concern. Our strategy, which has favored an aggressive preferential approach for biventricular repair in these patients, has also been applied to patients initially treated on a single-ventricle track elsewhere.

Methods: Nine patients (4 male patients) who had previously undergone the Fontan procedure (n=3) or bidirectional cavopulmonary shunting (n=6) with intent for a later Fontan procedure were referred to our center for complex 1½- or 2-ventricle repair over the last 10 years. Indications for conversion in these patients were protein-losing enteropathy (n=2), pulmonary arteriovenous malformation (n=1), and preference for biventricular anatomy (n=6). The conversion mainly consisted of takedown of the Fontan procedure or bidirectional cavopulmonary shunt connection, reconstruction of 1 or both of venae cavae, creation of an intraventricular pathway for left ventricular output, and placement of a right ventricle-pulmonary artery conduit (Rastelli-type operation).

Results: Five patients underwent 1½-ventricle repair, and 4 had complete biventricular repair. Median cardiopulmonary bypass and aortic crossclamp times were 202 minutes (range, 169-352 minutes) and 129 minutes (range, 100-168 minutes), respectively. There were 2 early deaths and 1 late death. At a median follow-up of 27 months (range, 3.3-99.8 months), all survivors are in New York Heart Association class I.

Conclusions: Patients initially treated with intent to perform single-ventricle palliation can be converted to 1½- or 2-ventricle physiology with acceptable outcomes.