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

Özet Amaç: Çalışmamızda konvansiyonel ekokardiyografi ile kalp fonksiyonları normal bulunan Duchenne Musküler Distrofili (DMD) hastaların miyokard fonksiyonlarının  pulse wave doku doppler görüntüleme (PWDDG) ve strain/strain rate (S/SR) ekokardiyografi aracılığıyla  ölçülmesi ve kontrol grubu ile karşılaştırılması amaçlandı. Materyal ve Metod: Bu çalışmada; ortalama yaşı 85,2 ± 38,4 ay olan 32 DMD’li erkek hasta ve 89 ± 38,9 ay olan 31 sağlıklı çocuk değerlendirildi. Demografik özellikler olgular ve kontrol grubunda değerlendirildi. Cinsiyet, yaş, vücut kitle indeksi, konvansiyonel  ekokardiyografi verileri, pulse wave doku Doppler görüntüleme (PW-TDI) verileri ve iki boyutlu (2D) longitudinal strain (LS) / longitudinal strain rate (LSR) ekokardiyografi değerleri karşılaştırıldı. Bulgular: Hasta ve kontrol grupları arasında kalp hızı bakımından istatistiksel olarak anlamlı fark saptandı (p<0.001) ve DMD’li grupta kalp hızı daha yüksekti. Ventriküler septumda bazal ölçümlerinde Em, S amplitüdü, izovolümetrik relaksasyon zamanı (İVRZ), miyokard performans indeksi (MPİ) bakımından istatistiksel yönden anlamlı fark saptanırken (p<0,05), sol ventrikü serbest duvar bazalindeki ölçümlerde de Em, S amplitüdü ve İVRZ bakımından istatistiksel yönden anlamlı fark bulundu (p<0,05). Sol ventrikülün serbest duvar bazalindeki strain ve strain rate değerlerinde DMD’ligrup ve kontrol grubu arasında istatistiki yönden  anlamlı fark saptandı (p<0.001). Yapılan apikal dört boşluk pozisyondaki global strain değerlerinde DMD’li grup ve kontrol grubu arasında istatistiki yönden anlamlı fark saptandı ( p<0,001).  Sonuç: Transtorasik ekokardiyografide normal aralık içinde sol ventrikül sistolik fonksiyon saptanan DMD'li asemptomatik hastalarda kontrol grubu ile karşılaştırıldığında sol ventrikül anterolateral duvarında LS ve LSR değerleri anlamlı olarak düşük bulundu. Anahtar kelimeler: Longitudinal strain, longitudinal strain rate, Duchenne musküler 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: İn 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, 

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