title: Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction.
abstract: OBJECTIVE
  Children with various single ventricle anomalies are at risk of developing systemic ventricular outflow tract obstruction (SVOTO) following volume unloading with cavopulmonary connection (CPC). We aim to evaluate the value of Damus-Kaye-Stansel (DKS) anastomosis at the time of CPC in eliminating late SVOTO risk.
  
  
  METHODS
  Retrospective review of single ventricle patients who underwent DKS concurrent with CPC between 1997 and 2012 was performed. Clinical, echocardiographic and angiographic outcomes were analysed.
  
  
  RESULTS
  Thirty-six children with single ventricle underwent DKS at the time of Glenn bidirectional CPC (n = 29) or Fontan total CPC (n = 7). The underlying anatomy was double inlet left ventricle (n = 18), double outlet right ventricle (n = 8), unbalanced atrioventricular septal defect (n = 4) and other (n = 6). Prior palliation included pulmonary artery band (n = 35), coarctation/arch repair (n = 11) and atrial septectomy (n = 8). Median age at the time of DKS was 8.9 months (range 3.6 months-9.1 years) and the median weight was 6.7 kg (range 5-27 kg). At the time of DKS, 17 patients (47%) had no SVOT gradient and 19 (53%) had SVOT gradient (mean 23.4 ± 18.7 mmHg). Overall survival was 89 and 83% at 1 month and 5 years, respectively. None of the deaths were related to SVOTO or DKS complications. When present, SVOT gradient decreased from 23.4 ± 18.7 mmHg preoperatively to 0 after DKS (P < 0.001). At the last follow-up, none of the patients developed any SVOT gradient; 78% of them had zero or trivial aortic/neoaortic valve regurgitation while 22% had mild regurgitation. None of the patients had evidence of compression of the left pulmonary artery or bronchus. Eighty-one percent of patients have reached or are suitable candidates awaiting final palliative surgery.
  
  
  CONCLUSIONS
  DKS can be safely performed in conjunction with CPC without added mortality risk. It is very effective in mitigating SVOTO risk, with sustainable good semilunar valves function. Our data support an aggressive approach to performing DKS concurrent with CPC in children with single ventricle pathologies at risk of developing SVOTO.
authors: The Heart Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors: Bahaaldin
authors: B
authors: Alsoufi
authors: Abdullah
authors: A
authors: Al-Wadai
authors: Mubashir
authors: M
authors: Khan
authors: Mamdouh
authors: M
authors: Al-Ahmadi
authors: Avedis
authors: A
authors: Kalloghlian
authors: Ziad
authors: Z
authors: Bulbul
authors: Majid
authors: M
authors: Al-Fayyadh
authors: Zohair
authors: Z
authors: Al-Halees
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D018610:Heart Bypass, Right / Q000379:methods / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D016019:Survival Analysis; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000517:prevention & control / Q000601:surgery
keywords: Cavopulmonary connection; Fontan; Single ventricle