Prithvi Sendi, Paul Martinez, Kaitlin Kobaitri, Balagangadhar R. Totapally
Nicklaus Children’s Hospital. Herbert Wertheim College of Medicine and Florida International University.
United States
Pediatric Research
Pediatr Res 2026;
DOI: 10.1038/s41390-026-05265-0
Abstract
Background: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare developmental lung disorder associated with high neonatal mortality. We aimed to describe the epidemiology, resource utilization, and outcomes of neonates with ACD/MPV.
Methods: Retrospective observational study using the Pediatric Health Information System database. ICD-10 diagnosis codes were used to identify neonates hospitalized with ACD/MPV between 2016 and 2025. Demographic characteristics, associated congenital anomalies, resource utilization, length of stay, and mortality were analyzed.
Results: We identified 58 neonates with ACD/MPV during the study period. The demographic distribution includes 51.7% female sex, 60.3% Whites, and 28.4% Hispanics or Latinos. Median gestational age was 38 weeks with a birthweight of 2,883 grams. Cytogenetic testing was documented in 43.1% and surgical tissue biopsy in 53.3% of the neonates. Congenital anomalies were present in 91%, with cardiovascular malformations predominating. Invasive mechanical ventilation (IMV) was used in 93.1%, and extracorporeal membrane oxygenation (ECMO) in 63.8%. ECMO utilization was more common among non-survivors (72.0 vs. 12.5%). Mortality was 86.2%.
Conclusion: ACD/MPV is a rare neonatal disorder characterized by high mortality and substantial resource utilization. Congenital malformations are frequent, with cardiovascular anomalies predominating. ACD/MPV should be considered in neonates with refractory pulmonary hypertension.
Category
Class III. Pulmonary Hypertension Associated with Developmental Diseases of the Lung
Genetic Factors Associated with Pulmonary Vascular Disease
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes