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

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

Document Type

Article

Publication Date

6-25-2022

Publication Title

Pediatric Cardiology

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.

First Page

847

Last Page

857

PubMed ID

35751685

Volume

45

Issue

4

Link to Full Text

Share

COinS