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

Ilias Iliopoulos; Christopher W Mastropietro; Saul Flores; Eva Cheung; Venugopal Amula; Monique Radman; David Kwiatkowski; Bao Nguyen Puente; Jason R Buckley; Kiona Y Allen; Rohit Loomba; Karan B Karki; Saurabh Chiwane; Katherine Cashen; Kurt Piggott; Yamini Kapileshwarkar; Keshava Murty Narayana Gowda; Aditya Badheka; Rahul Raman; Huaiyu Zang; John M Costello; Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators

doi:10.1007/s00246-022-02954-5

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

Pediatr Cardiol. 2024 Apr;45(4):847-857. doi: 10.1007/s00246-022-02954-5. Epub 2022 Jun 25.

Authors

Ilias Iliopoulos^{1

2}, Christopher W Mastropietro³, Saul Flores⁴, Eva Cheung⁵, Venugopal Amula⁶, Monique Radman⁷, David Kwiatkowski⁸, Bao Nguyen Puente⁹, Jason R Buckley¹⁰, Kiona Y Allen¹¹, Rohit Loomba¹², Karan B Karki¹³, Saurabh Chiwane¹⁴, Katherine Cashen¹⁵, Kurt Piggott¹⁶, Yamini Kapileshwarkar¹⁷, Keshava Murty Narayana Gowda¹⁸, Aditya Badheka¹⁹, Rahul Raman²⁰, Huaiyu Zang²¹, John M Costello¹⁰; Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators

Affiliations

¹ The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. ilias.iliopoulos@cchmc.org.
² Cardiac Intensive Care, The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229, USA. ilias.iliopoulos@cchmc.org.
³ Division of Critical Care, Department of Pediatrics, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN, USA.
⁴ Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
⁵ Department of Pediatrics, Columbia University College of Physicians and Surgeons, Morgan Stanley Children's Hospital of New York, New York, NY, USA.
⁶ Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA.
⁷ Division of Critical Care, Department of Pediatrics, University of Washington, Seattle Children's Hospital, Seattle, WA, USA.
⁸ Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Lucille Packard Children's Hospital, Palo Alto, CA, USA.
⁹ Division of Cardiac Critical Care, Children's National Health System, Washington, DC, USA.
¹⁰ Division of Cardiology, Department of Pediatrics, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, USA.
¹¹ Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
¹² Department of Pediatrics, Chicago Medical School, Advocate Children's Hospital, Chicago, IL, USA.
¹³ Division of Cardiology, Department of Pediatrics, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, TN, USA.
¹⁴ Division of Pediatric Critical Care, Department of Pediatrics, Saint Louis University, Cardinal Glennon Children's Hospital, Saint Louis, MO, USA.
¹⁵ Department of Pediatrics, Central Michigan University, Children's Hospital of Michigan, Detroit, MI, USA.
¹⁶ Division of Critical Care, LSU Health Sciences, Children's Hospital, New Orleans, LA, USA.
¹⁷ Department of Pediatrics, Children's Hospital of Illinois, Peoria, IL, USA.
¹⁸ Department of Pediatrics, Cleveland Clinic, Cleveland, OH, USA.
¹⁹ Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Iowa City, IA, USA.
²⁰ Department of Pediatrics, Mercy Medical Center, Des Moines, IA, USA.
²¹ The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

PMID: 35751685
DOI: 10.1007/s00246-022-02954-5

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.

Keywords: Congenital heart surgery; Heart defect; Pediatric cardiology; Postoperative outcomes; Pulmonary atresia.

Publication types

Multicenter Study

MeSH terms

Heart Defects, Congenital* / complications
Heart Ventricles
Humans
Infant, Newborn
Multicenter Studies as Topic
Pulmonary Atresia* / complications
Treatment Outcome
Ventricular Septum*

Supplementary concepts

Pulmonary Atresia with Intact Ventricular Septum