Cardiovascular Risk Scores among Asymptomatic Adults with Haemophilia

Area of Science:

• Cardiology
• Hematology

Background:

• Mortality rates for Brazilian people with haemophilia (PwH) are declining.
• However, cardiovascular disease (CVD) is an increasing cause of death among PwH.

Purpose of the Study:

• To assess the CVD risk score in PwH using the Pooled Cohort Equations Risk (PCER) Calculator.
• To compare PCER estimates with the Framingham Risk Score (FRS).
• To outline treatment recommendations based on CVD risk.

Main Methods:

• A cross-sectional study included 30 male PwH aged 40+ in Recife, Brazil.
• Exclusion criteria: prior CVD event or LDL cholesterol ≥ 5.0 mmol/L.
• Data collected via interviews, medical records, and blood tests; PCER and FRS were calculated.

Main Results:

• The median PCER score was 6.9%, with 50% of PwH classified as high risk (≥ 7.5%).
• Prevalence of traditional CVD risk factors: hypertension (67%), obesity (20%), diabetes (24%), hypertriglyceridemia (14%), hypercholesterolemia (47%), hypoHDLaemia (23%).
• Statin therapy was recommended for 54%; 47% had poorly controlled blood pressure. PCER and FRS showed good agreement (80%, κ = 0.60).

Conclusions:

• Half of older male PwH exhibit a high 10-year CVD risk.
• Urgent interventions are needed to manage dyslipidemia and hypertension in this population.
• The PCER tool effectively estimates CVD risk in PwH.