How are cardiac diseases treated in limb-girdle muscular dystrophy (LGMD)?

Updated: Aug 15, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Many LGMDs have associated cardiac disease. Newly diagnosed patients with LGMDs known to have cardiac involvement (LGMD1A, LGMD1B, LGMD1C, LGMD1E, LGMD2C-F, LGMD2G, LGMD2I, LGMD2M, LGMD2N, LGMD2R, LGMD2T, LGMD2U, LGMD2W, LGMD2X) should have early referral to a cardiologist. Cardiology referral should also be made for undiagnosed patients with LGMD.

Testing should include EKG and echocardiography. If these are abnormal or if cardiac symptoms develop, other tests may be needed including cardiac MRI, Holter monitoring, and event monitoring. Cardiac arrhythmias can be a major cause of morbidity and mortality (sudden cardiac death) and placement of a pacemaker can be a life-saving procedure.


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