Sandbox: Neuromuscular disease

Template:Neuromuscular disease AHA -2017 Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1],Associate Editor(s)-in-Chief: Arzu Kalayci, M.D. [2]

Approach to Cardiac Evaluation in Neuromuscular Diseases (NMDs)

Management of Cardiac Involvement Associated With Neuromuscular Diseases

Class I

"1. NMD providers and patient organizations should promote education regarding the importance of a proactive approach to screening, diagnosis, and management of cardiovascular complications of NMDs and the ideal care team required.(Level of Evidence: B) "

"2. All neurologists diagnosing and managing NMDs should work to identify either a car- diologist with expertise in these conditions or at minimum a collaborative electrophysiologist or HF specialist, depending on the condition being evaluated.(Level of Evidence: B) "

"3. Cardiac evaluation should be performed before anesthesia or sedation in any patient with NMD at risk for cardiac involvement. For those with a history or symptoms suggestive of cardiac involvement, cardiac evaluation should be in close proximity (3–6 months) to the anesthesia/sedation event.(Level of Evidence: C) "

"4. For NMD patients believed to be at increased cardiac risk during surgery, cardiac monitoring by an anesthesiologist experienced in the care of patients with NMDs should occur during major surgery, and the procedure should take place in a center with appropri- ate intensive care facilities.(Level of Evidence: C) "

Class IIa

"1. For conditions diagnosed in childhood, refer- ral to a pediatric HF specialist, when prac- ticable, is reasonable because of evolving diagnostic and management recommenda- tions within pediatric cardiomyopathies. (Level of Evidence: B) "

Cardiac Evaluation in Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD)

Class I

"1. All DMD and BMD patients should have an initial cardiac evaluation with examination, ECG, and imaging performed at diagnosis. (Level of Evidence: B) "

"2. Asymptomatic DMD/BMD patients with left ventricular dilation or dysfunction or arrhythmia (eg, supraventricular tachycardia, ventricular ectopy) should be reevaluated at least annually. (Level of Evidence: C) "

"3. Symptomatic DMD/BMD patients should be reevaluated more frequently than annually, with testing and frequency determined by the provider and clinical status. (Level of Evidence: C) "

"4. Female DMD/BMD carriers should undergo cardiac evaluation by examination, ECG, and noninvasive imaging in the second to third decade of life, with follow-up evaluations every 3 to 5 years thereafter. (Level of Evidence: C) "

"5. Echocardiography should be routinely used in the screening and follow-up care of DMD/ BMD patients. (Level of Evidence: B) "

Class IIa

"1. Every-2-year cardiac evaluation by examina- tion, ECG, and noninvasive imaging is rea- sonable in asymptomatic DMD/BMD patients <10 years of age, increasing to annual evalu- ation at 10 years of age. (Level of Evidence: B) "

"2. It is reasonable to consider periodic use of advanced tissue imaging modalities (eg, CMR with contrast) in the care of DMD/BMD patients for assessment of cardiac function, particularly in patients with poor acoustic windows or for assessment of myocardial fibrosis. (Level of Evidence: B) "

"3. Ambulatory electrocardiographic monitor- ing for patients with DMD/BMD is reasonable every 1 to 3 years, based on age, EF, and clinical assessment. (Level of Evidence: C) "

"4. In the absence of an implantable cardio- verter-de brillator (ICD) or other arrhythmia monitoring, at least annual ambulatory electrocardiographic monitoring is reason- able for patents with DMD/BMD with EF <35% or age ≥17 years. (Level of Evidence: B) "

Cardiac Evaluation in Limb-girdle muscular dystrophies (LGMD)

Class I

"1. In patients with LGMD, complete cardiac evaluation should begin at the time of diagnosis and should include examination, ECG, and echocardiography. (Level of Evidence: C) "

"1. LGMD patients with heart failure (HF) or those on HF therapy should be followed up more frequently. (Level of Evidence: C) "

Class IIa

"1. Follow-up cardiac evaluations to include examination, ECG, and echocardiography every 2 years for asymptomatic LGMD2C-F (sarcoglycanopathy) and LGMD2I patients (FKRP) with normal cardiac findings, and at least annually for those with abnormal cardiac findings, is reasonable. (Level of Evidence: C) "

"1. Follow-up cardiac evaluations to include examination, ECG, and ambulatory electro- cardiographic monitoring should be repeated every 2 years for asymptomatic LGMD1B patients with normal cardiac findings and annually for those with abnormal cardiac findings. Symptoms of palpitations, dizziness, or syncope should prompt additional investigation with ambulatory electrocardiographic monitoring, loop event electrocardiographic recording, or electrophysiology study as warranted. (Level of Evidence: C) "

Recommendations for Emery-Dreifuss muscular dystrophy (EDMD)

Class I

"1. Individuals with EDMD, regardless of genotype, should be referred for cardiology assessment at the time of EDMD diagnosis, even if asymptomatic. (Level of Evidence: C) "

Class IIa

"1. At least annual evaluation with echocardio- gram, ECG, and ambulatory ECG is reason- able for patients with autosomal dominant and X-linked recessive EDMD. (Level of Evidence: C) "

"2. Annual ECG and ambulatory ECG are reason- able for autosomal recessive EDMD. (Level of Evidence: C) "