Re-examination of the Electrocardiogram in Boys With Duchenne Muscular Dystrophy and Correlation With Its Dilated Cardiomyopathy

Department of Pediatrics, The Ohio State University, and the Heart Center, Nationwide Children's Hospital, Columbus, Ohio, USA.
The American journal of cardiology (Impact Factor: 3.43). 01/2009; 103(2):262-5. DOI: 10.1016/j.amjcard.2008.08.064
Source: PubMed

ABSTRACT Duchenne muscular dystrophy (DMD) results in dilated cardiomyopathy (DC). Characteristic electrocardiographic (ECG) changes include short PR interval, right ventricular hypertrophy (RVH), prolonged QTc interval, and prominent Q waves in leads I, aVL, V5, and V6 or in leads II, III, aVF, V5, and V6. We re-examined the prevalence and correlation of ECG changes with DC in DMD. Electrograms of 115 patients with DMD were evaluated. DC was defined as an echocardiographic ejection fraction<55%. PR interval and RVH were based on age-based normal values. Abnormal Q waves were >or=4 mm. Abnormal QTc interval was >or=450 ms. ST-segment depression was defined as >0.5 mm. Fisher's exact test evaluated significant differences between groups and logistic regression determined whether number of ECG changes predicted DC. Forty had DC. No significant differences existed between the number of ECG changes in DC and non-DC groups (p=0.279). Distribution of findings included short PR interval (43%), RVH (37%), prominent Q waves in leads V5 (34%) and V6 (33%), prominent Q waves in leads I, aVL, V5, and V6 (3, 1 with DC), prominent Q waves in leads II, III, aVF, V5, and V6 (9, 4 with DC), long QTc interval (0), ST depression (2, 1 with DC), and flat/biphasic ST segments (38, 15 with DC). In conclusion, ECG changes are similar in patients with DMD regardless of presence of DC. Previously reported characteristic ECG changes are seen in a minority of DMD cases. The most common findings are short PR interval and RVH. Prominent Q waves in leads II, III, aVF, V5, and V6 are more likely.

  • [Show abstract] [Hide abstract]
    ABSTRACT: The dystrophinopathies Duchenne and Becker muscular dystrophies (DMD and BMD) represent the most common inherited disorders of muscle. Improvements in cardiac care, attention to respiratory function, and judicious use of spinal correction surgery have led to increased survival in the DMD population. Meanwhile, advances in molecular therapeutics have led to promising therapies that are in or are entering clinical trials. An understanding of the dystrophinopathies, and recent advances in their molecular diagnosis and treatment, is of benefit to practicing neurologists.
    Neurologic Clinics 08/2014; 32(3):671–688. DOI:10.1016/j.ncl.2014.05.002 · 1.61 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades.
    Pediatric Clinics of North America 04/2015; DOI:10.1016/j.pcl.2015.03.008 · 2.20 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The medical care of patients with Duchenne muscular dystrophy (DMD) is an interdisciplinary and multifaceted task. The vast majority of those affected show a nearly constant course which is reflected in a corresponding stage-oriented treatment concept. Although there is still no causal therapy available for DMD, the course and in particular the quality of life of patients can be decisively improved by established medical practices. The orthopedic problems of DMD patients include contractures of the upper and lower extremities as well as sitting instability due to progressive scoliosis with pelvic imbalance. The orthopedic treatment incorporates conservative measures, such as physiotherapy, provision of orthotic devices and wheelchairs as well as surgery to resolve contractures of the lower extremities and surgical stabilization of the spine. Furthermore, in these patients orthopedic surgeons and trauma surgeons are confronted with the treatment and prophylaxis of fractures induced by osteoporosis. An early onset of glucocorticoid therapy markedly delays the loss of motor abilities. An important aspect in the care of DMD patients is the timely prophylaxis and treatment of respiratory insufficiency with regular sessions of breathing therapy, learning breathing and coughing techniques and the sufficiently early start of non-invasive mechanically assisted ventilation. Of similar relevance are also the early recognition and cardioprotective treatment of cardiomyopathy. The orthopedic surgeon accompanies the patient and family through all stages of the disease and must be appropriately informed on current management and treatment strategies even outside the limits of the personal field of specialization.
    Der Orthopäde 06/2014; 43(7). DOI:10.1007/s00132-013-2217-7 · 0.67 Impact Factor