Assessment of Left Ventricular Functions With Strain and Strain Rate Echocardiography in Children with Duchenne Muscular Dystrophy

Ozet Amac: Calismamizda konvansiyonel ekokardiyografi ile kalp fonksiyonlari normal bulunan Duchenne Muskuler Distrofili (DMD) hastalarin miyokard fonksiyonlarinin  pulse wave doku doppler goruntuleme (PWDDG) ve strain/strain rate (S/SR) ekokardiyografi araciligiyla  olculmesi ve kontrol grubu ile karsilastirilmasi amaclandi. Materyal ve Metod: Bu calismada; ortalama yasi 85,2 ± 38,4 ay olan 32 DMD’li erkek hasta ve 89 ± 38,9 ay olan 31 saglikli cocuk degerlendirildi. Demografik ozellikler olgular ve kontrol grubunda degerlendirildi. Cinsiyet, yas, vucut kitle indeksi, konvansiyonel  ekokardiyografi verileri, pulse wave doku Doppler goruntuleme (PW-TDI) verileri ve iki boyutlu (2D) longitudinal strain (LS) / longitudinal strain rate (LSR) ekokardiyografi degerleri karsilastirildi. Bulgular: Hasta ve kontrol gruplari arasinda kalp hizi bakimindan istatistiksel olarak anlamli fark saptandi (p<0.001) ve DMD’li grupta kalp hizi daha yuksekti. Ventrikuler septumda bazal olcumlerinde E m , S amplitudu, izovolumetrik relaksasyon zamani (IVRZ), miyokard performans indeksi (MPI) bakimindan istatistiksel yonden anlamli fark saptanirken (p<0,05), sol ventriku serbest duvar bazalindeki olcumlerde de E m , S amplitudu ve IVRZ bakimindan istatistiksel yonden anlamli fark bulundu (p<0,05). Sol ventrikulun serbest duvar bazalindeki strain ve strain rate degerlerinde DMD’ligrup ve kontrol grubu arasinda istatistiki yonden  anlamli fark saptandi (p<0.001). Yapilan apikal dort bosluk pozisyondaki global strain degerlerinde DMD’li grup ve kontrol grubu arasinda istatistiki yonden anlamli fark saptandi ( p<0,001). Sonuc: Transtorasik ekokardiyografide normal aralik icinde sol ventrikul sistolik fonksiyon saptanan DMD'li asemptomatik hastalarda kontrol grubu ile karsilastirildiginda sol ventrikul anterolateral duvarinda LS ve LSR degerleri anlamli olarak dusuk bulundu. Anahtar kelimeler: Longitudinal strain, longitudinal strain rate, Duchenne muskuler distrofi Abstract Aim: The goal of our study was to detect the left ventricular functions of duchenne muscular dystrophy ( DMD) patients using , pulsed-wave tissue Doppler imaging (PW-TDI, longitudinal strain (LS) and longitudinal strain rate (LSR ) echocardiography who had normal left ventricular functions in standart echocardiography before, and to match them with the results of the control group. Material and Methods: In this study compared 32 boys with DMD whose mean age was 85.2 ± 38.4 months were matched with 31 healthy males whose mean age was 89.0 ± 38.9 months. The following demographic features were assesed in both DMD patients  and controls: gender, age, body mass index, standard echocardiography parameters, pulsed-wave tissue Doppler imaging (PW-TDI) findings, and two-dimensional (2D) LS/LSR echocardiography measurements. Results: Asymptomatic boys with DMD were established to have high heart rate (p<0.001). In the calculations performed from the base of the ventricular septum, statistically considerable differences were determined   between the Em, S amplitude and isovolumetric relaxation time (IVRT), myocard performance index (MPI) values of the two groups (p<0.05). In the evaluates made from the base of the left ventricular free wall, Em, S amplitude and IVRT, MPI values were demonstrated to be more considerably different (p<0.05). The results of the LS and LSR measurements done from the base of the left ventricular free wall were considerably different between DMD and control group (p<0.001), and in the global strain measurement performed from the four chamber apical position, considerable distinction was noted between the two groups (p<0.001). Conclusion: In patients with DMD in whom standart echocardiography had assesed left ventricular systolic function within the normal range showed significantly lower LS and LSR values at the left ventricular anterolateral wall compared with the control group. Keywords: Longitudinal strain, longitudinal strain rate, duchenne muscular dystrophy,