Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum

Catheter Cardiovasc Interv. 2013 Jan 1;81(1):111-8. doi: 10.1002/ccd.24288. Epub 2012 Oct 17.

Abstract

Objective: To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC).

Background: PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed.

Methods: Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded.

Results: PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years.

Conclusions: The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Publication types

  • Comparative Study
  • Evaluation Study

MeSH terms

  • Boston
  • Cardiac Catheterization / adverse effects
  • Cardiac Catheterization / methods*
  • Cardiac Surgical Procedures / adverse effects
  • Cardiac Surgical Procedures / methods*
  • Cohort Studies
  • Echocardiography, Doppler / methods
  • Female
  • Follow-Up Studies
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / surgery*
  • Hospitals, Pediatric
  • Humans
  • Infant, Newborn
  • Male
  • Minimally Invasive Surgical Procedures / adverse effects
  • Minimally Invasive Surgical Procedures / methods
  • Pulmonary Atresia / diagnostic imaging
  • Pulmonary Atresia / surgery*
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Treatment Outcome

Supplementary concepts

  • Pulmonary Atresia with Intact Ventricular Septum