Assessment of Left
Ventricle Functions in Children with Familial Mediterranean Fever with Tissue
Doppler and Strain Echocardiography

Background/Purpose: The effects of Familial
Mediterranean Fever (FMF) on cardiac functions are generally without typical
symptoms. Global strain is a sensitive and relatively more objective indicator
of early myocardial deformation due to various conditions. We aimed to evaluate
cardiac functions in children with FMF using tissue Doppler and strain
echocardiography methods.

Methods: This is a cross
sectional study in 37 children with FMF with a mean follow up of 4 years
(range: 1-13 years) and 29 healthy children. We performed tissue Doppler and
strain echocardiography in addition to standard echocardiography in all of the
subjects. We did longitudinal strain measurements using apical 2, 3, and 4 chamber
views.

Results: Gender ratio and mean
age were similar between the groups (Table 1). Left ventricular ejection
fraction, similar between the patients and controls. Global strain (GS) was
significantly worse in patients with FMF compared to controls. None of the
patients or controls had an abnormal GS (>-15). The rate of borderline GS
was more common in the FMF group. There was also a borderline significant
difference between E’/A’ ratio which was lower in patients with FMF. GS was
significantly lower in males compared with females (-18.8±2.2 vs. -21±2,
p<0.001). Mean ages of the male and female subjects were similar (12.5±4.1
vs. 13.5±3.2, respectively, p=0.3)

Global strain was
significantly correlated with disease duration (r=0.33, p=0.046) in patients
with FMF and with E/A ratio in the whole group (r=0.34, p=0.005). There was
also an inverse correlation between GS and ejection fraction (EF) in the whole
group (r=-0.39, p=0.001). Linear regression analysis revealed independent
correlates of GS (r² of the model 0.44) as FMF group (B=1.8 95%CI
0.83-2.7, p<0.001), ejection fraction (B=-0.08, 95%CI [-0.1]-[-0.04],
p<0.001), and gender (B=1.4 95%CI 0.5-2.4, p=0.005). In patients with FMF,
disease duration (B=0.2, 95%CI 0.03-0.4, p=0.023) and EF (B=-0.13, 95%CI
[-0.2]-[-0.02], p=0.026) seemed to be independently associated with GS while
there was no significant gender association.

Conclusion: Global strain was
significantly worse in patients with FMF with no established cardiac problems.
Male gender and a lower EF seemed to be other independent determinants of a
worse GS. In patients with FMF, longer disease duration is another factor
associated with a worse GS. These are our preliminary findings and this study
is going on. No strict conclusions can be done because of the currently limited
sample size.

Table 1. Comparison of
the study groups

FMF: Familial Mediterranean fever, GS: global strain,
EF: ejection fraction