title: Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age.
abstract: BACKGROUND
  Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion. However, in China, patients with IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus (PDA) over one year of age are common. So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.
  
  
  METHODS
  From January 2009 to December 2012, 19 patients with IAA have undergone complete single-stage repair. The patients' mean age was 4.4 years, ranging 1 to 15 years; and their mean weight was 12.8 kg, ranging 4.2 to 36.0 kg. Fifteen IAA were type A, four were type B. Preoperative cardiac catheterization data were available from all patients. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured. The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case. All patients underwent echocardiographic examinations before discharged from the hospital. In addition, cardiac catheterization and echocardiographic examinations were performed during follow-up. Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients. Mean follow-up was (1.6±0.8) years.
  
  
  RESULTS
  There were two hospital deaths (2/19, 11%). One patient died of pulmonary hypertension crisis, and another died of postoperative low cardiac output. Five cases had other main postoperative complications but no postoperative neurologic complications. Seventeen survivors were followed up, and there were no late deaths or reoperation. Mean cross-clamp duration was (85±22) minutes and selective brain perfusion duration was (34±11) minutes. Two patients required delayed sternal closure at two days postoperatively. Intensive care unit and hospital stays were (9±8) days and (47±24) days, respectively. Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg. mPAP regressed significantly from preoperative (62.1±8.1) mmHg to postoperative (37.3±11.3) mmHg (P < 0.001) and (24.2±6.0) mmHg at six months after discharged from the hospital (P < 0.001). The pulmonary vascular resistance also regressed significantly from preoperative (1 501.4±335.7) dyn×s×cm(-5) to (485.0±215.1) dyn×s×cm(-5) at six months after discharged from the hospital (P < 0.001). The majority of the seventeen patients (89%) were in New York Heart Association (NYHA) class I, and 11% remained in NYHA class II.
  
  
  CONCLUSIONS
  Single-stage repair of patients with IAA, VSD and PDA over one year of age can have good surgical results and functional outcomes. Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial. mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Zhiqiang
authors.initials: Z
authors.lastname: Li
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Bin
authors.initials: B
authors.lastname: Li
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Xiangming
authors.initials: X
authors.lastname: Fan
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Junwu
authors.initials: J
authors.lastname: Su
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: He
authors.affiliation: Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. Email: liucardio@163.com.
authors.forename: Yinglong
authors.initials: Y
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004374:Ductus Arteriosus, Patent / Q000601:surgery*; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology; D007223:Infant; D008297:Male; D012307:Risk Factors; D016896:Treatment Outcome; D014655:Vascular Resistance / Q000502:physiology