Myocardial functions, blood pressure changes, and arterial stiffness in children with severe hemophilia A

Prolonging the life span of patients with hemophilia has led to the emergence of comorbidities. Cardiovascular diseases are one of the important causes of mortality in patients with hemophilia. This study investigated the myocardial functions, blood pressure changes, arterial stiffness, and risk factors associated with cardiovascular diseases in children with hemophilia.

In total, 17 children with severe hemophilia A and 23 healthy children were included in the study. Myocardial functions were evaluated using standard and tissue Doppler echocardiography. Peripheral and central blood pressure measurements were performed, and arterial stiffness was evaluated. Carotid intima-media thicknesses (CIMT) serum glucose, insulin, insulin resistance index, and lipoprotein levels were measured.

There were no differences between the two groups in terms of age, and biochemical parameters (P > 0.05). The HDL-C levels in the hemophilia group were lower than those in the control group (P < 0.05). Five of the patients had insulin resistance (29.4%), whereas four had low HDL-C levels (23.5%). There were no differences between the groups in terms of the CIMT, peripheral blood pressure, and central systolic blood pressure (P > 0.05). In the hemophilia group, central diastolic blood pressure (cDBP), arterial stiffness, and myocardial performance index were higher (P < 0.05, P = 0.01, P < 0.01), whereas the ejection time was shorter than in the control group (P < 0.05).

Compared with the control group, there is an onset of arterial stiffness, cDBP values tend to increase, and serum HDL-C levels are lower in the hemophilia group. Moreover, myocardial systolic functions demonstrate a deterioration that becomes more prominent with the increase in arterial stiffness.