Respiratory failure was long thought to be the major cause of death in people with Becker muscular dystrophy (BMD), the milder form of Duchenne muscular dystrophy (DMD). Heart failure and life-threatening arrhythmia have since emerged as the leading causes of death for these patients. Researchers, led by Dr. Koichi Kimura of the University of Tokyo, have reviewed the current strategies and suggest treatment options to treat heart failure in BMD.
Their article, titled “Therapeutic Strategy for Heart Failure in Becker Muscular Dystrophy,” was published in the International Heart Journal.
According to the authors, because the symptoms of BMD are generally mild, patients tend to delay their hospital visits. But this can sometimes mean that heart pathology is too advanced for initial treatment. The researchers suggest that early cardiac screening be strongly recommended, and that each patient be provided with in-depth information about the importance of constant hospital visits for evaluations.
Medication such as beta-blockers is a preferred therapy, especially in patients with an elevated heart rate. The authors suggest that an initial treatment with beta-blockers could improve tachycardia and arrhythmia, although further research in this area is needed.
Pacemakers and cardiac resynchronization therapy (CRT) devices are sometimes used in BMD, although recent studies have found these offer limited benefit. Difficulties include inadequate positioning at the time of device implantation due to thoracic deformities, and errors in the determination of device indications due to scoliosis.
Because the risk of device intervention is high, careful consideration and individualized discussions are needed before using this approach.
Corticosteroids are the main medications routinely used to treat both DMD and BMD. These seem to improve both muscle strength and heart function. While most studies suggest that they are only beneficial in DMD patients, some scientists suggest that they can be a treatment option even in the advanced stages of heart failure in BMD.
Points to be considered include the type of glucocorticoid to be used, and the dose and timing of the treatment. Other points include the type of genetic mutation causing the disease, whether developmental heart failure is present, and the patient’s kidney and respiratory functional status.
“Further understanding and optimization of treatment strategies for heart failure are of critical importance to improve prognosis and quality of life in patients with BMD,” the authors concluded.