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.

FIGURE 1

Operative technique. See text for details.

FIGURE 2

Flow diagram of the cohort of patients having undergone a Norwood procedure and the subsequent study population of patients who were palliated with bidirectional cavopulmonary anastomosis (BCPA) without cardiopulmonary bypass (CPB) from April 2003 to March 2010. CI, Confidence interval.

FIGURE 3

Intraoperative near-infrared spectroscopy as an indicator of cerebral oxygenation during off-pump bidirectional cavopulmonary anastomosis. A, Baseline near-infrared spectroscopic oximetry in an infant with hypoplastic left heart syndrome who had a right ventricle–pulmonary artery conduit modification of the Norwood procedure. The procedure was performed after achievement of general anesthesia for stage II palliation. B, The fraction of inspired oxygen is increased to 1, and cerebral oxygen saturation increases from 50% to 65%. C, The right pulmonary artery is controlled, and the superior vena caval–right atrial venous shunt is inserted. The superior vena cava is snared. The cerebral oxygen saturation decreases to 50%. D, The bidirectional cavopulmonary anastomosis is complete, and the caval snares and right pulmonary artery clamps are released. The cerebral oxygen saturation increases to 60%. E, The single right ventricle is retracted, and the right ventricle–pulmonary artery conduit is dissected, encircled, and ligated and divided. F, The cardiac retraction is terminated. G, The bidirectional cavopulmonary anastomosis with division of the right ventricle–pulmonary artery conduit is complete.