Outcomes of total correction of congenital heart malformations in the neonatal period

Director, Pediatric Cardiac Transplantation, University of Michigan Medical Center, USA
Richard G. Ohye

Controversy remains regarding the role of palliation and staged repair versus early primary repair for neonates with complex congenital malformations. The primary argument for staged repair has been that the mortality is lower. Unfortunately, palliation fails to correct the underlying pathophysiology, and includes the risks of the palliative procedure, the interstage period and the definite correction. In addition, the ongoing pathophysiology, including volume overload, pressure overload and cyanosis, has demonstrated and theoretical detrimental effects on the developing infant. Improvement in the outcomes for neonates undergoing complete repair have called into question a continued role for palliation.
At the C. S. Mott Children's Hospital of the University of Michigan, we have maintained a policy of aggressive primary repair for the majority of malformations, regardless of the age at presentation. Palliation is reserved primarily for patients who are in poor clinical condition, or may be at high risk for anticoagulation associated with cardiopulmonary bypass.
We examined our results for the biventricular repair of aortic atresia/hypoplasia and VSD. Patients were initially staged with a Norwood procedure prior to undergoing a complete repair. Over time, a few patients were done by primary repair. These patients tended to be the highest risk patients, such as the premature, the very low birth weight or those presenting in extremis, who we felt would not tolerate the shunt-dependent physiology of the Norwood. We found that despite being the sickest patients, they had equivalent outcomes, and primary complete repair has become our standard.
Similarly, when we evaluated our results for 10 patients undergoing a neonatal or infant Ross/Konno procedure, we found a survival of 100%. At a median follow-up of 48 months, there are no patients with any type of LVOTO, 8 patients with 0-1+ AI and one patient each with 2+ and 3+ AI. We feel that the Ross/Konno procedure is an ideal operation for the infant or neonate with significant aortic valve and LVOT disease.
The proper treatment for the neonate with symptomatic TOF remains particularly contentious. We reviewed 61 neonates undergoing TOF repair, including those with pulmonary atresia. Hospital survival was 98% (60/61) with a mean length of stay of 19 ± 27 days. There were 4 late deaths at a median follow-up of 62 months, for a 5-year actuarial survival of 93%. These results are as good, or better than any published for stage repair.
Our management of AVSD involves primary repair at the time of the development of intractable symptoms or electively at 3-6 months. A review of 132 patients undergoing AVSD repair revealed that 98% (130/132) of patients initially presenting to our institution underwent primary repair. Median age was 4.7 months and median weight was 4.9 kg. Ninety-nine patients (75%) underwent initial definitive repairs under 6 months of age, including 27 under 3 months and 5 in the neonatal period. Hospital survival was 97% (127/132) with two late deaths for a 5-year actuarial survival rate of 93%. No correlation could be shown between age at repair and survival, AV valve function or need for reoperation.