Abstract
Background
The mortality rate of Brazilian people with haemophilia (PwH) is decreasing, but the relative incidence of deaths associated with cardiovascular disease (CVD) is increasing.
Objectives
We aimed to describe the CVD risk score of PwH according to Pooled Cohort Equations Risk (PCER) Calculator tool and its treatment recommendations. We also compared the PCER estimates with the respective Framingham Risk Score (FRS).
Methods
This cross-sectional study included male PwH ≥ 40 years treated at the Comprehensive Haemophilia Treatment Centre of Pernambuco (Recife/Brazil). PwH with a previous CVD event or a low-density lipid cholesterol ≥ 5.0 mmol/L were excluded. Interviews, medical file reviews, and blood tests were performed. The PCER tool was used to estimate the CVD risk and compare it with the respective FRS. A p-value < 0.05 was accepted as statistically significant.
Results
Thirty PwH were included. Median age was 51.5 [interquartile range-IQR; 46.0-59.5] years. The prevalence of obesity, systemic arterial hypertension, diabetes mellitus, hypertriglyceridaemia, hypercholesterolaemia, and hypoHDLaemia were 20%, 67%, 24%, 14%, 47%, and 23%, respectively. The median PCER score was 6.9% [IQR; 3.1-13.2], with 50% having a high risk (PCER ≥ 7.5%). Statin use was suggested for 54% of PwH. Blood pressure was poorly controlled in 47% of PwH. The agreement between PCER and FRS was 80% (κ = 0.60; p = 0.001).
Conclusions
Half of the male people with haemophilia aged 40 years or older had a 10-year high risk of developing CVD with strong recommendations to improve control of dyslipidaemia and blood pressure.
Hemophilia A; Hemophilia B; Primary Prevention; Heart Disease Risk Factors
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Desde o desenvolvimento de produtos mais eficazes e seguros para tratar o tratamento da hemofilia, as pessoas que vivem com a doença estão vivendo mais. Além disso, esses indivíduos passaram a desenvolver doenças cardiovasculares características do envelhecimento. Propomos que hematologistas e outros profissionais de saúde que cuidam de indivíduos com hemofilia realizem avaliações regulares e formais dos fatores de risco cardiovascular nesses pacientes e, quando presentes, recomendamos que tais condições sejam tratadas de acordo com protocolos estabelecidos. Quando necessário, os pacientes devem ser encaminhados para um especialista. (PCER, Pooled Cohort Equations Risk)
Since the development of more effective and safe products to treat haemophilia, people with haemophilia are growing older. In addition, these individuals are developing cardiovascular diseases which are characteristic of ageing people. We propose that Haematologists and other clinicians that treat people with haemophilia formally and routinely evaluate cardiovascular risk factors among these individuals and, when present, the diseases should be treated according to estabilished protocols. When required, the patients should be referred to a specialist. PCER: pooled cohort equations risk.
