[One-stage repair of the interrupted aortic arch associated with cardiac anomaly]

To evaluate the clinical experiences of one-stage repair of the interrupted aortic arch (IAA) associated with cardiac anomaly in neonates and infants.

From July 1996 to October 2008, 48 neonates or infants with IAA associated with cardiac anomaly underwent one-stage repair. There are 30 males and 18 females ranging from 0.08 to 7.00 years old (1.97 +/- 2.05) with body weight 3 - 20 (9 +/- 5) kg. 42 patients had the middle-severe pulmonary hypertension (PH), the pulmonary pressure was 61 - 106 (82 +/- 14) mm Hg and the pulmonary arterial resistance was 66 - 762 (315 +/- 259) dynxsxcm(-5). There are 40 patients with type A IAA and 8 patients with type B IAA, all patients had an associated patent ductus arteriosus, other combined malformations included ventricular septal defect (VSD) in 33 cases, atrial septal defect (ASD) in 8 cases, mitral valve insufficiency in 6 cases, tricuspid valve insufficiency in 5 cases, aortopulmonary window in 4 cases, double outlet of the right ventricule in 3 cases, mitral valve stenosis in 2 cases, and et al. the operation was performed through median sternotomy under the general anesthesia and cardiopulmonary bypass (CPB, the deep hypothermia with circulatory arrest, the deep hypothermia with low flow rate, or the deep hypothermia with low flow rate and circulatory arrest), the interrupted aortic arch repair was achieved with the arch anastomosis in 30 patients and with an interposition graft in 18 patients, the concomitant operations included VSD repair in 33 cases, ASD repair in 8 cases, mitral valve plasty in 7 cases, tricuspid valve plasty in 4c ases, aortopulmonary window repair in 4 cases, intraventricular baffle tube repair in 2 cases, arterial switch operation in 1 case, and et al.

The time of the CPB and the aortic occlusion were 112 to 375 min (182 +/- 52) and 24 to 287 min (99 +/- 45) respectively. The endotracheal intubation time were 8 to 936 hours (179 +/- 133) and the ICU stay were 1 - 57 days (14 +/- 14). By echocardiograph examination after operation, the pressure gradient across the arch was obvious relieved and the combined cardiac malformations was satisfied corrected. 3 patients (3/48, 6.25%) died during the perioperative period. The cause of death included severe pulmonary hypertension crisis, dysfunction of the pulmonary or the serious low cardiac output syndrome. the main complications included reopen for bleeding in 2 cases, tracheostomy in 2 cases, the right diaphragma paralysis in 1 case, delayed sternal closure in 1 case, hydrothorax in 1 case, the severe pulmonary infection in 1 case. Physical activities of the survivals increased obviously and discharged from hospital uneventfully, all surviving patients had no late complications and death during the follow-up 3 months to 12 years.

One-staged primary repair of IAA with other intracardiac anomalies can be the preferred surgical approach and safely applied with good results as to lead a better life.