• V M Reddy and F L Hanley.
• Division of Cardiothoracic Surgery, University of California, San Francisco, USA.
• Semin. Pediatr. Surg. 2000 May 1;9(2):91-5.

AbstractCongenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative surgery, and definitive repair is delayed. However, the morbidity of such an approach has been shown to be high. Recent reports emphasize early surgical repair with good results in low birth weight and premature babies. However, there are no data on the outcomes in infants with very low birth weight (<1,500 g). Since 1990, the authors performed complete repair of congenital heart defects (other than isolated patent ductus arteriosus) in 20 infants weighing 1,500 g or less. Defects included aortic coarctation (n = 6), ventricular septal defect (n = 5), tetralogy of Fallot (n = 2), transposition of great arteries (n = 2), truncus arteriosus (n = 2), total anomalous pulmonary venous return (n = 1), double outlet right ventricle (n = 1), and pulmonary atresia with intact ventricular septum (n = 1). Preoperative morbidity was more common in patients who were referred late for corrective surgery. Modification of techniques of neonatal cardiopulmonary bypass were used. There were 2 early deaths (10%) caused by foot gangrene (n = 1), and pulmonary complications (n = 1). No patient had evidence of postbypass intracranial hemorrhage. At a median follow-up of 40 months, there was 1 late death, and 4 patients underwent a total of 5 surgical and catheter reinterventions. There was no evidence of neurological sequelae attributable to surgery. In most cases, delaying repair of congenital heart defects in low and very low birth weight infants does not confer any benefit and is associated with a higher incidence of preoperative morbidity. Complete repair of both simple and complex congenital heart lesions can be performed successfully in such patients with good early and medium-term results. Postoperative growth after repair approximates the normal growth curve for low birth weight infants without congenital heart disease. It is recommended that with few exceptions, such infants, especially when symptomatic, undergo early surgical correction rather than prolonged medical management or other forms of palliation.

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