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Dilated cardiomyopathy

Key sources
The following summarized guidelines for the evaluation and management of dilated cardiomyopathy are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2023; 2022; 2021), the Heart Failure Society of America (HFSA/AHA/ACC 2022), 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).
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Guidelines

1.Screening and diagnosis

Screening of family relatives: as per ESC 2023 guidelines, obtain cascade genetic testing, with pre- and post-test counseling, in adult at-risk relatives of a patient with cardiomyopathy with a confident genetic diagnosis (a pathogenic/likely pathogenic variant) in the family (starting with first-degree relatives, if available, and cascading out sequentially).
B
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2.Diagnostic investigations

General principles: obtain systematic evaluation in all patients with suspected or established cardiomyopathy using a multiparametric approach, including clinical evaluation, pedigree analysis, ECG, Holter monitoring, laboratory tests, and multimodality imaging.
B

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  • History and physical examination

  • TTE

  • Cardiac MRI

  • Cardiac CT

  • Nuclear imaging

  • Laboratory tests

  • Genetic testing

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

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  • Endomyocardial biopsy

4.Medical management

General principles
Ensure that all patients with cardiomyopathy and their relatives have access to multidisciplinary teams with expertise in the diagnosis and management of cardiomyopathies.
B
Identify and manage risk factors and concomitant diseases as an integral part of the management of patients with cardiomyopathy.
B

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  • Antiarrhythmic agents

  • Management of AF (antithrombotic therapy)

  • Management of AF (catheter ablation)

5.Nonpharmacologic interventions

Physical activity restrictions: advise practicing regular low-to-moderate-intensity exercise in all able patients with cardiomyopathy.
B
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  • Psychological support

6.Therapeutic procedures

Implantable cardioverter-defibrillator, general principles: perform ICD placement only in patients with an expected good quality survival > 1 year.
B
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  • ICD (primary prevention of SCD)

  • ICD (secondary prevention of SCD)

  • ICD (technical considerations)

  • Catheter ablation (ventricular arrhythmia)

  • Catheter ablation (AF)

7.Perioperative care

Perioperative assessment: obtain perioperative ECG monitoring in all patients with cardiomyopathy undergoing noncardiac surgery.
B
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8.Surgical interventions

Left ventricular assist device
Consider initiating mechanical circulatory support therapy in selected patients with cardiomyopathy with advanced HF (NYHA class III-IV) despite optimal pharmacological and device treatment, otherwise suitable for heart transplantation, to improve symptoms and reduce the risk of HF hospitalization from worsening HF and premature death while awaiting a transplant.
C
Consider initiating mechanical circulatory support therapy in selected patients with cardiomyopathy with advanced HF (NYHA class III-IV) despite optimal pharmacological and device therapy, ineligible for cardiac transplantation or other surgical options, and without severe RV dysfunction, to reduce the risk of death and improve symptoms.
C

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  • Heart transplantation

9.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

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  • Pediatric patients (evaluation)

  • Pediatric patients (management)

  • Pregnant patients

  • Patients with non-dilated LV cardiomyopathy (ICD implantation, primary prevention of SCD)

  • Patients with non-dilated LV cardiomyopathy (ICD implantation, secondary prevention of SCD)

  • Patients with cardiac amyloidosis (evaluation)

  • Patients with cardiac amyloidosis (management)

  • Patients with alcoholic cardiomyopathy

  • Patients with cocaine-related cardiomyopathy

  • Patients with chemotherapy-related cardiomyopathy

  • Patients with peripartum cardiomyopathy (evaluation)

  • Patients with peripartum cardiomyopathy (management)

  • Patients with cardiac sarcoidosis

  • Patients with acute myocarditis

  • Patients with eosinophilic myocarditis

  • Patients with HIV-associated cardiomyopathy

  • Patients with Chagas cardiomyopathy

  • Patients with autoimmune cardiomyopathy

  • Patients with obesity cardiomyopathy

  • Patients with GH cardiomyopathy

  • Patients with tachycardia-induced cardiomyopathy

  • Patients with LBBB-induced cardiomyopathy

  • Patients with PVC-induced cardiomyopathy (evaluation)

  • Patients with PVC-induced cardiomyopathy (management)

  • Patients with iron-overload cardiomyopathy

  • Patients with storage disease cardiomyopathy

  • Patients with thyroid disorders

  • Patients with ischemic stroke

10.Patient education

Genetic counseling
Provide genetic counseling by an appropriately trained healthcare professional, including genetic education, to inform decision-making and psychosocial support in families with an inherited or suspected inherited cardiomyopathy, regardless of whether genetic testing is being considered.
B
Provide pre- and post-test genetic counseling in all individuals undergoing genetic testing for cardiomyopathy.
B

11.Follow-up and surveillance

Follow-up: obtain routine follow-up using a multiparametric approach with ECG and echocardiography every 1-2 years in all clinically stable patients with cardiomyopathy.
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  • Transition to adult care