Guideline sources The following summarized guidelines for the evaluation and management of dilated cardiomyopathy are prepared by our editorial team based on guidelines from the Heart Failure Society of America (HFSA/AHA/ACC 2022), the European Society of Cardiology (ESC 2022; 2021), the American Heart Association (AHA/ASA 2021), the American Heart Association (AHA/HRS/ACC 2018), the American Heart Association (AHA 2016), the American Heart Association (AHA/ACC 2015), and the European Heart Rhythm Association (EHRA/HRS 2011). 1 2 3 4 5 6 7 8 Calculator Calculator ESC diagnostic criteria for dilate NYHA functional classification f Guidelines 1. Screening and diagnosis f f https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 S i il l ti 1/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Screening of family relatives: As per ESC 2022 guidelines: Obtain an ECG and echocardiography in a first-degree relative of patients with dilated cardiomyopathy if: the index patient was diagnosed at < 50 years of age or has clinical features suggestive of an inherited cause there is a family history of dilated cardiomyopathy or premature unexpected sudden death. B Consider obtaining an ECG and echocardiography in a first-degree relative of patients with apparently sporadic dilated cardiomyopathy. C As per AHA 2022 guidelines, offer genetic screening and counseling in first-degree relatives of selected patients with genetic or inherited cardiomyopathies to detect cardiac disease and prompt consideration of treatments to decrease HF progression and sudden death. B As per AHA 2016 guidelines, elicit a 3-generational family history to aid in establishing the diagnosis of familial dilated cardiomyopathy in patients with idiopathic dilated cardiomyopathy. B Show 2 more As per HRS 2011 guidelines, obtain mutation-specific genetic testing in family members and appropriate relatives following the identification of a dilated cardiomyopathy-causative mutation in the index case. A 2. Diagnostic investigations Cardiac MRI: As per ESC 2022 guidelines, consider obtaining cardiac MRI with late gadolinium enhancement to assess the etiology and the risk of ventricular arrhythmia/sudden cardiac death in patients with dilated cardiomyopathy. C As per ESC 2021 guidelines, consider obtaining cardiac MRI with late gadolinium enhancement in patients with dilated cardiomyopathy to distinguish between ischemic and non-ischemic myocardial damage. C As per AHA 2018 guidelines: Consider obtaining cardiac MRI with late gadolinium enhancement for diagnosis in patients with suspected non-ischemic cardiomyopathy from myocardial infiltrative processes. B Consider obtaining cardiac MRI with late gadolinium enhancement for assessing the risk of sudden cardiac arrest/sudden cardiac death in patients with suspected non-ischemic cardiomyopathy. C Genetic testing: As per ESC 2022 guidelines: Obtain genetic testing (including at least lamin A/C, PLN, RBM20, and FLNC genes) in patients with dilated cardiomyopathy and atrioventricular conduction delay at < 50 years, or having a family history of dilated cardiomyopathy or sudden cardiac death in a first-degree relative (at age < 50 years). B https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 2/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Consider obtaining genetic testing (including at least lamin A/C, PLN, RBM20, and FLNC genes) for risk stratification in patients with apparently sporadic dilated cardiomyopathy presenting at a young age or with signs of suspicion for an inherited etiology. C As per AHA 2022 guidelines, consider referring selected patients with non-ischemic cardiomyopathy for genetic counseling and testing to identify conditions likely to guide treatment in patients and family members. C As per AHA 2018 guidelines, consider providing genetic counseling and obtaining genetic testing in patients with non-ischemic cardiomyopathy developing conduction disease or LV dysfunction at < 40 years of age or having a family history of non-ischemic cardiomyopathy or sudden cardiac death in a first-degree relative (< 50 years of age) to detect a heritable disease to clarify prognosis and facilitate cascade screening of relatives. C As per AHA 2016 guidelines: Consider obtaining genetic testing in conjunction with genetic counseling in patients with familial or idiopathic cardiomyopathy. C Consider obtaining genetic testing in patients with familial dilated cardiomyopathy to confirm the diagnosis, to facilitate cascade screening within the family, and to help with family planning. B As per HRS 2011 guidelines: Obtain comprehensive or targeted (LMNA and SCN5A) genetic testing in patients with dilated cardiomyopathy and significant cardiac conduction disease (first-, second-, or third- degree heart block) and/or a family history of premature unexpected sudden death. A Consider obtaining genetic testing in patients with familial dilated cardiomyopathy to confirm the diagnosis, identify patients at the highest risk of arrhythmia and syndromic features, facilitate cascade screening within the family, and help with family planning. C 3. Diagnostic procedures Electrophysiology study: As per ESC 2022 guidelines, consider obtaining electrophysiological evaluation in patients with dilated cardiomyopathy when syncope remains unexplained after noninvasive evaluation. C As per AHA 2018 guidelines: Consider obtaining electrophysiologic testing to evaluate the risk of sustained VT in patients with non-ischemic cardiomyopathy presenting with syncope or other ventricular arrhythmia symptoms and not meeting indications for a primary prevention ICD. C Consider performing ICD placement or obtaining electrophysiologic testing for risk stratification for sudden cardiac death in patients with non-ischemic cardiomyopathy experienced syncope presumed to be due to ventricular arrhythmia and not meeting indications for a primary prevention ICD, if the expected meaningful survival is > 1 year. C 4. Medical management Antiarrhythmic agents: https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 3/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway As per ESC 2022 guidelines, consider adding oral amiodarone or replacing -blockers with sotalol in patients with dilated cardiomyopathy and an ICD experiencing recurrent, symptomatic ventricular arrhythmia despite optimal device programming and -blocker treatment. C As per AHA 2018 guidelines: Consider initiating amiodarone for the prevention of sudden cardiac death in patients with non-ischemic cardiomyopathy survived a cardiac arrest, having sustained VT or symptomatic ventricular arrhythmia, and are ineligible for an ICD (due to a limited life- expectancy and/or functional status or lack of access to an ICD). C Consider initiating amiodarone or sotalol in patients with non-ischemic cardiomyopathy and an ICD experiencing spontaneous ventricular arrhythmia or recurrent appropriate shocks despite optimal device programming and treatment with a -blocker. C 5. Therapeutic procedures Implantable cardioverter-defibrillator, primary prevention of SCD: As per ESC 2022 guidelines, consider performing ICD placement in patients with dilated cardiomyopathy, symptomatic HF (NYHA class II-III), and LVEF 35% after 3 months of optimal medical treatment. B Show 2 more As per HFSA/ACC/AHA 2022 guidelines, perform ICD placement for primary prevention of sudden cardiac death to reduce total mortality in patients with non-ischemic dilated cardiomyopathy or ischemic heart disease at least 40 days post-myocardial infarction with LVEF 35% and NYHA class II-III symptoms on chronic guideline-directed medical therapy, if the expected meaningful survival is > 1 year. A As per HRS/ACC/AHA 2018 guidelines, perform ICD placement in patients with non-ischemic cardiomyopathy, HF with NYHA class II-III symptoms, and LVEF of 35% despite guideline- directed medical therapy, if the expected meaningful survival is > 1 year. A Show 3 more Implantable cardioverter-defibrillator, secondary prevention of SCD: As per ESC 2022 guidelines: Perform ICD placement in patients with dilated cardiomyopathy survived sudden cardiac arrest due to VT/VF or experienced hemodynamically not-tolerated sustained monomorphic VT. B Consider performing ICD placement in patients with dilated cardiomyopathy and hemodynamically tolerated sustained monomorphic VT. C As per HRS/ACC/AHA 2018 guidelines: Perform ICD placement in patients with non-ischemic cardiomyopathy either survived sudden cardiac arrest due to VT/VF or experienced hemodynamically unstable VT B or stable sustained VT B not due to reversible causes, if the expected meaningful survival is > 1 year. B Consider performing ICD placement or obtaining electrophysiologic testing for risk stratification for sudden cardiac death in patients with non-ischemic cardiomyopathy https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 4/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway experienced syncope presumed to be due to ventricular arrhythmia and not meeting indications for a primary prevention ICD, if the expected meaningful survival is > 1 year. C Catheter ablation: As per ESC 2022 guidelines, consider performing catheter ablation in specialized centers in patients with dilated cardiomyopathy and recurrent, symptomatic sustained monomorphic VT or ICD shocks for sustained monomorphic VT, if antiarrhythmic drugs are ineffective, contraindicated, or not tolerated. C As per AHA 2018 guidelines, consider performing catheter ablation for reducing recurrent VT and ICD shocks in patients with non-ischemic cardiomyopathy and recurrent sustained monomorphic VT failed or intolerant to antiarrhythmic medications. C 6. Specific circumstances Athletes: As per ESC 2022 guidelines, discourage participation in high-intensity exercise including competitive sports in patients with dilated cardiomyopathy and a lamin A/C mutation. B As per AHA 2015 guidelines, discourage participation in most competitive sports in symptomatic athletes with dilated cardiomyopathy, with the possible exception of low- intensity (class 1A sports) in selected cases, at least until more information is available. B Pediatric patients (evaluation): exclude underlying causes such as primary arrhythmias, cardiotoxins, congestive heart disease, or other structural defects such as anomalous left coronary artery from the pulmonary artery before making the diagnosis of idiopathic dilated cardiomyopathy in pediatric patients with cardiomyopathy phenotype. B Show 8 more Pediatric patients (management): initiate guideline-directed medical therapy for adult HF (including diuretic agents, -blockers, ACEIs, and other medications as appropriate) in pediatric patients with dilated cardiomyopathy. B Show 4 more Patients with cardiac amyloidosis (evaluation): consider performing an endomyocardial biopsy to identify amyloid protein in cardiac tissue in patients with suspected cardiac amyloidosis, especially if there is no noncardiac tissue evidence of amyloidosis. C Show 9 more Patients with cardiac amyloidosis (management): initiate warfarin therapy (with a goal INR of 2-3) or direct thrombin inhibitors in patients with cardiac amyloidosis and paroxysmal or chronic AF or a history of embolic stroke/TIA. A Show 10 more Patients with alcoholic cardiomyopathy: advise total abstinence in patients with alcoholic cardiomyopathy. B Patients with cocaine-related cardiomyopathy: consider initiating standard therapy for LV dysfunction, including -blockers, in patients with cocaine-related cardiomyopathy with demonstrated abstinence for > 6 months. C Show 2 more https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 5/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Patients with chemotherapy-related cardiomyopathy: obtain cardiac functional assessment with LVEF measurement at baseline, after completing treatment, and while on treatment at regular intervals, or sooner if HF symptoms develop in patients treated with cardiotoxic chemotherapeutic agents. B Show 6 more Patients with peripartum cardiomyopathy (evaluation): ensure timely diagnosis of peripartum cardiomyopathy to prevent delay of treatment and potential related complications. B Show 5 more Patients with peripartum cardiomyopathy (management): manage patients with peripartum cardiomyopathy by a multidisciplinary team including cardiologists, high-risk obstetricians, and perinatologists, as well as possibly cardiac anesthesiologists, cardiac intensivists, and pediatricians. B Show 13 more Patients with cardiac sarcoidosis: obtain echocardiography to assess LVEF in patients with signs and symptoms of HF. B Show 8 more Patients with acute myocarditis: obtain a 12-lead ECG, TTE, and cardiac troponins in all patients with clinically suspected myocarditis. B Show 7 more Patients with eosinophilic myocarditis: consider performing an endomyocardial biopsy in patients with suspected eosinophilic myocarditis. C Patients with HIV-associated cardiomyopathy: screen for human immunodeficiency virus as a possible cause of dilated cardiomyopathy in patients with dilated cardiomyopathy and risk factors for human immunodeficiency virus. B Show 2 more Patients with Chagas cardiomyopathy: As per ESC 2022 guidelines, consider initiating amiodarone to reduce the arrhythmia burden in patients with Chagas cardiomyopathy presenting with symptomatic premature ventricular complexes or VT. C Show 2 more As per AHA 2016 guidelines, screen for Chagas disease in patients with dilated cardiomyopathy and epidemiological risk factors for Chagas disease, such as origin from Central or South America or exposure to infected vectors. B Patients with autoimmune cardiomyopathy: consider obtaining cardiac biomarkers such as natriuretic peptides (BNP, N-terminal pro-BNP) and cardiac troponin to identify patients with the autoimmune disease with or at risk for HF. C Show 11 more Patients with obesity cardiomyopathy: obtain standard guideline-directed workup for assessment of the pathogenesis and prognosis in patients with HF and obesity. B Show 4 more Patients with growth hormone cardiomyopathy: https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 6/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Test for acromegaly or GH deficiency in patients with dilated cardiomyopathy with other signs and symptoms of those clinical disorders. B Treat the primary disorder of excess or deficiency of GH in all patients with coexisting dilated cardiomyopathy. B Patients with tachycardia-induced cardiomyopathy: ensure maintenance of sinus rhythm or control of ventricular in treating patients with tachycardia-induced cardiomyopathy. B Patients with LBBB-induced cardiomyopathy: consider offering cardiac resynchronization therapy in patients with suspected cardiomyopathy caused by LBBB. C Patients with PVC-induced cardiomyopathy (evaluation): Suspect premature ventricular complex-induced cardiomyopathy in patients with unexplained reduced ejection fraction and premature ventricular complex burden of at least 10%. B Consider obtaining cardiac MRI in patients with suspected premature ventricular complex- induced cardiomyopathy. C Patients with PVC-induced cardiomyopathy, management: As per ESC 2022 guidelines, perform catheter ablation in patients with cardiomyopathy suspected to be caused by frequent and predominately monomorphic premature ventricular complexes. B Show 3 more As per HRS/ACC/AHA 2018 guidelines, consider performing catheter ablation in patients with recurrent episodes of idiopathic VF initiated by premature ventricular complexes with a consistent QRS morphology. B Show 2 more As per AHA 2016 guidelines, consider offering radiofrequency ablation in patients with premature ventricular contraction-induced cardiomyopathy. C Patients with iron-overload cardiomyopathy: Screen for iron overload including serum ferritin and transferrin saturation when evaluating a new case of cardiomyopathy. B Obtain cardiac MRI in the setting of transferrin saturation > 45% or ferritin > 250 g/L (males) or > 200 g/L (females). B Patients with storage disease cardiomyopathy: initiate enzyme replacement therapy in patients with Pompe or Fabry lysosomal storage disorder. B Patients with thyroid disorders: Obtain thyroid function tests as part of the initial workup in all patients with dilated cardiomyopathy. B Treat hypothyroidism and hyperthyroidism. Consider initiating -blockers in patients with cardiac-related symptoms and signs of hyperthyroidism. B Patients with ischemic stroke: Initiate warfarin for at least 3 months to reduce the risk of recurrent stroke or TIA in patients with ischemic stroke or TIA and LA or LA appendage thrombus in the setting of ischemic or non-ischemic cardiomyopathy and LV dysfunction. B Individualize the decision between anticoagulation and antiplatelet therapy in patients with ischemic stroke or TIA in sinus rhythm with ischemic or non-ischemic cardiomyopathy and https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 7/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway reduced ejection fraction without evidence of LA or LV thrombus. B Clinical findings Symptoms Past medical history Abdominal discomfort AUD Dyspnea Acromegaly Exercise intolerance Amyloidosis Fatigue Becker muscular dystrophy Loss of appetite Chagas disease Nausea Congenital heart disease Orthopnea Cushing's syndrome Palpitations Dermatomyositis Syncope Diabetes mellitus Weight loss Duchenne muscular dystrophy Eosinophilic granulomatosis with polyangiitis Vital signs Exposure to chemotherapy Tachycardia Granulomatosis with polyangiitis systolic BP HF Hyperthyroidism Cardiac exam Hypothyroidism Gallop rhythm Kawasaki disease MR murmur Myocarditis Pulsus alternans Myotonic dystrophy Obesity Integument exam Polyarteritis nodosa Pallor SLE Sarcoidosis ECG findings Scleroderma Substance use disorder AV block VHD LBBB Ventricular arrhythmia Vascular exam Genetic testing Narrow pulse pressure Peripheral edema Lmna gene mutation jugular venous pressure Myh7 gene mutation Scn5a gene mutation Abdominal exam Ttn gene mutation Ascites Hepatomegaly Serological findings anti-heart antibodies https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 8/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway Imaging findings Studies 2022 PAUSE-SCD In patients with cardiomyopathy and monomorphic VT with an indication for ICD implantation, ablation therapy was superior to conventional therapy with respect to the composite outcome of VT recurrence, cardiovascular hospitalization or death. Roderick Tung et al. Circulation. 2022 Jun 21. 2022 PARTITA In patients with dilated cardiomyopathy who received implantable cardioverter defibrillator shock for VT, ablation was superior to standard therapy with respect to the composite outcome of death from any cause or hospitalization for worsening HF. Paolo Della Bella et al. Circulation. 2022 Jun 21. Show 7 more References 1. Biykem Bozkurt, Monica Colvin, Jennifer Cook et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016 Dec 6;134 23):e579-e646. Open 2. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 3. Paul A Heidenreich, Biykem Bozkurt, David Aguilar et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022 Apr 1;101161CIR0000000000001063. Open 4. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 5. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open 6. Theresa A McDonagh, Marco Metra, Marianna Adamo et al. 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. Eur Heart J. 2021 Sep 21;42 36 3599 3726. Open https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 9/10 6/29/23, 10:18 PM Dilated cardiomyopathy Pathway 7. Dawn O Kleindorfer, Amytis Towfighi, Seemant Chaturvedi et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association. Stroke. 2021 Jul;52 7):e364-e467. Open 8. Barry J Maron, James E Udelson, Robert O Bonow et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 3 Hypertrophic Cardiomyopathy, Arrhythmogenic Right Ventricular Cardiomyopathy and Other Cardiomyopathies, and Myocarditis: A Scientific Statement From the American Heart Association and American College of Cardiology. Circulation. 2015 Dec 1;132 22):e273 80. Open 9. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 10. Expert Panel on Cardiac Imaging, Prabhakar Rajiah, Jacobo Kirsch et al. ACR Appropriateness Criteria Nonischemic Myocardial Disease with Clinical Manifestations Ischemic Cardiomyopathy Already Excluded). J Am Coll Radiol. 2021 May;18 5S S83 S105. Open 11. Yigal M Pinto, Perry M Elliott, Eloisa Arbustini et al. Proposal for a revised definition of dilated cardiomyopathy, hypokinetic non-dilated cardiomyopathy, and its implications for clinical practice: a position statement of the ESC working group on myocardial and pericardial diseases. Eur Heart J. 2016 Jun 14;37 23 1850 8. Open https://web.pathway.md/diseases/rec8M1y7BZO2OaSC6 10/10