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The Diagnostic Accuracy of Different Echocardiographic Predictors of Subtle Myocardial Dysfunction in Asymptomatic Duchenne Muscular Dystrophy Patients


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1 Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
     

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Introduction: Duchennemuscular dystrophy (DMD) is an uncommon X-linked disease affecting in average 1 in 3600 live male births. Cardiac disease is the major cause of mortality in affected patients. Several Echocardiographic parameters are available for evaluation of systolic and diastolic function among Duchenne patients. The aim of this paper is to determine which of those parameters has the highest accuracy in detection subtle myocardial affection in asymptomatic Duchenne patients. Method:28 asymptomatic Duchenne patients as well as 28 age and sex matched healthy controls have been screened with Echocardiography including Motion mode, conventional and Tissue Doppler, 2 Dimensional (2D) and 3 Dimensional (3D) speckle tracking Echocardiography. Results: Conventional Echocardiographic parameters failed to differentiate cases from controls while Tissue Doppler derived parameter such as the ratio between Transmitral flow velocity in early diastole and Left Ventricular averaged basal septal and lateral tissue velocity (LV E/E’) as well as global longitudinal (GLS) and Global Circumferential strain (GCS) (2D and 3D) proved to be useful in differentiation between them. 3D derived ejection fraction also showed statistically significant difference between cases and controls (54.2±4.5 vs. 70.2±6.1, P Value<0.001) respectively, 3D derived parameters showed the highest sensitivities and specificities among relevant parameters (100%) for detection of subtle myocardial affection in Duchenne patients. Conclusion: This paper concludes that 3 D derived EF, GCS and GLS can be reliably used in detection of subtle myocardial affection in Duchenne patients.

Keywords

Duchenne, Cardiac Affection,3D Speckle Tracking, 3D Ejection Fraction.
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  • The Diagnostic Accuracy of Different Echocardiographic Predictors of Subtle Myocardial Dysfunction in Asymptomatic Duchenne Muscular Dystrophy Patients

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Authors

Omnia Raafat
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Reem Ibrahim Ismail
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Hanan Zekri
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Antoine Abdelmassih
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Noha Ali
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Erini Farid
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Shaimaa Atef
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt
Habiba-Allah Ismail
Cairo University (Kasr Al Ainy), Manial Street, P.O. BOX: 12411, Egypt

Abstract


Introduction: Duchennemuscular dystrophy (DMD) is an uncommon X-linked disease affecting in average 1 in 3600 live male births. Cardiac disease is the major cause of mortality in affected patients. Several Echocardiographic parameters are available for evaluation of systolic and diastolic function among Duchenne patients. The aim of this paper is to determine which of those parameters has the highest accuracy in detection subtle myocardial affection in asymptomatic Duchenne patients. Method:28 asymptomatic Duchenne patients as well as 28 age and sex matched healthy controls have been screened with Echocardiography including Motion mode, conventional and Tissue Doppler, 2 Dimensional (2D) and 3 Dimensional (3D) speckle tracking Echocardiography. Results: Conventional Echocardiographic parameters failed to differentiate cases from controls while Tissue Doppler derived parameter such as the ratio between Transmitral flow velocity in early diastole and Left Ventricular averaged basal septal and lateral tissue velocity (LV E/E’) as well as global longitudinal (GLS) and Global Circumferential strain (GCS) (2D and 3D) proved to be useful in differentiation between them. 3D derived ejection fraction also showed statistically significant difference between cases and controls (54.2±4.5 vs. 70.2±6.1, P Value<0.001) respectively, 3D derived parameters showed the highest sensitivities and specificities among relevant parameters (100%) for detection of subtle myocardial affection in Duchenne patients. Conclusion: This paper concludes that 3 D derived EF, GCS and GLS can be reliably used in detection of subtle myocardial affection in Duchenne patients.

Keywords


Duchenne, Cardiac Affection,3D Speckle Tracking, 3D Ejection Fraction.



DOI: https://doi.org/10.37506/v11%2Fi2%2F2020%2Fijphrd%2F195129