Abstract
Background: Hemophilia is a congenital bleeding disorder with X-linked inheritance that is associated with a deficiency of coagulation factor 8 (hemophilia A) or 9 (hemophilia B). The protective effect of hemophilia is challenging in the prevention of atherothrombotic heart diseases due to the lack of coagulation factors. Objectives: The present study aims to investigate congenital and acquired heart diseases and atherosclerosis risk factors in hemophilia patients. Methods: In the present cross-sectional descriptive study, electrocardiography (ECG) and echocardiography were performed and evaluated on 50 hemophilia patients in Dezful, southwest Iran. Fasting blood sugar, low-density lipoprotein (LDL), cholesterol, plasma high-density lipoprotein (HDL), systemic blood pressure, and body mass index (BMI) were also calculated. The data were analyzed in SPSS software version 21. The results showed 86% with hemophilia A, 12% with hemophilia B, and one patient (2%) with post-pregnancy hemophilia (mean age = 28.12 ± 14.12 years; age range = 6 - 76 years). Results: The most common congenital heart disease was mitral valve prolapse (MVP) with or without mitral regurgitation, consisting of 5 patients (10%); the most common acquired cardiac disorder was diastolic dysfunction grade I, consisting of 5 patients (10 %), and 2 patients (4%) also had systolic pulmonary artery hypertension (pulmonary artery systolic pressure more than 35 mmHg). Bicuspid aortic valve (BAV), atrial septal defect (ASD), and hypertrophic cardiomyopathy (HCM) were observed in one patient each. Ischemic heart disease was observed only in a 76-year-old patient. Examination of the electrocardiogram had no abnormal findings in the rest, except for two patients with HCM and ASD who had ventricular hypertrophy. The most common risk factor was obesity (16%), followed by hypertension (10%) and dyslipidemia (10%), and only 1 (2%) diabetic patient was identified. Patients with risk factors often had more than one risk factor, were over 30 years old, and had a severe form of hemophilia A. Conclusions: In the examination of echocardiography-ECG and risk factors in hemophilia patients, there was no significant abnormality report, while acquired heart diseases and risk factors proportional to age are evident from 30 years of age onwards.