Pulmonary atresia with intact ventricular septum: Midterm outcomes from a multicenter cohort

Ilias Iliopoulos, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. ilias.iliopoulos@cchmc.org.
Christopher W. Mastropietro, Division of Critical Care, Department of Pediatrics, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN, USA.
Saul Flores, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
Eva Cheung, Department of Pediatrics, Columbia University College of Physicians and Surgeons, Morgan Stanley Children's Hospital of New York, New York, NY, USA.
Venugopal Amula, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA.
Monique Radman, Division of Critical Care, Department of Pediatrics, University of Washington, Seattle Children's Hospital, Seattle, WA, USA.
David Kwiatkowski, Division of Cardiology, Department of Pediatrics, Stanford University School of Medicine, Lucille Packard Children's Hospital, Palo Alto, CA, USA.
Bao Nguyen Puente, Division of Cardiac Critical Care, Children's National Health System, Washington, DC, USA.
Jason R. Buckley, Division of Cardiology, Department of Pediatrics, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, USA.
Kiona Y. Allen, Division of Cardiology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Rohit Loomba, Advocate Aurora HealthFollow
Karan B. Karki, Division of Cardiology, Department of Pediatrics, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, TN, USA.
Saurabh Chiwane, Division of Pediatric Critical Care, Department of Pediatrics, Saint Louis University, Cardinal Glennon Children's Hospital, Saint Louis, MO, USA.
Katherine Cashen, Department of Pediatrics, Central Michigan University, Children's Hospital of Michigan, Detroit, MI, USA.
Kurt Piggott, Division of Critical Care, LSU Health Sciences, Children's Hospital, New Orleans, LA, USA.
Yamini Kapileshwarkar, Department of Pediatrics, Children's Hospital of Illinois, Peoria, IL, USA.
Keshava Murty Gowda, Department of Pediatrics, Cleveland Clinic, Cleveland, OH, USA.
Aditya Badheka, Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Iowa City, IA, USA.
Rahul Raman, Department of Pediatrics, Mercy Medical Center, Des Moines, IA, USA.
Huaiyu Zang, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
John M. Costello, Division of Cardiology, Department of Pediatrics, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, USA.

Affiliations

Advocate Children's Hospital

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.

Document Type

Article

PubMed ID

35751685

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