Abstract
Cardiovascular magnetic resonance (CMR) studies in patients with pacemakers or implantable cardioverter/defibrillators (ICD) are increasingly required in daily clinical practice. Therefore, in the last years the manufacturers developed not only MR-conditional pacemakers, but also MR-conditional ICDs. However, the clinical experience regarding the feasibility and limitations of MR studies of the heart in patients with ICDs is still limited. In particular, there are hardly any CMR studies in the same patients performed prior to and post ICD implantation allowing a one-to-one comparison of the obtained CMR images. This is the first presentation of a CMR study in a patient with the world’s first and so far only MR-conditional ICD. In our case, a major problem related to the presence of the MR conditional ICD was an image artifact caused by the device’s generator which hampered the visualization of the midventricular and apical anterior and antero-lateral segments in all sequences performed. Considering previous studies, right chest implantation of the ICD could probably have helped in this setting and may be preferred in future ICD implantations. Our case report nicely illustrates the real clinical need for specially designed implantable devices that ensure safe and high-quality imaging in patients in whom serial CMR is required.
Highlights
Becker muscular dystrophy is an inherited, recessive, X-linked dystrophin deficiency disease with similar, but more benign clinical picture compared to Duchenne muscular dystrophy
Screening by echocardiography and cardiovascular magnetic resonance (CMR) had revealed cardiac involvement already three years ago and etiology was confirmed by endomyocardial biopsy with immunohistochemical proof of myocardial dystrophin deficiency at that time
To the best of our knowledge, this is the first presentation of MR of the heart in a patient with the world’s first
Summary
Becker muscular dystrophy is an inherited, recessive, X-linked dystrophin deficiency disease with similar, but more benign clinical picture compared to Duchenne muscular dystrophy. In muscular dystrophy patients with cardiac involvement, imaging by cardiovascular magnetic resonance (CMR) - in addition to current standard of care (monitoring by ECG and echocardiography) brings several advantages: By providing accurate and reproducible measurements for left ventricular (LV) volumes and function as well as by visualizing the characteristic non-ischemic pattern of late gadolinium. Case presentation A 26-year-old male patient with Becker muscular dystrophy and secondary dilated cardiomyopathy received an MR-conditional ICD (ICD; Biotronik Lumax 740 VRT DX) for primary prevention of sudden cardiac death. Before ICD implantation, he underwent regular follow-up echocardiography and CMR studies (every year) as a participant in an ongoing research project on patients with muscular dystrophy. Severely impaired systolic function (LV-EF = 35%) due to diffuse hypokinesia, highly pronounced in the inferior and lateral free wall (Figure 1A-C, left panels; Movies 1A-C in Additional file 1). Due to a) the important added value of the CMR-derived information regarding the pattern and severity of myocardial disease and b) the necessity for close follow-up, a “MR conditional” single chamber ICD was implanted in 2011
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