Stage II palliation of hypoplastic left heart syndrome without cardiopulmonary bypass

Abstract

Objectives: Bidirectional cavopulmonary anastomosis has been performed without cardiopulmonary bypass for some single-ventricle heart defects. Limited data are available for the outcomes of off-pump bidirectional cavopulmonary anastomosis in infants with hypoplastic left heart syndrome. The purpose of this study is to determine the early outcomes for stage II palliation of hypoplastic left heart syndrome without cardiopulmonary bypass.

Methods: This is a retrospective review of infants having surgical palliation of hypoplastic left heart syndrome from April 2003 to March 2010 at a single institution.

Results: Seventy-five infants had a modified Norwood procedure, 65 with a right ventricle-pulmonary artery conduit, 10 with an aortopulmonary shunt, 2 with atrioventricular valve repair, and 3 with extracorporeal life support. Sixty-eight patients had hypoplastic left heart syndrome or one of its variants, and 7 had other single-ventricle lesions. There were 2 stage I deaths. Stage I survival was 97% (95% confidence interval, 88%-99%). Another 5 infants succumbed in the interstage period. Of the 68 stage I and interstage survivors, 61 had bidirectional cavopulmonary anastomoses, 20 without cardiopulmonary bypass. Median age was 6 months (range, 4-13 months), and median weight was 6.1 kg (range, 5.2-9.0 kg). There were no conversions to cardiopulmonary bypass when off-pump bidirectional cavopulmonary anastomosis was attempted. There were no hospital deaths. Median ventilation duration was 10 hours (range, 6-18 hours), and length of stay was 5 days (range, 4-9 days). Follow-up was available on all infants at a median duration of 17 months (range, 3-43 months), with no unplanned reinterventions.

Conclusions: Bidirectional cavopulmonary anastomosis without the use of cardiopulmonary bypass can be performed safely and with low mortality for selected infants with hypoplastic left heart syndrome. Midterm to long-term outcomes remain to be determined.