"Cardiac MRI (CMR) is able to more precisely characterise the concentric hypertrophic cardiomyopathy (see article for general discussion of CMR features), and resultant changes to cardiac haemodynamics, caused by Danon disease 7,8. Late gadolinium enhancement, often indicative of fibrosis, is nearly always seen in affected patients, most commonly involving the lateral wall but also the anterior and posterior walls, in a subendocardial, intramyocardial or transmural distribution 7. The degree of fibrosis is important because when severe, has been suggested to be an indication for an implantable cardioverter defibrillator to prevent sudden cardiac death from arrhythmogenic events 1,3,7,8."
"The mainstay of treatment is cardiac transplantation 1-3,9. While awaiting transplantation, treatment should be enacted for congestive cardiac failure, including consideration of an implantable cardioverter defibrillator to prevent sudden cardiac death, especially in patients with a high degree of fibrosis shown on cardiac MRI 1,3,7,8. In patients with concurrent arrhythmias, cardiac ablation should also be considered 3. Patients usually do not require treatment for myopathy."
Expected headings
"Ultrasound: echocardiography"
"Cardiac MRI (CMR) is able to more precisely characterise the concentric hypertrophic cardiomyopathy (see article for general discussion of CMR features), and resultant changes to cardiac haemodynamics, caused by Danon disease 7,8. Late gadolinium enhancement, often indicative of fibrosis, is nearly always seen in affected patients, most commonly involving the lateral wall but also the anterior and posterior walls, in a subendocardial, intramyocardial or transmural distribution 7. The degree of fibrosis is important because when severe, has been suggested to be an indication for an implantable cardioverter defibrillator to prevent sudden cardiac death from arrhythmogenic events 1,3,7,8."
"The mainstay of treatment is cardiac transplantation 1-3,9. While awaiting transplantation, treatment should be enacted for congestive cardiac failure, including consideration of an implantable cardioverter defibrillator to prevent sudden cardiac death, especially in patients with a high degree of fibrosis shown on cardiac MRI 1,3,7,8. In patients with concurrent arrhythmias, cardiac ablation should also be considered 3. Patients usually do not require treatment for myopathy."