MANAGEMENT OF HYPOPLASTIC AORTIC-ARCH ASSOCIATED WITH NEONATAL COARCTATION

Controversy still exists as to whether hypoplastic aortic arch frequen tly associated with neonatal coarctation, should be enlarged at the ti me of coarctation repair. To determine the indications for and the out come of repair of hypoplastic aortic arch, pre- and postoperative angi ograms/echocardigraphy of 77 cases with isolated (n = 25, Group 1) or complex (n = 52, Group 2)neonatal coarctation operated upon between 1/ 80 and 12/89 were reviewed. Age was 5-14 days (mean 8+/-1.6). Aortic a rch/ascending aorta diameter ratio (AR/AA) as a measure of the degree of aortic arch hypoplasia was 0.39-0.64 (0.52 +/-0.04) in isolated and 0.15-0.47 (0.34+/-00.6) in complex coarctation (p < 0.05). Left subcl avian flap aortoplasty was used in 72 patients; alone in 25, in combin ation with pulmonary artery banding in 43 patients, and simultaneously with intracardiac repair in 4 patients. Extensive reconstruction of a ortic arch and coarctation with synthetic patch was performed in the r emaining 5 patients (AR/AO = 0.16 +/- 0.03) using cardiopulmonary by-p ass at the time of intracardiac repair. Operative mortality was 2/76 ( 2.5%). Follow-up is 6.6 +/- 1.4 years. Recoarctation occurred in 3 pat ients (4%). AR/AA increased to 0.86 +/- 0.1 in isolated (p < 0.05 vs p reoperative) and to 0.7 +/- 0.1 in complex coarctation (p < 0.05 vs pr eoperative). In the majority of cases, hypoplastic aortic arch associa ted with coarctation grows satisfactorily following simple repair of c oarctation with no significant residual narrowing. In patients with se vere arch hypoplasia (AR/AA < 0.2) extended patching of the coarctatio n segment and arch provides an excellent surgical relief of obstructio n at the aortic arch in complex coarctation.