57 Questions
Which of the following is a recommendation for endomyocardial biopsy in patients with cardiomyopathy?
It is recommended in patients with cardiomyopathy and suspected myocarditis
What is the primary goal of genetic counselling and testing in cardiomyopathies?
To identify the underlying genetic mutation
Which of the following is a recommendation for cardiac transplantation in patients with cardiomyopathy?
It is recommended in patients with cardiomyopathy and severe heart failure
What is the primary indication for left ventricular assist device therapy in patients with cardiomyopathy?
To bridge patients to cardiac transplantation
Which of the following is a recommendation for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy?
Rate control is the primary goal of treatment
What is the primary purpose of exercise recommendations in hypertrophic cardiomyopathy?
To reduce the risk of sudden cardiac death
What is the minimum percentage of agreement required among voting members for a recommendation to be approved by the Task Force?
75%
Which of the following is a recommendation for exercise in patients with arrhythmogenic right ventricular cardiomyopathy?
Low-intensity exercise is recommended
What is the primary purpose of exercise recommendations in dilated cardiomyopathy?
To manage symptoms of heart failure
What is the primary purpose of the declaration of interest forms provided by the experts of the writing and reviewing panels?
To provide a list of potential sources of conflicts of interest
What is the term used to describe a recommendation that is supported by evidence and/or general agreement?
Class I
What is the phrase used to describe a Class I recommendation in the guidelines?
Is recommended or is indicated
Where can the declarations of interest of the experts of the writing and reviewing panels be found?
On the ESC website
What is the purpose of the ESC declaration of interest rules?
To review and disclose potential sources of conflicts of interest
What is the primary purpose of the Task Force voting procedure?
To achieve a minimum of 75% agreement among voting members for a recommendation to be approved
What is the term used to describe the classification system used to categorize recommendations in the guidelines?
Classes of recommendations
What is Recommendation Table 23 focused on?
Prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy
Which recommendation table discusses implantable cardioverter defibrillators in dilated cardiomyopathy?
Recommendation Table 24
What is the focus of Recommendation Table 25?
Resting and ambulatory electrocardiogram monitoring in patients with non-dilated left ventricular cardiomyopathy
Which table discusses key discussion points of pre- and post-test genetic counselling?
Table 13
What is the topic of Table 14?
Pre-natal and pre-implantation options and implications
Recommendation Table 23 is related to which type of cardiomyopathy?
Hypertrophic cardiomyopathy
What is the topic of Table 15?
Atrial fibrillation burden and management in cardiomyopathies
Which table discusses psychological considerations in cardiomyopathy patients?
Table 16
Which of the following is recommended for patients with suspected ATTR-related cardiac amyloidosis?
DPD/PYP/HMDP bone-tracer scintigraphy
Who should provide genetic counseling in cardiomyopathy patients?
An appropriately trained healthcare professional
In which scenario is genetic testing recommended for cardiomyopathy?
In patients fulfilling diagnostic criteria for cardiomyopathy
What is recommended for adult at-risk relatives of a patient with cardiomyopathy?
Cascade genetic testing with pre- and post-test counseling
In which situation is orthotopic cardiac transplantation recommended?
In patients with advanced heart failure (NYHA class III-IV)
What is recommended for patients with HCM or cardiac amyloidosis and AF or atrial flutter?
Oral anticoagulation
In which scenario is atrial fibrillation catheter ablation recommended?
For rhythm control after one failed or intolerant class I or III AAD
What is recommended for a deceased individual identified to have cardiomyopathy at post-mortem?
Genetic testing if a genetic diagnosis would facilitate management of surviving relatives
What is recommended for a patient with cardiomyopathy who has a family history of cardiomyopathy?
Genetic testing
What is recommended for pre-natal diagnostic testing?
It should be performed early in pregnancy
What is recommended for patients who have an expectation of good quality survival >1 year?
Implantation of a cardioverter defibrillator
What should be considered when guiding ICD implantation?
Evidence, individual preferences, beliefs, circumstances, and values
Who should be counselled on the risk of inappropriate shocks, implant complications?
All patients prior to ICD implantation
In which patients is ICD implantation recommended?
Patients with HCM, DCM, and ARVC who have survived a cardiac arrest
How often should comprehensive SCD risk stratification be performed in cardiomyopathy patients?
At initial evaluation and at 1–2 year intervals
What is recommended when an ICD is indicated?
Evaluation whether the patient could benefit from CRT
How often should clinically stable patients with cardiomyopathy undergo routine follow-up?
Every 1–2 years
What is recommended for first-degree relatives who have the same disease-causing variant as the proband?
Clinical evaluation using a multiparametric approach
What is recommended for first-degree relatives without a phenotype who do not have the same disease-causing variant as the proband?
Discharge from further follow-up and advised to seek re-assessment if symptoms develop
What is recommended when no P/LP variant is identified in the proband or genetic testing is not performed?
Clinical evaluation using a multiparametric approach
What is the recommended approach for evaluating patients with suspected or established cardiomyopathy?
Multiparametric approach that includes clinical evaluation, pedigree analysis, ECG, Holter monitoring, laboratory tests, and multimodality imaging
Why is it recommended to evaluate family history in patients with suspected cardiomyopathy?
To aid in diagnosis, provide clues to underlying aetiology, determine inheritance pattern, and identify at-risk relatives
What is the purpose of routine laboratory tests in 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
What is recommended for echocardiographic evaluation in patients with cardiomyopathy?
Comprehensive evaluation of cardiac dimensions and LV and RV systolic (global and regional) and LV diastolic function
In which patients with cardiomyopathy is contrast-enhanced cardiac magnetic resonance (CMR) recommended?
Patients with cardiomyopathy at initial evaluation
Why is it recommended that all patients with cardiomyopathy have access to multidisciplinary teams with expertise in cardiomyopathies?
To aid in diagnosis and management of cardiomyopathies
What is the recommended offer to individuals who have experienced the premature sudden cardiac death of a family member with cardiomyopathy?
Psychological support by an appropriately trained health professional
In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended at initial evaluation?
All patients with HCM, at rest and during Valsalva manoeuvre
What is recommended for adolescents with cardiomyopathy?
Timely and adequate preparation for transition of care from paediatric to adult services
Who should be offered psychological support by an appropriately trained health professional?
All individuals with an inherited cardiomyopathy who receive an implantable cardioverter defibrillator
What is the purpose of pedigree analysis in patients with suspected cardiomyopathy?
To aid in diagnosis, provide clues to underlying aetiology, determine inheritance pattern, and identify at-risk relatives
What is the purpose of transthoracic 2D and Doppler echocardiography at initial evaluation in patients with HCM?
To detect left ventricular outflow tract obstruction
In which patients with HCM is transthoracic 2D and Doppler echocardiography recommended during Valsalva manoeuvre?
All patients with HCM, in the sitting and semi-supine positions
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.
Learn the meanings of medical abbreviations and definitions, including RWMA, SAECG, SAM, and more. Test your knowledge of medical terminology.
Make Your Own Quizzes and Flashcards
Convert your notes into interactive study material.
Get started for free