Podcast
Questions and Answers
Which of the following is a recommendation for endomyocardial biopsy in patients with cardiomyopathy?
Which of the following is a recommendation for endomyocardial biopsy in patients with cardiomyopathy?
What is the primary goal of genetic counselling and testing in cardiomyopathies?
What is the primary goal of genetic counselling and testing in cardiomyopathies?
Which of the following is a recommendation for cardiac transplantation in patients with cardiomyopathy?
Which of the following is a recommendation for cardiac transplantation in patients with cardiomyopathy?
What is the primary indication for left ventricular assist device therapy in patients with cardiomyopathy?
What is the primary indication for left ventricular assist device therapy in patients with cardiomyopathy?
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Which of the following is a recommendation for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy?
Which of the following is a recommendation for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy?
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What is the primary purpose of exercise recommendations in hypertrophic cardiomyopathy?
What is the primary purpose of exercise recommendations in hypertrophic cardiomyopathy?
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What is the minimum percentage of agreement required among voting members for a recommendation to be approved by the Task Force?
What is the minimum percentage of agreement required among voting members for a recommendation to be approved by the Task Force?
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Which of the following is a recommendation for exercise in patients with arrhythmogenic right ventricular cardiomyopathy?
Which of the following is a recommendation for exercise in patients with arrhythmogenic right ventricular cardiomyopathy?
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What is the primary purpose of exercise recommendations in dilated cardiomyopathy?
What is the primary purpose of exercise recommendations in dilated cardiomyopathy?
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What is the primary purpose of the declaration of interest forms provided by the experts of the writing and reviewing panels?
What is the primary purpose of the declaration of interest forms provided by the experts of the writing and reviewing panels?
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What is the term used to describe a recommendation that is supported by evidence and/or general agreement?
What is the term used to describe a recommendation that is supported by evidence and/or general agreement?
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What is the phrase used to describe a Class I recommendation in the guidelines?
What is the phrase used to describe a Class I recommendation in the guidelines?
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Where can the declarations of interest of the experts of the writing and reviewing panels be found?
Where can the declarations of interest of the experts of the writing and reviewing panels be found?
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What is the purpose of the ESC declaration of interest rules?
What is the purpose of the ESC declaration of interest rules?
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What is the primary purpose of the Task Force voting procedure?
What is the primary purpose of the Task Force voting procedure?
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What is the term used to describe the classification system used to categorize recommendations in the guidelines?
What is the term used to describe the classification system used to categorize recommendations in the guidelines?
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What is Recommendation Table 23 focused on?
What is Recommendation Table 23 focused on?
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Which recommendation table discusses implantable cardioverter defibrillators in dilated cardiomyopathy?
Which recommendation table discusses implantable cardioverter defibrillators in dilated cardiomyopathy?
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What is the focus of Recommendation Table 25?
What is the focus of Recommendation Table 25?
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Which table discusses key discussion points of pre- and post-test genetic counselling?
Which table discusses key discussion points of pre- and post-test genetic counselling?
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What is the topic of Table 14?
What is the topic of Table 14?
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Recommendation Table 23 is related to which type of cardiomyopathy?
Recommendation Table 23 is related to which type of cardiomyopathy?
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What is the topic of Table 15?
What is the topic of Table 15?
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Which table discusses psychological considerations in cardiomyopathy patients?
Which table discusses psychological considerations in cardiomyopathy patients?
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Which of the following is recommended for patients with suspected ATTR-related cardiac amyloidosis?
Which of the following is recommended for patients with suspected ATTR-related cardiac amyloidosis?
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Who should provide genetic counseling in cardiomyopathy patients?
Who should provide genetic counseling in cardiomyopathy patients?
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In which scenario is genetic testing recommended for cardiomyopathy?
In which scenario is genetic testing recommended for cardiomyopathy?
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What is recommended for adult at-risk relatives of a patient with cardiomyopathy?
What is recommended for adult at-risk relatives of a patient with cardiomyopathy?
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In which situation is orthotopic cardiac transplantation recommended?
In which situation is orthotopic cardiac transplantation recommended?
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What is recommended for patients with HCM or cardiac amyloidosis and AF or atrial flutter?
What is recommended for patients with HCM or cardiac amyloidosis and AF or atrial flutter?
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In which scenario is atrial fibrillation catheter ablation recommended?
In which scenario is atrial fibrillation catheter ablation recommended?
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What is recommended for a deceased individual identified to have cardiomyopathy at post-mortem?
What is recommended for a deceased individual identified to have cardiomyopathy at post-mortem?
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What is recommended for a patient with cardiomyopathy who has a family history of cardiomyopathy?
What is recommended for a patient with cardiomyopathy who has a family history of cardiomyopathy?
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What is recommended for pre-natal diagnostic testing?
What is recommended for pre-natal diagnostic testing?
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What is recommended for patients who have an expectation of good quality survival >1 year?
What is recommended for patients who have an expectation of good quality survival >1 year?
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What should be considered when guiding ICD implantation?
What should be considered when guiding ICD implantation?
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Who should be counselled on the risk of inappropriate shocks, implant complications?
Who should be counselled on the risk of inappropriate shocks, implant complications?
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In which patients is ICD implantation recommended?
In which patients is ICD implantation recommended?
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How often should comprehensive SCD risk stratification be performed in cardiomyopathy patients?
How often should comprehensive SCD risk stratification be performed in cardiomyopathy patients?
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What is recommended when an ICD is indicated?
What is recommended when an ICD is indicated?
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How often should clinically stable patients with cardiomyopathy undergo routine follow-up?
How often should clinically stable patients with cardiomyopathy undergo routine follow-up?
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What is recommended for first-degree relatives who have the same disease-causing variant as the proband?
What is recommended for first-degree relatives who have the same disease-causing variant as the proband?
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What is recommended for first-degree relatives without a phenotype who do not have the same disease-causing variant as the proband?
What is recommended for first-degree relatives without a phenotype who do not have the same disease-causing variant as the proband?
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What is recommended when no P/LP variant is identified in the proband or genetic testing is not performed?
What is recommended when no P/LP variant is identified in the proband or genetic testing is not performed?
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What is the recommended approach for evaluating patients with suspected or established cardiomyopathy?
What is the recommended approach for evaluating patients with suspected or established cardiomyopathy?
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Why is it recommended to evaluate family history in patients with suspected cardiomyopathy?
Why is it recommended to evaluate family history in patients with suspected cardiomyopathy?
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What is the purpose of routine laboratory tests in patients with suspected or confirmed cardiomyopathy?
What is the purpose of routine laboratory tests in patients with suspected or confirmed cardiomyopathy?
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What is recommended for echocardiographic evaluation in patients with cardiomyopathy?
What is recommended for echocardiographic evaluation in patients with cardiomyopathy?
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In which patients with cardiomyopathy is contrast-enhanced cardiac magnetic resonance (CMR) recommended?
In which patients with cardiomyopathy is contrast-enhanced cardiac magnetic resonance (CMR) recommended?
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Why is it recommended that all patients with cardiomyopathy have access to multidisciplinary teams with expertise in cardiomyopathies?
Why is it recommended that all patients with cardiomyopathy have access to multidisciplinary teams with expertise in cardiomyopathies?
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What is the recommended offer to individuals who have experienced the premature sudden cardiac death of a family member with cardiomyopathy?
What is the recommended offer to individuals who have experienced the premature sudden cardiac death of a family member with cardiomyopathy?
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In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended at initial evaluation?
In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended at initial evaluation?
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What is recommended for adolescents with cardiomyopathy?
What is recommended for adolescents with cardiomyopathy?
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Who should be offered psychological support by an appropriately trained health professional?
Who should be offered psychological support by an appropriately trained health professional?
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What is the purpose of pedigree analysis in patients with suspected cardiomyopathy?
What is the purpose of pedigree analysis in patients with suspected cardiomyopathy?
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What is the purpose of transthoracic 2D and Doppler echocardiography at initial evaluation in patients with HCM?
What is the purpose of transthoracic 2D and Doppler echocardiography at initial evaluation in patients with HCM?
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In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended during Valsalva manoeuvre?
In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended during Valsalva manoeuvre?
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Study Notes
Abbreviations and Definitions
- RWMA stands for Regional wall motion abnormality
- SAECG stands for Signal-averaged electrocardiogram
- SAM stands for Systolic anterior motion
- SCD stands for Sudden cardiac death
- SGLT2i stands for Sodium–glucose co-transporter 2 inhibitor
- SMVT stands for Sustained monomorphic ventricular tachycardia
- SPECT stands for Single-photon emission computed tomography
- SRT stands for Septal reduction therapy
- TIA stands for Transient ischaemic attack
- TMEM43 stands for transmembrane protein 43
Classes of Recommendations
- Class I recommendations are based on evidence and/or general agreement that a given treatment or procedure is beneficial, useful, or effective
- Recommendations are defined according to their classes, with wording to use for each class
Recommendations for Cardiomyopathy
- Recommendation Table 7: Recommendations for endomyocardial biopsy in patients with cardiomyopathy
- Recommendation Table 8: Recommendations for genetic counselling and testing in cardiomyopathies
- Recommendation Table 9: Recommendations for cardiac transplantation in patients with cardiomyopathy
- Recommendation Table 10: Recommendation for left ventricular assist device therapy in patients with cardiomyopathy
- Recommendation Table 11: Recommendations for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy
Exercise and Cardiomyopathy
- Cardiovascular benefits of exercise in patients with cardiomyopathy
- Exercise-related sudden cardiac death and historical exercise recommendations for patients with cardiomyopathy
- Exercise recommendations in hypertrophic cardiomyopathy
- Exercise recommendations in arrhythmogenic right ventricular cardiomyopathy
- Exercise recommendations in dilated cardiomyopathy
Prevention of Sudden Cardiac Death
- Recommendation Table 23: Additional recommendations for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy
- Recommendation Table 24: Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy
- Recommendation Table 25: Recommendation for resting and ambulatory electrocardiogram monitoring in patients with non-dilated left ventricular cardiomyopathy
Multidisciplinary Care for Cardiomyopathy Patients
- All patients with cardiomyopathy and their relatives should have access to multidisciplinary teams with expertise in diagnosis and management of cardiomyopathies.
Transition of Care from Paediatric to Adult Services
- Timely and adequate preparation for transition of care from paediatric to adult services, including joint consultations, is recommended for all adolescents with cardiomyopathy.
Diagnostic Work-up in Cardiomyopathies
- A systematic evaluation using a multiparametric approach that includes clinical evaluation, pedigree analysis, ECG, Holter monitoring, laboratory tests, and multimodality imaging is recommended for all patients with suspected or established cardiomyopathy.
- Evaluation of family history and creation of a three- to four-generation family tree is recommended to aid in diagnosis, provide clues to underlying aetiology, determine inheritance pattern, and identify at-risk relatives.
Laboratory Tests in the Diagnosis of Cardiomyopathies
- Routine laboratory tests are recommended in all patients with suspected or confirmed cardiomyopathy to evaluate aetiology, assess disease severity, and aid in detection of extracardiac manifestations and assessment of secondary organ dysfunction.
Echocardiographic Evaluation in Patients with Cardiomyopathy
- A comprehensive evaluation of cardiac dimensions and LV and RV systolic (global and regional) and LV diastolic function is recommended in all patients with cardiomyopathy at initial evaluation and during follow-up to monitor disease progression and aid risk stratification and management.
Cardiac Magnetic Resonance Indication in Patients with Cardiomyopathy
- Contrast-enhanced CMR is recommended in patients with cardiomyopathy at initial evaluation.
Genetic Counselling and Testing in Cardiomyopathies
- Genetic counselling, provided by an appropriately trained healthcare professional, is recommended for families with an inherited or suspected inherited cardiomyopathy, regardless of whether genetic testing is being considered.
- Genetic testing for cardiomyopathy should be performed with access to a multidisciplinary team, including those with expertise in genetic testing methodology, sequence variant interpretation, and clinical application of genetic testing, typically in a specialized cardiomyopathy service or in a network model with access to equivalent expertise.
- Pre- and post-test genetic counselling is recommended in all individuals undergoing genetic testing for cardiomyopathy.
Index Patients
- Genetic testing is recommended in patients fulfilling diagnostic criteria for cardiomyopathy in cases where it enables diagnosis, prognostication, therapeutic stratification, or reproductive management of the patient, or where it enables cascade genetic evaluation of their relatives who would otherwise be enrolled into long-term surveillance.
Family Members
- Cascade genetic testing, with pre- and post-test counselling, is recommended in adult at-risk relatives if a confident genetic diagnosis (i.e. a P/LP variant) has been established in an individual with cardiomyopathy in the family.
Cardiac Transplantation in Patients with Cardiomyopathy
- Orthotopic cardiac transplantation is recommended for eligible cardiomyopathy patients with advanced heart failure (NYHA class III–IV) or intractable ventricular arrhythmia refractory to medical/invasive/device therapy, and who do not have absolute contraindications.
Management of Atrial Fibrillation and Atrial Flutter in Patients with Cardiomyopathy
- Oral anticoagulation is recommended in all patients with HCM or cardiac amyloidosis and AF or atrial flutter (unless contraindicated).
- Atrial fibrillation catheter ablation is recommended for rhythm control after one failed or intolerant class I or III AAD to improve symptoms of AF recurrences in patients with paroxysmal or persistent AF and cardiomyopathy.
Implantable Cardioverter Defibrillator in Patients with Cardiomyopathy
- Implantation of a cardioverter defibrillator is recommended in patients who have an expectation of good quality survival >1 year.
- It is recommended that ICD implantation be guided by shared decision-making that is evidence-based, considers a person’s individual preferences, beliefs, circumstances, and values, and ensures that the person understands the benefits, harm, and possible consequences of different treatment options.
Routine Follow-up of Patients with Cardiomyopathy
- All clinically stable patients with cardiomyopathy should undergo routine follow-up using a multiparametric approach that includes ECG and echocardiography every 1–2 years.
- Clinical evaluation with ECG and multimodality imaging is recommended in patients with cardiomyopathy whenever there is a substantial or unexpected change in symptoms.
Family Screening and Follow-up Evaluation of Relatives
- Following cascade genetic testing, clinical evaluation using a multiparametric approach that includes ECG and cardiac imaging and long-term follow-up is recommended in first-degree relatives who have the same disease-causing variant as the proband.
Psychological Support in Patients and Family Members with Cardiomyopathies
- Psychological support by an appropriately trained health professional should be offered to all individuals who have experienced the premature sudden cardiac death of a family member with cardiomyopathy.
- Psychological support by an appropriately trained health professional should be offered to all individuals with an inherited cardiomyopathy who receive an implantable cardioverter defibrillator.
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Description
Learn the meanings of medical abbreviations and definitions, including RWMA, SAECG, SAM, and more. Test your knowledge of medical terminology.
