title: Neonatal right ventricle to pulmonary connection as a palliative procedure for pulmonary atresia with ventricular septal defect or severe tetralogy of Fallot.
abstract: OBJECTIVE
  Right ventricle to pulmonary artery connection (RVPA connection) without prosthetic material has been our ideal strategy to palliate pulmonary atresia with ventricular septal defect (VSD) or severe tetralogy of Fallot for the last decade. We speculate that RVPA connection ensures adequate postoperative haemodynamics for symptomatic neonates and promotes pulmonary artery rehabilitation. The present study was undertaken to assess the outcome of this strategy.
  
  
  METHODS
  Between 2000 and 2010, among 107 patients who benefited from an RVPA connection, 57 were neonates. Forty-eight of these underwent autologous tissue reconstruction, 5 using left atrial appendage. Median weight was 2.9 kg (range 1.8-4.4). Median Nakata index was 100 mm2/m2 (range 17-185 mm2/m2); 12% had major aortopulmonary collaterals. All patients were reviewed retrospectively. End-points were death or complete repair; reintervention for restrictive pulmonary blood flow was considered as failure. At follow-up, we evaluated reintervention after complete repair, and quality of life.
  
  
  RESULTS
  There were 2 early deaths (RV hypoplasia and RV failure) and 3 late sudden deaths (range 3-6 months). Pulmonary blood flow required to be increased in 8 patients: 4 underwent shunt after a median delay of 1 month; RVPA connection enlargement was needed in 3; 1 patient had percutaneous angioplasty. Finally, 47 patients (81%) had a complete repair, of which 70% were performed without prosthetic material at a median age of 7 months (range 2-53), with a median Nakata index of 221 mm2/m2 (range 102-891). One patient died early and 1 was a failure with opening of the VSD after intracardiac repair. At last follow-up, 4 patients were still awaiting repair, with 1 late death and 5 who had required reintervention after intracardiac repair; there were 3 conduit replacements and 2 balloon dilatation patch enlargements.
  
  
  CONCLUSIONS
  The neonatal RVPA connection approach (i) provides an acceptable survival rate with a satisfactory haemodynamic adaptation, (ii) facilitates rehabilitation of PAs and (iii) avoids the use of prosthetic graft at correction.
authors.affiliation: Department of Pediatric Cardiac Surgery, University Paris Descartes, Sorbonne Paris Cité and Sick Children Hospital, Paris, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Gerelli
authors.forename: Mathieu
authors.initials: M
authors.lastname: van Steenberghe
authors.forename: Bari
authors.initials: B
authors.lastname: Murtuza
authors.forename: Mirela
authors.initials: M
authors.lastname: Bojan
authors.forename: Ekoué Diana
authors.initials: ED
authors.lastname: Harding
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.forename: Pascal R
authors.initials: PR
authors.lastname: Vouhé
authors.forename: Olivier
authors.initials: O
authors.lastname: Raisky
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D002315:Cardiopulmonary Bypass; D005260:Female; D006343:Heart Septal Defects / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods*; D011183:Postoperative Complications; D018633:Pulmonary Atresia / Q000601:surgery*; D056346:Sternotomy; D013771:Tetralogy of Fallot / Q000601:surgery*
keywords: Major aortopulmonary collaterals; Neonatal; Palliative surgery; Pulmonary atresia with ventricular septal defect; Surgical management; Tetralogy of Fallot with pulmonary atresia