270 - Alveolar capillary dysplasia in neonates

Poster Number: 270
Publication Number: 270.2032

Background: Alveolar capillary dysplasia (ACD) is a rare and often fatal neonatal condition characterized by developmental abnormalities in the pulmonary system. Existing literature on this condition primarily consists of individual case reports or small case series, and comprehensive national data on ACD is currently lacking.

Objective: The objective of this study was to describe the clinical characteristics, resource utilization, and outcomes of neonates with ACD using two large national databases, namely the Healthcare Cost and Utilization Project’s (HCUP) Kids Inpatient Database (KID) and Pediatric Health Information System (PHIS) database.

Design/Methods: This retrospective cross-sectional study analyzed data from the KID database for the years 2012, 2016, and 2019, as well as the PHIS database for the years 2016 to 2021. KID is a national pediatric discharge data published every 3 years by the HCUP. The PHIS database is comprised of data from 51 children's hospitals in the U.S. Both databases were filtered using ICD-9 and ICD-10 codes specific to ACD, and the study included only neonatal cases. The data analysis and reporting adhered to the guidelines provided by HCUP and PHIS. Descriptive data were presented in frequencies for categorical variables and medians (IQR) for continuous variables. To establish the prevalence of ACD in the United States, we used the number of ACD discharges in the KID database and the annual U.S. birth rate. These studies were exempted by the IRB.

Results: There were 58 cases in the KID (during 3 years) and 35 in the PHIS (during 5 years) database. The prevalence of ACD was 1 case per 200,000 births in the United States (Table 1). Table 2 presents the demographic and clinical characteristics, resource utilization, and outcomes of neonates diagnosed with alveolar capillary dysplasia. Survivors had a higher median admission age and longer hospital stays (Table 3). Approximately 50% of these neonates experienced pulmonary hypertension, and around 20% suffered from intracranial hemorrhage. The use of ECMO was observed in 38% to 57% of cases, with a mortality rate within this group reaching 86% to 100%. The median duration of ECMO support was 10.5 (5-17.5) days. The overall mortality rate was between 72% and 86%.

Conclusion(s): Alveolar capillary dysplasia is an uncommon neonatal condition, occurring in approximately 1 out of every 200,000 births in the United States. Infants affected by ACD face a significant risk of mortality and often require substantial healthcare resources. Recognizing ACD early and diagnosing it promptly could potentially lead to resource savings.