title: Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty.
abstract: Radiofrequency perforation and valvuloplasty (RFV) is an effective initial treatment in patients with pulmonary atresia and intact ventricular septum (PA-IVS) and mild to moderate right ventricle and tricuspid valve hypoplasia. Outcomes and risk factors for the need for additional interventions in these patients are poorly defined. All patients with PA-IVS who underwent RFV at our center between January 2000 and July 2011 were reviewed. Twenty-three patients met the inclusion criteria. All patients underwent successful valvuloplasty with no procedural deaths and one major complication. Excluding two patients with limited follow-up, 6 (29 %) patients underwent no subsequent interventions, whereas 9 (42 %) patients underwent surgical right-ventricular outflow tract augmentation. All except one patient with adequate follow-up have a biventricular circulation with saturation >92 %. Patients who did not undergo any right-ventricular outflow tract intervention after valvuloplasty had a significantly lower gradient across the pulmonary valve after valvuloplasty (9.9 mmHg ± 8.4 vs. 19.1 mmHg ± 10.4, p = 0.05). Significantly more patients who received a supplemental source of pulmonary blood flow had a tricuspid valve z-score <-0.7 compared with patients who did not receive supplemental blood flow [2 (15 %) vs. 7 (70 %), p = 0.008]. In our cohort of patients with PA-IVS, radiofrequency perforation with valvuloplasty was an effective and safe first step in establishing a biventricular circulation. Postvalvuloplasty pulmonary valve gradient may be predictive of subsequent outflow tract intervention, and tricuspid hypoplasia may be predictive of the need for a supplemental source of pulmonary blood flow.
authors.affiliation: Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA, matthew.schwartz@orlandohealth.com.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Schwartz
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Glatz
authors.forename: Yoav
authors.initials: Y
authors.lastname: Dori
authors.forename: Jonathan J
authors.initials: JJ
authors.lastname: Rome
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Gillespie
mesh_terms: D000792:Angiography / Q000379:methods; D000800:Angioplasty, Balloon / Q000009:adverse effects / Q000379:methods; D001775:Blood Circulation; D017115:Catheter Ablation / Q000009:adverse effects / Q000379:methods; D002675:Child, Preschool; D004452:Echocardiography / Q000379:methods; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008991:Monitoring, Physiologic; D017063:Outcome Assessment, Health Care; D011183:Postoperative Complications / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D018633:Pulmonary Atresia / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D011664:Pulmonary Valve / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D014481:United States / Q000453:epidemiology