Procedural outcomes of pulmonary atresia with intact ventricular septum in neonates: A multicenter study


Eva W. Cheung, Division of Pediatric Critical Care & Hospital Medicine, Columbia University Irving Medical Center, New York, New York. Electronic address:
Christopher W. Mastropietro, Division of Pediatric Critical Care, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, Indiana.
Saul Flores, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.
Venugopal Amula, Division of Pediatric Critical Care, University of Utah Health, Salt Lake City, Utah.Follow
Monique Radman, Division of Pediatric Critical Care, University of Washington, Seattle Children's Hospital, Seattle, Washington.
David Kwiatkowski, Division of Pediatric Cardiology, Stanford University School of Medicine, Lucille Packard Children's Hospital, Palo Alto, California.
Bao Nguyen Puente, Division of Cardiac Critical Care, Children's National Health System, Washington, District of Columbia.
Jason R. Buckley, Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina.Follow
Kiona Allen, Division of Pediatric Cardiology, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.Follow
Rohit Loomba, Advocate Aurora HealthFollow
Karan Kakri, Division of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, Tennessee.
Saurabh Chiwane, Division of Pediatric Critical Care, Saint Louis University, Cardinal Glennon Children's Hospital, Saint Louis, Missouri.Follow
Katherine Cashen, Division of Critical Care Medicine, Duke University, Duke Children's Hospital, Durham, North Carolina.Follow
Kurt Piggott, Department of Pediatrics, LSU School of Medicine Children's Hospital, New Orleans, Louisiana.
Yamini Kapileshwarkar, Department of Pediatrics, Children's Hospital of Illinois, Peoria, Illinois.
Keshava Murthy Gowda, Department of Pediatrics, Cleveland Clinic, Cleveland, Ohio.
Aditya Badheka, Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Iowa City, Iowa.Follow
Rahul Raman, Department of Pediatrics, Mercy Medical Center, Des Moines, Iowa.
John M. Costello, Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina.Follow
Huaiyu Zang, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Ilias Iliopoulos, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.


Advocate Children's Hospital


Background: Multicenter contemporary data describing short-term outcomes following initial interventions of neonates with pulmonary atresia intact ventricular septum (PA-IVS) are limited. This multicenter study aims to describe characteristics and outcomes of PA-IVS neonates following their initial catheter or surgical intervention and identify factors associated with major adverse cardiac events (MACE).

Methods: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009-2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression model.

Results: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, 16 (6%) suffered stroke, 23 (8%) died. The presence of two major coronary artery stenoses (adjusted OR: 4.99; 95% CI: 1.16-21.39) and lower weight at first intervention (adjusted OR: 1.52, 95% CI: 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n=10).

Conclusions: In a multicenter cohort, one in five neonates with PA-IVS experienced MACE following their initial intervention. Patients with two major coronary artery stenoses or lower weight at time of initial procedure were most likely to experience MACE and warrant vigilance during pre-intervention planning and post-intervention management.

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