The evolution of electrocardiographic changes in patients with Duchenne muscular dystrophies

Abstract

PurposeMyocardial dysfunction and dysrhythmias are inevitable consequences of Duchenne muscular dystrophy. We aimed to evaluate specific trends of electrocardiographic changes that reflect the progress of cardiomyopathy in patients with Duchenne muscular dystrophy.MethodsFifty electrocardiograms (ECGs) of 30 patients (ages 1 to 27 years) who had not been prescribed medications for heart failure treatment at the time of examination were retrospectively analyzed and compared with 116 ECGs of age-matched healthy 116 controls. Heart rate, leads with fragmented QRS (fQRS), corrected QT, Tpeak-to-Tend, and Tpeak-to-Tend/QT were analyzed.ResultsThe patients with Duchenne muscular dystrophy failed to show a normal age-related decline in heart rate but showed an increasing trend in the prevalence of fQRS, corrected QT, corrected Tpeak-to-Tend, and Tpeak-to-Tend/QT over time. In the ≤10-year-old patient group, a significant difference was found only in the prevalence of fQRS between the patients and the controls. The prevalence of fQRS, heart rate, Tpeak-to-Tend/QT, and corrected Tpeak-to-Tend demonstrated significant differences between the patients and the controls in the middle age group (11 to 15 years old). All the indexes were statistically significantly different in the ≥16-year-old patient group.ConclusionThe prevalence of lead with fQRS representing regional wall motion abnormalities was higher in the young patients than in the young healthy controls, and this might be one of the first signs of myocardial change in the patients. Markers of depolarization and repolarization abnormalities were gradually prominent in the patients aged >10 years. Further studies are needed to confirm these findings.

Duchenne muscular dystrophy trajectory in R-DMDdel52 preclinical rat model identifies COMP as biomarker of fibrosis

“…This increased QTpc value is very specific to R-DMDdel52 rats, making QTpc the best functional discriminating criterion, equivalent in reliability to genotyping. Similar modifications, quantified here with neither sedative nor anesthetic molecules known to interfere with ECG values [ 47 ], have previously been identified in DMD patients [ 7 , 41 , 53 , 70 ]. The prolonged QTpc interval with normal P waves points to a ventricular de- or repolarization defect, likely caused by the progressive cardiac fibrosis observed in DMD rats.…”

Clinical utility of 12‐lead electrocardiogram in evaluating heart disease in patients with muscular dystrophy: Assessment of left ventricular hypertrophy, conduction disease, and cardiomyopathy

“…(Haukilahti et al, 2016 ) Considerations of fQRS have previously been applied to patients with DMD as a representation of regional wall motion abnormalities, and as an early indicator of adverse cardiac remodeling in these patients. (Cho et al, 2017 ; Yoo et al, 2017 ) We believe that this concept can be applied to our broader MD cohort considering its prevalence and the associated reduction in LV systolic function. Importantly, fQRS has been shown to be a reliable indicator of adverse cardiac remodeling in the presence of confounding ECG findings, which is relevant to our cohort given the high prevalence of conduction abnormalities.…”

“…(Baldasseroni et al, 2002 ; Iuliano et al, 2002 ) Additionally, QRS fragmentation (fQRS) has been associated with major adverse cardiac events (MACE) including life‐threatening arrhythmias and mortality in patients with heart disease, (Das et al, 2008 ; Terho et al, 2014 ) as well as an association with systolic dysfunction in patients with DMD. (Cho et al, 2017 ; Yoo et al, 2017 ) We identified ECG‐LVH, LBBB, and fQRS as common and recurrent ECG features in our heterogenous MD patient cohort, and we investigated their clinical utility for the front‐line cardiac assessment of patients with MD.…”

Duchenne Muscular Dystrophy (DMD) with Severe Cardiomyopathy

“…It is important to recognize baseline ECG abnormality as a predictor of disease progression. Common ECG abnormalities include a Q wave in lead III or V6, right ventricular hypertrophy, sinus tachycardia, tall right precordial R waves, and short PR interval [11].…”