title: Absent pulmonary valve syndrome: surgical treatment and considerations.
abstract: BACKGROUND
  The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution.
  
  
  METHODS
  Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure.
  
  
  RESULTS
  Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%.
  
  
  CONCLUSIONS
  Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.
authors.affiliation: Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, Paris Sud University, Le Plessis Robinson, France.
authors.forename: F
authors.initials: F
authors.lastname: Godart
authors.forename: L
authors.initials: L
authors.lastname: Houyel
authors.forename: F
authors.initials: F
authors.lastname: Lacour-Gayet
authors.forename: A
authors.initials: A
authors.lastname: Serraf
authors.forename: M
authors.initials: M
authors.lastname: Sousa-Uva
authors.forename: J
authors.initials: J
authors.lastname: Bruniaux
authors.forename: J
authors.initials: J
authors.lastname: Petit
authors.forename: J D
authors.initials: JD
authors.lastname: Piot
authors.forename: J P
authors.initials: JP
authors.lastname: Binet
authors.forename: S
authors.initials: S
authors.lastname: Conte
authors.forename: C
authors.initials: C
authors.lastname: Planché
mesh_terms: D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000601:surgery; D006350:Heart Valve Prosthesis; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008297:Male; D010496:Pericardium / Q000637:transplantation; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000453:epidemiology; D011651:Pulmonary Artery / Q000601:surgery; D018633:Pulmonary Atresia / Q000453:epidemiology / Q000601:surgery*; D011664:Pulmonary Valve / Q000637:transplantation; D011665:Pulmonary Valve Insufficiency / Q000000981:diagnostic imaging / Q000453:epidemiology; D013997:Time Factors; D014694:Ventricular Outflow Obstruction / Q000601:surgery