Journal article
Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort
Pediatric cardiology, Vol.45(4), pp.847-857
04/2024
DOI: 10.1007/s00246-022-02954-5
PMID: 35751685
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.
Details
- Title: Subtitle
- Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort
- Creators
- Ilias Iliopoulos - Cincinnati Children's Hospital Medical CenterChristopher W Mastropietro - Riley Hospital for ChildrenSaul Flores - Baylor College of MedicineEva Cheung - Morgan Stanley Children's HospitalVenugopal Amula - University of UtahMonique Radman - University of WashingtonDavid Kwiatkowski - Lucile Packard Children's HospitalBao Nguyen Puente - Children's NationalJason R Buckley - Medical University of South CarolinaKiona Y Allen - Lurie Children's HospitalRohit Loomba - Advocate Children's HospitalKaran B Karki - Le Bonheur Children's HospitalSaurabh Chiwane - Cardinal Glennon Children's HospitalKatherine Cashen - Children's Hospital of MichiganKurt Piggott - Louisiana State University Health Sciences Center New OrleansYamini Kapileshwarkar - Children's Hospital of IllinoisKeshava Murty Narayana Gowda - Cleveland ClinicAditya Badheka - University of IowaRahul Raman - Mercy Medical CenterHuaiyu Zang - Cincinnati Children's Hospital Medical CenterJohn M Costello - Medical University of South CarolinaCollaborative Research from the Pediatric Cardiac Intensive Care Society Investigators
- Resource Type
- Journal article
- Publication Details
- Pediatric cardiology, Vol.45(4), pp.847-857
- DOI
- 10.1007/s00246-022-02954-5
- PMID
- 35751685
- ISSN
- 0172-0643
- eISSN
- 1432-1971
- Language
- English
- Electronic publication date
- 06/25/2022
- Date published
- 04/2024
- Academic Unit
- Critical Care; Stead Family Department of Pediatrics
- Record Identifier
- 9984353947902771
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