title: Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature.
abstract: BACKGROUND
  Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome.
  
  
  METHODS
  109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated.
  
  
  RESULTS
  Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%).
  
  
  CONCLUSIONS
  Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.
authors.affiliation: Deutsches Kinderherzzentrum Sankt Augustin, Sankt Augustin, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Murin
authors.forename: Nicodème
authors.initials: N
authors.lastname: Sinzobahamvya
authors.forename: Hedwig Ch
authors.initials: HCh
authors.lastname: Blaschczok
authors.forename: Joachim
authors.initials: J
authors.lastname: Photiadis
authors.forename: Christoph
authors.initials: C
authors.lastname: Haun
authors.forename: Boulos
authors.initials: B
authors.lastname: Asfour
authors.forename: Viktor
authors.initials: V
authors.lastname: Hraska
mesh_terms: D000015:Abnormalities, Multiple; D001013:Aorta, Thoracic / Q000002:abnormalities / Q000601:surgery*; D001028:Aortopulmonary Septal Defect / Q000401:mortality / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002315:Cardiopulmonary Bypass; D005260:Female; D005858:Germany; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012086:Reoperation; D013997:Time Factors; D016896:Treatment Outcome