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. Impact <UnorderedList Mark="Bullet"> <ItemContent> This is the largest multicenter study in the U.S to date exploring the epidemiology, resource utilization, and outcomes of neonates with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV). </ItemContent> <ItemContent> ACD/MPV is a rare neonatal disorder and carries a very high mortality (86%) despite advanced therapies. </ItemContent> <ItemContent> ACD/MPV is frequently associated with congenital malformations, which were present in over 91% of the cases, with cardiovascular anomalies predominating. </ItemContent> <ItemContent> ACD/MPV should be suspected in neonates with prolonged or refractory pulmonary hypertension, and early diagnostic evaluation, like genetic testing or lung biopsy, should be conducted to avoid prolonged futile interventions. </ItemContent> </UnorderedList>

Alveolar capillary dysplasia with misalignment of pulmonary veins in neonates: a multicenter cohort study

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2026年6月29日

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Prithvi Sendi,
Paul Martinez,
Kaitlin Kobaitri,
Balagangadhar R. Totapally

摘要

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.

Impact

This is the largest multicenter study in the U.S to date exploring the epidemiology, resource utilization, and outcomes of neonates with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV).

ACD/MPV is a rare neonatal disorder and carries a very high mortality (86%) despite advanced therapies.

ACD/MPV is frequently associated with congenital malformations, which were present in over 91% of the cases, with cardiovascular anomalies predominating.

ACD/MPV should be suspected in neonates with prolonged or refractory pulmonary hypertension, and early diagnostic evaluation, like genetic testing or lung biopsy, should be conducted to avoid prolonged futile interventions.