title: Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes.
abstract: OBJECTIVE
  Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis.
  
  
  METHODS
  We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients.
  
  
  RESULTS
  There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required.
  
  
  CONCLUSIONS
  The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.
authors.affiliation: Division of Pediatric Cardiac Surgery, Lucile Packard Children's Hospital/Stanford University School of Medicine, Stanford, CA, USA.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Monge
authors.forename: Richard D
authors.initials: RD
authors.lastname: Mainwaring
authors.forename: Ahmad Y
authors.initials: AY
authors.lastname: Sheikh
authors.forename: Rajesh
authors.initials: R
authors.lastname: Punn
authors.forename: V Mohan
authors.initials: VM
authors.lastname: Reddy
authors.forename: Frank L
authors.initials: FL
authors.lastname: Hanley
mesh_terms: D016738:Alagille Syndrome / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D001157:Arterial Occlusive Diseases / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D002140:California; D002315:Cardiopulmonary Bypass; D003251:Constriction, Pathologic; D006439:Hemodynamics; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000637:transplantation*; D011859:Radiography; D019651:Plastic Surgery Procedures / Q000009:adverse effects / Q000401:mortality; D012189:Retrospective Studies; D056346:Sternotomy; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D017725:Ventricular Pressure; D018980:Williams Syndrome / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*