Occurrence of Clinically Diagnosed Hypertrophic Cardiomyopathy in the United States

• Published in: The American journal of cardiology 2016-05, Vol.117 (10), p.1651-1654
• Main Authors: Maron, Martin S., MD, Hellawell, Jennifer L., MD, Lucove, Jaime C., MSPH, Farzaneh-Far, Ramin, MD, Olivotto, Iacopo, MD
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Age; Aged; Aged, 80 and over; Cardiomyopathy; Cardiomyopathy, Hypertrophic - diagnosis; Cardiomyopathy, Hypertrophic - epidemiology; Cardiovascular; Cardiovascular disease; Data analysis; Echocardiography - methods; Female; Follow-Up Studies; Health care policy; Health Insurance Portability & Accountability Act 1996-US; Humans; Incidence; Insurance Claim Review; Male; Medicaid; Middle Aged; Mortality; Patients; Population; Prevalence; Retrospective Studies; Studies; United States - epidemiology; Young Adult
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2016.02.044

Abstract Hypertrophic cardiomyopathy (HC) is the most common genetic heart disease, and an important cause of sudden death and heart failure symptoms. The current prevalence for HC (1:500) is based on echocardiographic population studies in which a substantial proportion of affected individuals have not come to clinical recognition. Therefore, we sought to define the subset of patients with HC who are diagnosed in the US. A proprietary integrated claims database including medical condition International Classification of Diseases, 9th revision (ICD-9) diagnostic codes for over 160 million individual patients in the US was interrogated for 2013 to identify the prevalence of clinically recognized HC. Patients with ≥1 claim for any of the HC ICD-9 diagnosis codes from January to December 2013 were identified. The combined occurrence rate of HC was stratified by age and gender and multiplied by the 2013 United States population in the same age/gender categories to produce the final projected prevalence. The analysis was performed on 169,089,614 patients, of whom 59,009 unique patients were identified with ≥ 1 claim for HC. The projected estimated occurrence of diagnosed HC in the US in 2013 was 1:3,300 for a total of 98,958 persons. Average age at HC diagnosis was in the 5th decade of life, with 43% of the cohort comprised of women. In conclusion, leveraging a claims-based data analytic technique, about 100,000 patients are diagnosed clinically with HC in the US, an occurrence which is less than the prevalence reported in systematic population studies based on echocardiographic diagnosis. This observation supports the view that many HC patients are undiagnosed throughout life, and enhances our understanding of the burden of this genetic heart disease on the healthcare system.