Familial Mediterranean fever is an autosomal recessive autoinflammatory inherited disease. We aimed to evaluate cardiac involvement in children with familial Mediterranean fever during the attack-free period.

The prospective study included 75 familial Mediterranean fever patients during the attack-free period and 50 healthy children. Cardiac evaluation was performed using electrocardiography, 24-hour ambulatory Holter monitoring, and conventional and tissue Doppler echocardiography. Aortic stiffness indices were calculated.

There were no differences between the groups in age, height, sex, body mass index, and arterial blood pressure parameters (p > 0.05). QT and corrected QT dispersion parameters were similar in both groups (p > 0.05). The E wave velocity and the E/A ratio of the mitral and tricuspid valves decreased, and the A wave velocity of the tricuspid and mitral valve increased in familial Mediterranean fever by the Doppler echocardiography (p < 0.05). The myocardial contraction velocities (Sd), early relaxation velocity (Ed), and Ed/late relaxation velocity (Ad) of both ventricles were decreased in familial Mediterranean fever group, whereas the Ad of both ventricles and the interventricular septum was increased in familial Mediterranean fever group. Aortic strain and distensibility were decreased, and pressure strain elastic modules (Ep), pressure strain normalised (Ep*) by diastolic pressure, and aortic stiffness β index were increased in familial Mediterranean fever patients (p < 0.05). When time domain heart rate variability parameters were evaluated, SDNN-i, RMSSD, and PNN50 significantly decreased in familial Mediterranean fever patients (p < 0.05), whereas SDNN and SDANN were similar in both groups (p > 0.05).

Our findings showed that cardiac involvement could exist in familial Mediterranean fever patients, even during nonattack periods.

Familial Mediterranean fever may carry a potential for cardiovascular disorders because of sustained inflammation during its course; however, there has been a limited number of studies investigating the cardiac functions in children. The aim of this study was to assess both ventricular diastolic functions using conventional echocardiography and tissue Doppler imaging in children with familial Mediterranean fever.

The study population included 25 patients with familial Mediterranean fever – mean age was 11.8 plus or minus 5.30 years – and 23 healthy patients as controls – mean age was 9.88 plus or minus 3.69 years. Both ventricular functions were measured using echocardiography comprising standard M-mode and conventional Doppler and tissue Doppler imaging during an attack-free period.

The conventional echocardiographic parameters with myocardial performance index were in normal ranges and similar in patients with familial Mediterranean fever and controls, with a p-value more than 0.05. However, right ventricular diastolic dysfunction was observed in patients with familial Mediterranean fever documented by tissue Doppler imaging, with a p-value less than 0.05 for E't and A't wave ratio.

Using tissue Doppler imaging, we have demonstrated that although left ventricular functions were comparable in the patients and healthy children, right ventricular diastolic function indices were impaired in patients with familial Mediterranean fever during childhood. Impaired right ventricular diastolic function may be an early manifestation of cardiac involvement in children with familial Mediterranean fever.