Cardiac Events in Duchenne, Becker Muscular Dystrophies Can Now Be Predicted

Cardiac Events in Duchenne, Becker Muscular Dystrophies Can Now Be Predicted

PH and sclerodermaA study entitled “Left ventricular systolic function and the pattern of late-gadolinium-enhancement independently and additively predicts adverse cardiac events in muscular dystrophy patients” published in the September issue of the Journal of Cardiovascular Magnetic Resonance report two new independent predictors for adverse cardiac events in Duchenne and Becker muscular dystrophies.

Duchenne and Becker muscular dystrophies (DMD and BMD) are characterized by absence or defected production of dystrophin, a key protein connecting muscle fibers to the surrounding extracellular matrix, leading to degeneration of skeletal, smooth and cardiac muscle. Consequently, cardiac involvement with progressive heart failure and arrhythmias is associated with increased mortality in DM and BMD patients. Recently, cardiovascular magnetic resonance (CMR) has been used to diagnose and follow-up cardiac involvement in these patients; however, few studies have reported its role on assessing such factors in DM/BMD patients.

Thus, in this study the authors aimed to determine how cardiac involvement measured by CMR associates with death and adverse cardiac events in DM/BMD patients.

The authors analyzed eighty-eight male patients (20 DMD and 68 BMD), for approximately four years, who completed a cardiovascular magnetic resonance (CMR) comprising cine- and late-gadolinium-enhancement (LGE)-CMR and subsequently followed for adverse cardiac events. The study primary endpoint included cardiac death or cardiac transplantation and a secondary endpoint hospitalization due to heart failure or non/sustained ventricular tachycardia (VT).

The authors were able to identify independent predictors for adverse cardiac events in DM/BMD patients. Specifically, left ventricular (LV) systolic dysfunction, the best independent predictor, and the CMR parameter termed “presence of transmural LGE.” The latter was also an important factor to risk stratification of DMD/BMD patients.

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