Risk Factors and Cardiovascular Disease (CVD) Related Outcomes in Hospitalized Patients with Hemophilia 10 Year Follow up

Introduction: Comprehensive management for patients with hemophilia has drastically improved outcomes, quality of care, and longevity. Because of increases in life span, patients with hemophilia may be at risk for other chronic conditions including cardiovascular disease (CVD). Though initially it was thought that hemophilia might have been protective for cardiovascular disease further research has shown that CVD remains a significant risk for the aging hemophilia population. This study aims to determine the prevalence of risk factors and outcomes for CVD in hospitalized adult and pediatric patients with the discharge diagnosis of Hemophilia A or B compared to patients without Hemophilia. We examine longitudinal changes over the previous decade.

Methods: The Healthcare Cost and Utilization Project’s Nationwide Inpatient Sample (HCUP-NIS) was utilized for analysis of years 2007 and 2017. The NIS uses a stratified probability sample of 20% of all inpatient discharges (representing more than 97% of the US population). Hemophilia-A and B were identified using ICD-9 code 286.0 and 286.1, ICD-10 codes D66 and D67 respectively and sampling weights were applied to generate nationally representative estimates. Cardiovascular risk factors and outcomes were determined by evaluating ICD-9 codes for 2007 data and ICD-10 codes for 2017 data. For comparative historical data, 2007 NIS data from a prior published study [Goel et al., Hemophilia (2012), 18, 688-692] were used. The NIS is a de-identified, publicly available data set. This study was deemed exempt from review from the Johns Hopkins Institutional Review Board. This analysis was conducted in accordance with the HCUP data use agreement guidelines.

Results: In 2017, there were 10,555 admissions with Hemophilia A or B listed as one of all diagnoses. The mean age of hemophilia patients was 44.31 years compared to 49.57. years for all admissions. The most prevalent risk factor in 2017 was hypertension (32.4% for admissions with hemophilia as compared to 35.3% for all admissions) followed by hyperlipidemia (19.4% compared to 27.5%), diabetes (17.4% compared to 22.8%) and obesity (10.8% compared to 14.4%). CVD outcomes, in descending order of frequency were atherosclerotic coronary artery disease (11.6% for admissions with hemophilia compared to 16.9% for all admissions), heart failure (10.2% compared to 14.2%), acute myocardial infarction (AMI) (2.2% compared to 3.9%), and stroke (2.2% compared to 2.4% respectively).

Comparing to 10 year prior data, in 2007, there were 9,737 admissions with Hemophilia A or B listed as one of all diagnoses. The mean age of hemophilia patients was 30.89 years compared to 47.16 years for all admissions. The most prevalent risk factor in 2007 was hypertension (27.0% in admissions with hemophilia compared to 36.7% for all admissions); followed by diabetes (11.2% compared to 18.5%), hyperlipidemia (9.5% compared to 17%), and obesity (3.6% compared to 5.8%). CVD outcomes, in descending order of frequency were, atherosclerotic coronary artery disease (10.1% compared to 16.7%), heart failure (6.6% compared to 10.8%), AMI (2.1% compared to 2.4%), and stroke (2.0% compared to 1.7%).

Between 2007 and 2017 the crude prevalence rates of all CVD risk factors as well as CVD outcomes generally increased for admissions with hemophilia as well as all-cause hospitalizations.

Conclusions: The frequency of all CVD risk factors (obesity, diabetes, hypertension, and hyperlipidemia) as well as CVD outcomes (atherosclerosis, congestive heart failure, AMI, and stroke) increased between 2007 and 2017 in hospitalized patients both with and without hemophilia.

While the unadjusted prevalence rates for all CVD risk factors and CVD outcomes were less in hospitalized patients with hemophilia compared to the general hospitalized population in both 2007 and 2017, CVD remains a significant risk for the hemophilia population. An improved understanding of the various risk factors will help to improve CVD outcomes in the aging hemophilia population.