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Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort

Abstract

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009–2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was − 3.06 (25%, 75%: − 4.00, − 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29–0.75, p < 0.001) and OR = 3.75 (95% CI 1.46–9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

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Acknowledgements

The authors acknowledge the staff of the Heart Institute Research Core at Cincinnati Children’s Hospital Medical Center for the regulatory, data management and statistical support provided. The authors also acknowledge Dr. Andrew Redington, Heart Institute Co-Director, Cincinnati Children’s Hospital Medical Center for critically appraising the manuscript.

Funding

This study received intramural funding from Heart Institute Research Core at Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA.

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All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by II and HZ. The first draft of the manuscript was written by II and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Ilias Iliopoulos.

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The study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

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Iliopoulos, I., Mastropietro, C.W., Flores, S. et al. Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort. Pediatr Cardiol (2022). https://doi.org/10.1007/s00246-022-02954-5

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Keywords

  • Pulmonary atresia
  • Congenital heart surgery
  • Heart defect
  • Postoperative outcomes
  • Pediatric cardiology