Surgery for congenital heart disease in low-birth weight neonates: a comprehensive statewide Connecticut program to improve outcomes.

Low-birth weight (LBW) remains a significant risk factor in surgery for congenital heart disease (CHD). The timing of surgery and the choice of complete repair vs palliative measures are controversial issues. Delay of surgery to achieve weight gain may result in poorer outcomes. The results of a statewide, cardiac surgery protocol in LBW infants using specific selection criteria for repair vs palliation or delay over a 42-month period are reviewed. From September 1999 through February 2003, 32 low-birth weight infants (1,320 to 2,500 grams) underwent surgery for congenital heart disease in Connecticut. The congenital heart surgery programs in the two major pediatric centers in the state (Yale-New Haven Hospital and Connecticut Children's Medical Center) were amalgamated to form a joint program, with the same surgical team involved in all cases. Median gestational age was 34 weeks with 18 (60%) premature (< or = 37 wks). Median age at operation was eight days. Fourteen infants (44%) had recognized syndromes. Primary diagnoses included variant of single ventricle (8), ventricular septal defect (VSD) (4), tetralogy of Fallot (TOF) (2), pulmonary atresia (PA) with VSD (3), simple transposition of the great arteries (TGA) (1), TGA with VSD (TGA/VSD) (2), atrioventricular canal (AVC) (4), double outlet right ventricle (DORV) (3), aortic coarctation (CoA) (2), and interrupted aortic arch with VSD (IAA/VSD) (3). Eighteen patients (56%) were able to undergo procedures resulting in normal biventricular physiology. Eight patients (25%) had palliation for single ventricle, including two Norwood procedures. Seven patients potentially suitable for biventricular repair underwent palliative surgery because of contraindications to complete repair that were unrelated to weight. Follow-up (100%) ranged from two to 41 months. There were two early deaths (6%), one cardiac related (Norwood with ischemia), and one with sepsis. There was one early reintervention (PA stenosis following arterial switch). There were seven late deaths (22%), two cardiac related (one sudden death following Blalock-Taussig (BT) shunt, one sepsis related to low output), and four noncardiac. In three patients who received pulmonary artery banding (PAB), delay of surgery may have contributed to death due to progressive pulmonary disease. Of 23 long-term survivors, five have undergone biventricular repair following palliation, and two have had further palliation for univentricular heart physiology. A strategy of early surgical intervention favoring primary repair, or surgical palliation for those patients with single ventricle, results in good overall survival in symptomatic low-birth weight neonates with congenital heart disease. Delay in surgery due to LBW may not be beneficial in most cases and could result in lower overall survival and increased cost. A program utilizing statewide resources results in excellent outcomes for this challenging group of patients.