Objective : From June 1995 to January 2003, 49 consecutive neonates of less than 2500 g underwent early surgery for congenital heart disease. A retrospective analysis was performed to evaluate the early to medium-term outcome.
Patients and methods : Major cardiac surgery for congenital heart defects included a complete correction in 31 patients (group I) and a palliative procedure in 18 patients (group II). Mean age at operation was 15,2 days (1d-90 d) and mean weight was 2190 g (1300g-2500 g). Twenty-four children (49 %) were born prematurely. All neonates were critically ill (NYHA III-IV) and 47 % were ventilated preoperatively.
Heart defects included mainly ventricular septal defect (11), tetralogy of Fallot complexes (8), aortic coarctation (9), transposition complexes (7), single ventricle anomalies (4), pulmonary atresia with intact septum (4), interrupted aortic arch (3) and total anomalous pulmonary venous return (3).
Results : Overall surgical mortality was 18 % : 4 neonates died after primary repair and 5 after palliation, representing respectively 13 % and 28 % of each group. Postoperative morbidity occurred in half of the patients (53 %). Age, weight, prematurity, type of surgical procedure and use of cardiopulmonary bypass did not influence the early outcome. After a mean follow-up of 2,82 years (2 months to 6 years), overall survival was 76 % (87 % in the primary repair group and 54 % in the palliation group). All children were in NYHA class I-II. Freedom from reintervention at 18 months was 68 % after correction versus 8 % after palliation.
Conclusions : Cardiac surgery for congenital malformations in critically ill, low weight neonates can be achieved with acceptable mortality, at the cost of an increased morbidity. In the presence of favourable anatomical features, even for complex anomalies, low weight per se should not preclude complete correction in favour of prolonged medical therapy or eventual palliation. Moreover, primary repair appears to result in an early survival benefit, remaining constant over time.