Retrospective Analysis of Burden of Illness of Congenital Pulmonary Valve Disease in a Large, Commercially Insured US Population

Background Babies born with congenital pulmonary valve disease (CPVD) face a lifetime burden of disease and significant medical expense beginning in their first year of life. Objectives To assess costs and burden of care in patients with CPVD from birth through age 65. Methods This retrospective study used administrative claims data to examine healthcare costs and utilization among commercially insured patients with ≥2 claims with CPVD diagnosis between January 1, 2006, and April 30, 2024; the earliest CPVD claim defined the index date. Per-patient-per-year costs were calculated by age category. Mean annual costs for each age group were multiplied by age category duration to estimate full age category period costs. Costs for each age category period were summed to estimate lifetime costs through 65 years of age. Sensitivity analyses were conducted in subgroups with continuous eligibility from birth through 60 months and 120 months. Results Among eligible patients (N = 22 751), 53.2% experienced an average of 1.8 CPVD-related inpatient admissions during their first year: mean (standard deviation) cumulative admission days, 50.2 (74.2); with $267 945 (737 565; median, 6487 [interquartile range (IQR), 0-238 486] in associated costs. Among those with inpatient admissions for CPVD-related procedures during childhood, average annual costs ranged from $97 791 (288 606; 32 029 [15 984-72 762]) to $514 395 (926 572; 222 373 [114 107-530 671] by age category. Emergency department utilization ranged between 8% and 21% by age category. Sensitivity analysis confirmed high total annual CPVD-related costs over 60 months (N = 872) at $59 537 (111 982; 13 690 [918-66 625]) and 120 months (N = 174) at $28 893 (69 019; 2852 [400-23 858]). By age 21, projected cumulative CPVD-related medical costs averaged $788 188 and reached $2.1 million through age 65. Over half of patients had nearly 2 months of hospital stays in their first year, with substantial inpatient costs. The financial burden and demanding care began early and remained steady, reflecting the chronic nature of CPVD. Conclusion This study demonstrates a substantial burden of illness and lifetime mosaic of ongoing medical needs for individuals with CPVD and the potential for advances in early life care to reduce this sizable projected lifetime burden.