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September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e322 CLINICAL STATEMENTS AND GUIDELINES 7.7.3 Left Ventricular Assist Device Recommendation for Patients With an LVAD References that support the recommendation are summarized in Online Data Supplement 36. COR LOE Recommendation IIa C-LD 1. In patients with an LVAD and sustained VA, an ICD can be beneficial.S7.7.3-1 Recommendation-Specific Supportive Text 1. Patients with an LVAD have a high risk of VA, particularly those with a history of arrhythmias.S7.7.3-2–S7.7.3-4 The increased risk of VA may be due to myocardial irritation from insertion of the LVAD inflow cannula, LV compression due to a suctioning effect from the LVAD, inotropic support frequently needed by some patients, and repolarization changes that can occur after LVAD placement. Although VT/VF is tolerated by some patients with an LVAD, others experience a decrease in flow as the RV is unsupported; syncope and hypoperfusion can result. Having an ICD can allow for prompt termination of VA before significant hemodynamic consequences occur. Data on ICDs in patients with an LVAD are from observational series. A systematic review of 6 observational studies observed that within 7 months, 26% of patients with an LVAD had died.S7.7.3-1 The death rate was lower among patients who previously had an ICD (16% versus 32%), suggesting a 39% relative-risk reduction in all-cause mortality in an adjusted analysis.S7.7.3-1 Patients with a history of pre-LVAD VA have nearly a ≥10-fold risk of post-LVAD VA.S7.7.3-2–S7.7.3-4 In many of the initial studies demonstrating ICD benefit, older pulsatile LVAD devices were in use.S7.7.3-2,S7.7.3-5 Studies of ICD use with the newer, continuous flow LVADs have inconsistently shown benefit.S7.7.3-1,S7.7.3- 4,S7.7.3-6,S7.7.3-7 Of note, approximately 2 of 10 patients with an LVAD develop an LVAD related infection in the first year.S7.7.3-8,S7.7.3-9 7.7.4 ICD Use After Heart Transplantation Recommendation for ICD Use After Heart Transplantation References that support the recommendation are summarized in Online Data Supplement 37. COR LOE Recommendation IIb B-NR 1. In patients with a heart transplant and severe allograft vasculopathy with LV dysfunction, an ICD may be reasonable if meaningful survival of greater than 1 year is expected.S7.7.4-1–S7.7.4-3 Recommendation-Specific Supportive Text 1. Development of disease in the transplanted heart places some patients at an increased risk of SCD that has ranged from 10% to 35% in observational studies.S7.7.4-4,S7.7.4-5 Both rejection and a decreased LVEF are predictors of SCD. The mechanisms underlying SCD in patients with a heart transplant include damage to the conduction system itself and VA due to coronary vasculopathy or during episodes of acute rejection. Several small case series observing appropriate ICD termination of VA suggest that an ICD can be beneficial in selected patients, particularly those with severe allograft vasculopathy, unexplained syncope, a history of SCA, and severe LV dysfunction.S7.7.4-1–S7.7.4-3 Additionally, a patient with severe allograft vasculopathy who is being considered for retransplant may be appropriate for an ICD as a bridging device. Secondary prevention indications for an ICD in patients with a heart transplant are identical to those in other patients. 7.8 Neuromuscular Disorders Recommendations for Neuromuscular Disorders References that support the recommendations are summarized in Online Data Supplement 38. COR LOE Recommendations I B-NR 1. In patients with neuromuscular disorders, primary and secondary prevention ICDs are recommended for the same indications as for patients with NICM if meaningful survival of greater than 1 year is expected.S7.8-1,S7.8-2 IIa B-NR 2. In patients with Emery-Dreifuss and limbgirdle type IB muscular dystrophies with progressive cardiac involvement, an ICD is reasonable if meaningful survival of greater than 1 year is expected.S7.8-3–S7.8-8 IIa B-NR 3. In patients with muscular dystrophy, followup for development of cardiac involvement is reasonable, even if the patient is asymptomatic at presentation.S7.8-9–S7.8-12 IIb B-NR 4. In patients with myotonic dystrophy type 1 with an indication for a permanent pacemaker, an ICD may be considered to minimize the risk of SCA from VT if meaningful survival of greater than 1 year is expected.S7.8-9,S7.8-13,S7.8-14 Synopsis The muscular dystrophies are a group of inherited diseases affecting skeletal and cardiac muscle. Some present primarily as a NICM (eg, Duchenne, Becker, and limb-girdle types 2C, 2F, and 2I), while others present primarily as conduction system degeneration with a variable association with cardiomyopathy (eg, myotonic dystrophy types 1 and 2, Emery-Dreifuss, limb-girdle type 1B; summarized in Table 9).S7.8-15 Because SCD can occur either due to VA or due to bradyarrhythmias from rapid and unpredictable progression of conduction system disease, the clinician is faced with the challenge of identifying those patients who would benefit from prophylactic pacemak- Downloaded from http://ahajournals.org by on October 7, 2026