Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases.
Gaspar Del Rio-PertuzCristina MoratayaKanak ParmarSarah DubayErwin Argueta-SosaPublished in: Orphanet journal of rare diseases (2022)
There are scarce publications regarding the presentation and outcome of Becker muscular dystrophy in adulthood when idiopathic dilated cardiomyopathy is the initial disease manifestation. We performed a systematic review using Medline, Embase, Cochrane, and Scopus to identify cases of adults with idiopathic dilated cardiomyopathy who were subsequently diagnosed with Becker muscular dystrophy from inception through August 2020. Six cases were found. We identified young males (Median age: 26 years) with Becker muscular dystrophy who first presented with dilated cardiomyopathy. Most patients initially presented with congestive heart failure symptoms (5/6, 83%), and had a median left ventricular ejection fraction of 23%. One case did have calf pseudohypertrophy. Musculoskeletal symptoms later appeared one to six years after the initial dilated cardiomyopathy presentation. Heart transplantation was the most common management strategy (4/6, 67%). A left ventricular assist device was used in one case as a bridge to heart transplant. Dilated cardiomyopathy can be the initial presentation of Becker muscular dystrophy in the third to fourth decades of life in adult patients, and musculoskeletal symptoms can be subclinical.
Keyphrases
- muscular dystrophy
- ejection fraction
- aortic stenosis
- heart failure
- duchenne muscular dystrophy
- left ventricular
- left ventricular assist device
- case report
- end stage renal disease
- sleep quality
- newly diagnosed
- chronic kidney disease
- atrial fibrillation
- randomized controlled trial
- depressive symptoms
- prognostic factors
- cardiac resynchronization therapy
- systematic review
- hypertrophic cardiomyopathy
- transcatheter aortic valve replacement
- aortic valve
- mitral valve
- physical activity
- acute heart failure
- percutaneous coronary intervention
- meta analyses
- acute coronary syndrome