title: Long-term outcome of surgically repaired unilateral anomalous pulmonary artery origin.
abstract: The long-term outcome after surgical reimplantation of a unilateral congenitally anomalous pulmonary artery (UCAPA) origin is inadequately described. We performed a single-center retrospective review of patients with pulmonary artery (PA) sling or anomalous origin of 1 PA from the ascending aorta who underwent surgical repair from 1980 to 2006. Baseline data were available for 20 patients with PA sling and 29 patients with anomalous origin of 1 PA from the ascending aorta. Follow-up data for 38 patients, representing 190 cumulative patient-years, were available. Of 28 (74%) patients who developed pulmonary artery stenosis (PAS), 17 (45%) patients underwent a first intervention for PAS, and 9 (24%) patients underwent a second intervention. Median time from surgical repair to diagnosis of PAS was 0.4 years (95% confidence interval [CI] 0, 0.9). Median time from diagnosis of PAS to first intervention was 2.7 years (95% CI 0.07, 5.4). In the 29 patients initially repaired at our center, no first intervention occurred later than 2.8 postoperative years. Median time from first to second intervention was 2 years (95% CI 0, 4.8). Percutaneous intervention for postoperative PAS resulted in acute hemodynamic and anatomic improvement with modest risk profile. On multivariate analysis, recent surgical repair (after 1993) was a risk factor for PAS (p = 0.03, hazard ratio [HR] 1.9), and operative first intervention (vs. percutaneous) was a risk factor for second intervention (p = 0.05, HR 11.2). After reimplantation of a UCAPA origin, PAS is frequent and occurs early postoperatively. Close follow-up after repair is necessary.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, MA, USA. bryangol@med.umich.edu
authors.forename: Bryan H
authors.initials: BH
authors.lastname: Goldstein
authors.forename: Lisa
authors.initials: L
authors.lastname: Bergersen
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
authors.forename: Dionne A
authors.initials: DA
authors.lastname: Graham
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.forename: Peter
authors.initials: P
authors.lastname: Lang
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery*; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome