Congenital heart diseaseCohort study
20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries
- Retrospective cohort study
- single-centre
Authors' conclusion
In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.
Study context
- Population
- Patients undergoing surgery for pulmonary atresia or severe stenosis with major aortopulmonary collateral arteries (MAPCAs) and biventricular heart disease, November 2001–December 2021
- Participants
- 780 patients
Main finding
As reported in the abstract
Complete repair and mortality
- Complete repair achieved
- 704 (90%)
- Complete repair at first surgery at the centre
- 521 (67%)
- Median RV to aortic pressure ratio after repair (25th, 75th percentiles: 0.28, 0.40)
- 0.34
- Cumulative incidence of mortality at 10 years
- 15% (95% CI: 12%-19%)
- No difference in mortality by era of surgery
- P = 0.53
Observational data: associations, not effects.
Further findings
Factors associated with higher risk of death (multivariable Cox regression)
| Comparison | HR (95% CI) | P value | |
|---|---|---|---|
| Alagille syndrome | 2.8 (1.4–5.7) | P = 0.004 | |
| Preoperative respiratory support | 2.0 (1.2–3.3) | P = 0.008 | |
| palliative first surgery at our center | 3.5 (2.3–5.4) | P < 0.001 | |
| 0.20.5125Hazard ratio (log scale) |
Original publication
20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries. J Am Coll Cardiol. Published 19 September 2023. doi:10.1016/j.jacc.2023.06.041
Summarised from the published abstract; every number is checked against it