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

Abstract

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, 

Keywords

• Longitudinal strain,longitudinal strain rate,duchenne muscular dystrophy

References

1. Reference1. Emery AEH. The Muscular Dystrophies. Lancet 2002 ;359:687-695.
2. 2. John SG, James B, Richard D, et al. Recommendations for use ofechocardiography in clinical trials.A report from the american society ofechocardiography’s guidelines and standards committee and the task force on echocardiography. J Am Soc Echocardiogr 2009;9:975-1014
3. 3. Nesbitt GC, Mankad S, OhJ K. Strain imagine chocardiography: methods and clinical applications. Int J Cardiovasc Imaging 2009 ;25:9-22.
4. 4. PellerinD, SharmaR, Elliott P,et al. Tissue Doppler, strain, and strain rate echocardiography for the assesment ofleft and right systolic ventricular function. Heart 2003; 3:9-17.
5. 5. Markham LW, Michelfelder EC, Border WL, et al.Abnormalities of Diastolic Function Precede Dilated Cardiomyopathy Associated with Duchenne Muscular Dystrophy . J Am Soc Echocardiogr 2006 ;7:865-871.
6. 6. Thomas TO, Morgan TM, Burnette WB, et al. Correlation of Heart Rate and Cardiac Dysfunction in Duchenne Muscular Dystrophy. Pediatr Cardiol 2012 ;33:1175-1179.
7. 7. Mori K, Edagawa T, Inoue M, et al. Peak negative myocardial velocity gradient and wall-thickening velocity during early diastole are noninvasive parameters of left ventricular diastolic function in patients with Duchenne's progressive muscular dystrophy. J Am Soc Echocardiogr 2004; 4:322-329.
8. 8. Mertens L, Ganame J, Claus P, et al. Early regional myocardial dysfunction in young patiens with Duchenne muscular dystrophy. J Am Soc Echocardiogr 2008; 9:1049-1054

9. 9. Giatrakos N, Kinali M, Stephens D, et al. Cardiac tissue velocities and strain rate in the early detection of myocardial dysfunction of asymptomatic boys with Duchenne's muscular dystrophy: relationship to clinical outcome. Heart 2006 ;6:840-842.
10. 10. Mori K, Hayabuchi Y, Inoue M, et al.Myocardial strain imaging for early detection of cardiac involvement in patients with Duchenne's progressive muscular dystrophy. Echocardiography 2007: 6:598-608.
11. 11. Ogata H, Nakatani S, Ishikawa Y, et al. Myocardial strain changes in Duchenne muscular dystrophy without overt cardiomyopathy. Int J Cardiol 2007;2:190-195.12. Cox GF, Kunkel LM. Dystrophies and heart disease. Curr Opin Cardiol. 1997:3:329-43.
12. 13.Frankel KA, Rosser RJ. The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis. Hum Pathol 1976 ;4:375-386.
13. 14. Roberts WC, Siegel RJ, McManus BM. Idiopathic dilated cardiomyopathy: analysis of 152 necropsy patients. Am J Cardiol 1987 ;16:1340-1355.
14. 15. Nomura H, Hizawa K. Histopathological study of the conduction system of the heart in Duchenne progressive muscular dystrophy. ActaPathol Jpn 1982;6:1027-1033.
15. 16. Miller G, D’Orsogna L, O’Shea JP. Autonomic function and the sinus tachycardia of Duchenne muscular dystrophy. Brain Dev 1989;4:247-250.
16. 17. Rhodes J, Margossian R, Darras BT, et al. Safety and Efficacy of Carvedilol Therapy for Patients with Dilated Cardiomyopathy Secondary to Muscular Dystrophy. Pediatr Cardiol 2008 ;2:343-351.
17. 18. Sarnat H.B. Muscular dystrophies. In Berhman RE, Kliegman RM, Jenson HB, (eds). Nelson Textbook of Pediatrics, 19th ed. Philedelphia, Saunders; 2012; 2060-2069.
18. 19. Kinali M, Manzur A.Y, Muntoni F. Update on the management of Duchenne muscular dystrophy. Arch Dis Child 2008;93:986-990.
19. 20. Khraiche D, Pellerin D.Tissue Doppler, Doppler strain,and non-Doppler strain:tips, lmitations, and applications.In: Nihoyannopoulos P, Kisslo J (eds). Echocardiography, Springer-VerlagLondon Limited 2009;79-100.
20. 21. Eidem BW. Tissue Doppler echocardiography in children with acquired or congenital heart disease. Pediatrics and ChildHealth 2009;19:98-105.
21. 22.Kajimoto H, Ishigaki K, Okumura K, Tomimatsu H, Nakazawa M, Saito K. Beta-Blocker Therapy for Cardiac Dysfunction in Patients With Muscular Dystrophy. Circ J 2006; 8:991-994.
22. 23. Duboc D, Meune C, Lerebours G, et al.Effect of Perindopril on the Onset and Progression of Left Ventricular Dysfunction in Duchenne Muscular Dystrophy. J am Coll Cardiol 2005 ;6:855-857.
23. 24. Duboc D, Meune C, Pierre B, et al.Perindopril preventive treatment on mortality in Duchenne muscular dystrophy: 10 years' follow-up.Am Heart J 2007; 3:596-602.
24. 25. Ogata H, Ishikawa Y, Ishikawa Y, et al. Beneficial effects of beta-blockers and angiotensin-converting enzyme inhibitors in Duchenne muscular dystrophy. J Cardiol 2009 ;1:72-78.
25. 26. Spurney C, Yu Q, Nagaraju K. Speckle tracking analysis of theleft ventricularanterior wall shows significantly decreased relative radial strain patterns in dystrophin deficient mice after 9 months of age.Version 2. PLoS Curr 2011;19;3:1-5.
26. 27. Carstensen HG, Larsen LH, Hassager C, et al. Basal longitudinal strain predicts future aortic valve replacement in asymptomatic patients with aortic stenosis. Eur Heart J Cardiovasc Imaging 2016;17:283-292.