Abstract
BackgroundWith the continuous improvement of the respiratory care of Duchenne muscular dystrophy patients, cardiac manifestations (heart failure and arrhythmias) become the leading causes of morbidity and mortality. Early identification of cardiac muscle affection is crucial to start anti-failure drugs that reverse remodeling and improve prognosis. This study aimed to detect subtle cardiac changes in Duchenne muscular dystrophy patients and carriers using electrocardiography and echocardiography.ResultsThis study included genetically diagnosed Duchenne muscular dystrophy patients (28 males) and carriers (25 females) and compared them to healthy gender-matched control groups. All study participants underwent clinical assessment, 12-lead electrocardiography, and global longitudinal strain augmented echocardiography. In the current study, Duchenne muscular dystrophy patients had higher heart rates, smaller left ventricular internal diameters, left atrial diameter, lower ejection fraction, and worse left ventricular global longitudinal strain in comparison with the control group. The global longitudinal strain inversely correlated with the age of Duchenne muscular dystrophy patients. The number of exon mutations did not affect electrocardiography and echocardiographic findings. Exon mutations 45–47 and 51–54 were significantly associated with an ejection fraction less than 60%. Duchenne muscular dystrophy carriers had smaller left ventricular wall diameters, left ventricular end-diastolic diameter, left atrial diameter, and worse left ventricular global longitudinal strain in comparison with the control group.ConclusionsLeft ventricular global longitudinal strain could detect subtle left ventricular systolic dysfunction in Duchenne muscular dystrophy patients and carriers before the decline of left ventricular ejection fraction.
Highlights
With the continuous improvement of the respiratory care of Duchenne muscular dystrophy patients, cardiac manifestations become the leading causes of morbidity and mortality
The genetic diagnosis was done using multiplex ligation-dependent probe amplification (MLPA) in Centogene laboratories, Germany using SALSA MLPA probemix P034-B2/P035-B1 provided by MRC-Holland to test for deletion or duplication exon mutations of the Duchenne muscular dystrophy (DMD) gene (Gene parts included in the messenger ribonucleic acid that are translated into proteins are known as exons)
Duchenne muscular dystrophy patients On comparing ECG and echocardiographic findings between group A and B, group A had a smaller LV end-diastolic diameter (LVEDD), LV end-systolic diameter (LVESD), left atrium (LA) diameter, higher heart rate, lower E/e’ ratio, lower Left ventricular (LV) ejection fraction (EF) using modified Simpson’s method, and worse global longitudinal strain (GLS) compared to group B (Fig. 1)
Summary
With the continuous improvement of the respiratory care of Duchenne muscular dystrophy patients, cardiac manifestations (heart failure and arrhythmias) become the leading causes of morbidity and mortality. This study aimed to detect subtle cardiac changes in Duchenne muscular dystrophy patients and carriers using electrocardiography and echocardiography. Respiratory and cardiac muscle weakness is considered the most common cause of morbidity and mortality in these patients [2]. With the continuous improvement of their respiratory care, cardiac manifestations (heart failure and arrhythmias) become the leading causes of morbidity and mortality. Cardiac manifestations may occur in some female carriers [3]. Identification of cardiac muscle affection is crucial to start anti-failure drugs that reverse remodeling and improve prognosis. Transthoracic echocardiography is a simple alternative that is more suitable for those younger than
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