Abstract

Background: Recurrent coarctation of aorta (reCoA) after Norwood operation might result in impaired cardiac function and the following poor prognosis in HLHS and its variants. The purpose of this study was to evaluate the initial arch repair technique as well as the outcome of aggressive treatment for re-CoA. Patients and methods: Twenty-four (24) reCoA patients after 80 Norwood operations between 1993 and 2012 were evaluated (30.8%, classical HLHS: 20, variants: 4). Initial procedure was primary Norwood in 11 (BT: 4, RV-PA: 7) and bilateral PAB in 13 (modified Norwood: 8, Norwood+BDG: 5). Arch repair technique at Norwood operation was direct anastomosis in 14, patch augmentation in 10. Results: Interval from Norwood to reCoA was 7 - 538 day (median: 102 day), and age at operation was not related with reCoA. Balloon aortoplasty (PTA) was performed in 22 patients, totally 31 times, and initial PTA resulted in the significant decrease in pressure gradient (PG) between AAo and DAo from 14.7±13.5 mmHg to 6.6±10.9 mmHg (P=0.04). PTA non-effective cases underwent stent implantation (2) or surgical intervention (10; patch plasty in 9, SCF in 2). Timing of surgery was pre BDG in 1, at BDG in 5, after BDG in 4, and the material of patch plasty was Xenopatch in 2, Hemashield graft in 1 and ePTFE in 6. There was significant decrease in pressure gradient from 28.0±14.7 mmHg to 5.1±4.3 mmHg (P<0.01). Two patients underwent surgery without PTA and other 2 patients underwent additional PTA after surgical repair. At present, 16 patients reached TCPC and 4 are awaiting TCPC. There were 4 late deaths after BDG with CHF (3) or pneumonia (1), but reCoA was not related with late death (PG: 0-4 (2) mmHg). The surviving 20 patients revealed RVEDV of 145.7±50.7%ofN, RVEF of 55.8±10.3%, PG of 0-12 (1) mmHg and BNP of 5.8-144.8 (16.8) pg/ml. Regarding the initial arch repair technique, patch enlargement at lesser curvature and swing-back technique are related with less reCoA (P<0.05). Conclusions: Re-CoA after Norwood operation was often recognized. Patch enlargement at lesser curvature or swing-back technique might be useful for arch reconstruction. Aggressive intervention for re-CoA in combination of balloon plasty and surgical repair could result in good cardiac function and the following excellent long-term outcome.

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