Stage II Palliation of Hypoplastic Left Heart Syndrome Without Cardiopulmonary Bypass

Study Questions:

What are the early outcomes for stage II palliation with a bidirectional cavopulmonary anastomosis (BCPA) for hypoplastic left heart syndrome (HLHS) without cardiopulmonary bypass (CPB)?


This was a retrospective review of infants undergoing staged palliation for HLHS from April 2003 to March 2010 at a single institution. The analysis focuses on a select group of 20 patients undergoing the second stage BCPA without CPB following a Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) conduit. Patients were evaluated for feasibility, as well as operative, postoperative, and short-term outcomes. A small subset of patients also underwent neurodevelopmental testing with the Bayley Scales of Infant Development at 1 year.


Of 75 HLHS patients undergoing palliation, 65 had a Norwood procedure with an RV-PA conduit, and 61 patients survived to second stage palliation. Of these survivors, 20 patients had their BCPA performed without CPB. All patients had venous shunting for a median duration of 11 minutes with continuous near-infrared spectroscopy monitoring. Intraoperative blood usage was avoided in 60%. In this select group of patients, there was no hospital mortality. Two patients died at late follow-up, with survivors having no interval interventions at a median duration of 17 months. A control group of patients was identified, who underwent a BCPA with CPB excluding patients who had concomitant procedures. Compared to control patients, off CPB patients were less likely to receive a blood transfusion (p < 0.001), had a significantly shorter hospital stay (5 vs. 10 days, p = 0.001), and had similar neurodevelopmental testing in a small subset of patients (n = 4 on CPB, n = 3 off CPB).


A BCPA can be performed in select patients without the use of CPB. Specifically, the Norwood procedure with an RV-PA conduit brought to the left of the aorta is ideal to stage patients with this approach by establishing antegrade pulmonary blood flow that can be maintained during occlusion of the right PA. The procedure was performed safely with decreased use of intraoperative blood products and shorter hospital stay.


An off CPB BCPA can be accomplished in patients with antegrade pulmonary blood flow, who do not need concomitant procedures such as pulmonary arterioplasty or atrioventricular valve repair. Theoretically, the avoidance of bypass should minimize the complications associated with bypass. However, in this highly select population, the morbidity and mortality associated with bypass is minimal, and the anticipated hospital stay is relatively short. This is a reasonable surgical approach to consider in the appropriate patient population so long as adequate cerebral venous drainage can be ensured during the period of caval occlusion. The study is limited by the small sample size; therefore, comparative safety to established on-pump BCPA cannot be determined.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: Survivors, Norwood Procedures, Follow-Up Studies, Hospital Mortality, Heart Bypass, Right, Heart Defects, Congenital, Child Development, Hypoplastic Left Heart Syndrome, Cardiopulmonary Bypass, Pulmonary Artery

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