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. 2025 Sep 10;15(3):e70164.
doi: 10.1002/pul2.70164. eCollection 2025 Jul.

Pulmonary Hypertension Outcomes After Closure of Atrial Septal Defect in Infants With Developmental Lung Disease

John L Wiegand  1 , James A Thompson  2 , Bruce F Landeck  2 , Jamie L Fierstein  3   4 , Dina Ashour  3 , Joana S Machry  5 , Amy L Kiskaddon  2   3   6   7 , Marisol Betensky  8   9 , Grace A Freire  2
Affiliations

Pulmonary Hypertension Outcomes After Closure of Atrial Septal Defect in Infants With Developmental Lung Disease

John L Wiegand et al. Pulm Circ. .

Abstract

Pulmonary hypertension (PHTN) in infants with developmental lung disease, such as bronchopulmonary dysplasia (BPD), chronic lung disease of infancy (CLD), or congenital diaphragmatic hernia (CDH), can be exacerbated by atrial septal shunts secondary to atrial septal defects (ASD). While transcatheter ASD closure may reduce pulmonary overcirculation, data on post-closure hemodynamic and pharmacologic outcomes remain limited. This single-center retrospective study aimed to characterize changes in PHTN severity, respiratory support, and medication use 1 year after early transcatheter ASD closure (defined as closure at ≤ 1 year of age). Eligible patients were infants with BPD, CLD, or CDH who underwent early transcatheter ASD closure between 2021 and 2024 and had preprocedural PHTN medication use and respiratory support. Sixteen infants met the inclusion criteria. At 1 year, excluding the 3 who died, 10 of 13 infants (76.9%) showed improved PHTN severity, including 6 (60%) with complete resolution. Of the 13 infants, 6 (46.2%) weaned off all respiratory support. Average diuretic dosage (mg/kg/day) decreased by 92.9%, and vasodilator dosage declined by 47.0%. Infants with ASDs ≥ 5 mm and gestational age (GA) < 32 weeks required significantly longer diuretic therapy than those with smaller ASDs (< 5 mm) and GA ≥ 32 weeks. No similar associations were found with vasodilator weaning. These findings suggest early transcatheter ASD closure may offer therapeutic benefit in select high-risk infants, resulting in improved hemodynamics and reduced medication dependence. Although limited by small sample size and retrospective design, this study supports the potential for individualized weaning strategies and the need for prospective multicenter investigations.

Keywords: chronic lung disease of infancy; eccentricity index; respiratory support; transcatheter intervention; vasodilator and diuretic weaning.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Pulmonary Hypertension (PHTN) severity level of infants by time point. Asterisks represent a significant difference in the proportion of PHTN severity levels from pre‐procedure. One infant was missing data at 3 months; 1 infant died at 6 months, and 2 additional infants died at 12 months postprocedure.
Figure 2
Figure 2
Level of respiratory support of infants by time point. One infant died at 6 months, and 2 additional infants died at 12 months postprocedure.
Figure 3
Figure 3
Change in median dosage (mg/kg/day) of vasodilators and diuretics over time. The individual squares in the graphs represent the dosage (mg/kg/day) of study infants at each time point. p‐values reported indicate significant differences in dosages from pre‐procedure according to the corrected α.
Figure 4
Figure 4
Median of the average weaning days per infant by atrial septal defect size for vasodilators and diuretics.
Figure 5
Figure 5
Median of the average weaning days per infant by gestational age for vasodilators and diuretics.
Figure 6
Figure 6
Median of the average weaning days per infant by baseline pulmonary hypertension severity for vasodilators and diuretics.
See this image and copyright information in PMC

References

    1. Hansmann G., Koestenberger M., Alastalo T. P., et al., “2019 Updated Consensus Statement on the Diagnosis and Treatment of Pediatric Pulmonary Hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), Endorsed by AEPC, ESPR and ISHLT,” Journal of Heart and Lung Transplantation 38, no. 9 (2019): 879–901, 10.1016/j.healun.2019.06.022. - DOI - PubMed
    1. Rosenzweig E. B., Abman S. H., Adatia I., et al., “Paediatric Pulmonary Arterial Hypertension: Updates on Definition, Classification, Diagnostics and Management,” European Respiratory Journal 53, no. 1 (2019): 1801916, 10.1183/13993003.01916-2018. - DOI - PMC - PubMed
    1. Kim D. H., Kim H. S., Choi C. W., Kim E. K., Kim B. I., and Choi J. H., “Risk Factors for Pulmonary Artery Hypertension in Preterm Infants With Moderate or Severe Bronchopulmonary Dysplasia,” Neonatology 101, no. 1 (2012): 40–46, 10.1159/000327891. - DOI - PubMed
    1. Gupta V. S. and Harting M. T., “Congenital Diaphragmatic Hernia‐Associated Pulmonary Hypertension,” Seminars in Perinatology 44, no. 1 (2020): 151167, 10.1053/j.semperi.2019.07.006. - DOI - PubMed
    1. Engel C., Leyens J., Bo B., et al., “Arterial Hypertension in Infants With Congenital Diaphragmatic Hernia Following Surgical Repair,” European Journal of Pediatrics 183, no. 7 (2024): 2831–2842, 10.1007/s00431-024-05509-3. - DOI - PMC - PubMed

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