Achievements and Limitations of a Strategy of Rehabilitation of Native Pulmonary Vessels in Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

摘要

Background A strategy of rehabilitation for pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) comprises repetitive shunting and patching procedures of the central pulmonary arteries. We wanted to determine the feasibility and limitations of a strategy of rehabilitation.;Methods The outcomes of 37 consecutive patients operated from June 2003 to December 2014 for PA/VSD/MAPCAs were reviewed. The patients were directed to a rehabilitation strategy, except when they presented in heart failure with very large collaterals.;Results Four patients with very large MAPCAs underwent a one-stage repair with unifocalization of collateral vessels at a median age of 8.6 months. There was no mortality in this group after a median follow-up of 4.6 years. Following a strategy of staged rehabilitation, 33 patients had 2.01 ± 0.9 procedures before repair. Median age at primary shunting was 3.3 weeks (0.4 to 31.9 weeks). Repair rate was 73% (22 patients), at a median age of 1.7 years. Three patients (10%) were left palliated and 3?patients (10%) died. Median follow-up in this group was 4.5 years. Complementary procedures to the rehabilitation strategy consisted in pulmonary artery reconstruction in 25 patients (76%) and MAPCAs ligation in 7?patients (21%). Pulmonary balloon angioplasty was required in 12 patients (36%) and MAPCAs coil occlusion in 8 patients (24%).;Conclusions A strategy of rehabilitation can be implemented in almost 90% of the cases, with a low mortality rate. Following this strategy, 73% of the patients can be successfully repaired.;;Dr d’Udekem discloses a financial relationship with MSD and Actelion.;The design of the study was approved by the Royal Children's Hospital human research ethics committee. The records of all consecutive neonates with PA/VSD/MAPCAs diagnosed between June 2003 and December 2014 were reviewed and follow-up was obtained from the hospital database. Forty patients were operated on for PA/VSD/MAPCAs in our institution during this period. All patients, except those in heart failure with large MAPCAs, were entered into the rehabilitation strategy. Three patients with late overseas referral were excluded from the study. Four patients underwent a one-stage repair and 33 were directed into a rehabilitation pathway. One patient had a congenitally corrected transposition of the great arteries. The following extracardiac anomalies were noted: craniosynostosis (1 patient), bilateral undescended testes (1 patient), right kidney cystic dysplasia (1 patient), horseshoe kidney (1 patient), butterfly kidney (1 patient), a left Wilms tumor (1 patient) and left-sided hydronephrosis (1 patient).Jump to SectionPatients and MethodsPreoperative InvestigationsPrimary Shunting Procedures in the Strategy of RehabilitationFurther Shunting ProceduresPAs Reconstruction and Additional ProceduresComplete RepairStatistical AnalysesResultsOne-Stage Repair as First InterventionRehabilitation StrategyMortality After RehabilitationRepair After RehabilitationAdditional Procedures in a Strategy of RehabilitationFailure of RehabilitationCommentLimitations and StrengthsConclusionReferences;The diagnosis was obtained by echocardiography and delineation of the anatomy was mainly specified by CT angiography in our center. In recent times, catheterization before the first intervention has been restricted to rare patients to further specify the anatomy of the vessels.;The first stage of the rehabilitation of the PAs consisted most frequently in a central shunting (Fig 1Fig 1A). The size of the shunt was 3 mm in 8 patients, 3.5 mm in 17 patients, and 4 mm in 2 patients. The alternative procedures used to promote the growth of the native PAs were: the modified Blalock-Taussig (MBT) shunt and a right ventricle-to-pulmonary artery (RV-PA) conduit. These procedures were performed through a midline sternotomy. A Gore-Tex vascular graft (W. L. Gore & Associates, Flagstaff, AZ) was used for all of t