ESC 2023 2023 ESC Guidelines for the management of cardiomyopathies
European Society of Cardiology｜循環器 / 心不全・心筋疾患｜124 ページ｜チャンク 1286｜推奨 171 件｜current
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| 頁 | クラス | レベル | 推奨文 | 表 |
|---|---|---|---|---|
| p.17 | I | C | It is recommended that all patients with cardiomyopathy and their relatives have access to multidisciplinary teams with expertise in the diagnosis and management of cardiomyopathies. | Recommendation Table 1 — Recommendations for the provision of service of multidisciplinary cardiomyopathy teams |
| p.17 | I | C | Timely and adequate preparation for transition of care from paediatric to adult services, including joint consultations, is recommended in all adolescents with cardiomyopathy.58,59 aClass of recommendation. bLevel of evidence. | Recommendation Table 1 — Recommendations for the provision of service of multidisciplinary cardiomyopathy teams |
| p.18 | I | C | It is recommended that all patients with suspected or established cardiomyopathy undergo systematic evaluation using a multiparametric approach that includes clinical evaluation, pedigree analysis, ECG, Holter monitoring, laboratory tests, and multimodality imaging.63 | Recommendation Table 2 — Recommendations for diagnostic work-up in cardiomyopathies |
| p.18 | I | C | It is recommended that all patients with suspected cardiomyopathy undergo evaluation of family history and that a three- to four-generation family tree is created to aid in diagnosis, provide clues to underlying aetiology, determine inheritance pattern, and identify at-risk relatives.64–66 ECG, electrocardiogram. aClass of recommendation. bLevel of evidence. | Recommendation Table 2 — Recommendations for diagnostic work-up in cardiomyopathies |
| p.22 | I | C | Routine (first-level) laboratory testsc 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 | Recommendation Table 3 — Recommendations for laboratory tests in the diagnosis of cardiomyopathies |
| p.22 | IIa | C | secondary organ dysfunction. Additional (second-level) testsc should be considered in patients with cardiomyopathy and extracardiac features to aid in detection of metabolic and syndromic causes, following specialist evaluation. aClass of recommendation. bLevel of evidence. cSee Table 8. | Recommendation Table 3 — Recommendations for laboratory tests in the diagnosis of cardiomyopathies |
| p.22 | I | B | 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.78,83–102 LV, left ventricular; RV, right ventricular. aClass of recommendation. bLevel of evidence. | Recommendation Table 4 — Recommendation for echocardiographic evaluation in patients with cardiomyopathy |
| p.24 | I | B | Contrast-enhanced CMR is recommended in patients with cardiomyopathy at initial evaluation.10,90,116,119–143 | Recommendation Table 5 — Recommendations for cardiac magnetic resonance indication in patients with cardiomyopathy |
| p.24 | IIa | C | Amyloidosis Bone scintigraphy Myocardial inflammation PET-CT Contrast-enhanced CMR should be considered in patients with cardiomyopathy during follow-up to monitor disease progression and aid risk stratification and management.89,90,120–122,127,129,136–147 | |
| p.24 | IIa | C | Contrast-enhanced CMR should be considered for the serial follow-up and assessment of therapeutic response in patients with cardiac amyloidosis, Anderson–Fabry disease, sarcoidosis, inflammatory cardiomyopathies, and haemochromatosis with cardiac involvement.148–152 | |
| p.24 | IIa | B | In families with cardiomyopathy in which a disease-causing variant has been identified, contrast-enhanced CMR should be considered in genotype-positive/phenotype-negative family members to aid diagnosis and detect early disease.10,122,126,128,129,135–143,145,153–159 | |
| p.24 | IIb | C | In cases of familial cardiomyopathy without a genetic diagnosis, contrast-enhanced CMR may be considered in phenotype-negative family members to aid diagnosis and detect early disease.10,128 CMR, cardiac magnetic resonance. aClass of recommendation. bLevel of evidence. Continued | Recommendation Table 5 — Recommendations for cardiac magnetic resonance indication in patients with cardiomyopathy |
| p.26 | I | B | DPD/PYP/HMDP bone-tracer scintigraphy is recommended in patients with suspected ATTR-related cardiac amyloidosis to aid diagnosis.166–168 | Recommendation Table 6 — Recommendations for computed tomography and nuclear imaging |
| p.26 | IIa | C | Contrast-enhanced cardiac CT should be considered in patients with suspected cardiomyopathy who have inadequate echocardiographic imaging and contraindications to CMR.169,170 | Recommendation Table 6 — Recommendations for computed tomography and nuclear imaging |
| p.26 | IIa | C | In patients with suspected cardiomyopathy, CT-based imaging should be considered to exclude congenital or acquired coronary artery disease as a cause of the observed myocardial abnormality.171 | Recommendation Table 6 — Recommendations for computed tomography and nuclear imaging |
| p.26 | IIa | C | 18F-FDG-PET scanning should be considered for the diagnostic work-up in patients with cardiomyopathy in whom cardiac sarcoidosis is suspected.164,172,173 © ESC 2023 | Recommendation Table 6 — Recommendations for computed tomography and nuclear imaging |
| p.26 | IIa | C | In patients with suspected cardiomyopathy, EMB should be considered to aid in diagnosis and management when the results of other clinical investigations suggest myocardial inflammation, infiltration, or storage that cannot be identified by other means.174–177 EMB, endomyocardial biopsy. aClass of recommendation. bLevel of evidence. | Recommendation Table 7 — Recommendation for endomyocardial biopsy in patients with cardiomyopathy |
| p.35 | I | B | Genetic counselling Genetic counselling, provided by an appropriately trained healthcare professional and including genetic education to inform decision-making and psychosocial support, is recommended for families with an inherited or suspected inherited cardiomyopathy, regardless of whether genetic testing is being considered.204,206,208,209,221–224 | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | B | It is recommended that genetic testing for cardiomyopathy is 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 | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | B | service or in a network model with access to equivalent expertise.222,224–226 Pre- and post-test genetic counselling is recommended in all individuals undergoing genetic testing for cardiomyopathy.204,208,227–236 | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | C | If pre-natal diagnostic testing is to be pursued by the family, it is recommended that this is performed early in pregnancy, to allow decisions regarding continuation or co-ordination of pregnancy to be made. | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | IIa | C | A discussion about reproductive genetic testing options with an appropriately trained healthcare professional should be considered for all families with a genetic diagnosis. Index patients | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | B | 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 | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | C | enrolled into long-term surveillance.227–231,237,238 Genetic testing is recommended for a deceased individual identified to have cardiomyopathy at post-mortem if a genetic diagnosis would facilitate management of surviving relatives.239–243 Genetic testing may be considered in patients | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | IIb | C | fulfilling diagnostic criteria for cardiomyopathy when it will have a net benefit to the patient, considering the psychological impact and preference, even if it does not enable diagnosis, prognostication, or therapeutic stratification, or cascade genetic screening of their relatives. | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | IIb | C | Genetic testing in patients with a borderline phenotype not fulfilling diagnostic criteria for a cardiomyopathy may be considered only after detailed assessment by specialist teams. | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | I | B | It is recommended that cascade genetic testing, with pre- and post-test counselling, is offered to 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 (starting with first-degree relatives if available, and cascading out sequentially).204,227–232 Cascade genetic testing with pre- and post-test | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | IIa | B | counselling should be considered in paediatric 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 (starting with first-degree relatives, if available, and cascading out sequentially), considering the underlying cardiomyopathy, expected age of onset, presentation in the family, and clinical/legal | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | IIa | C | consequences.233–236,244 Testing for the presence of a familial variant of unknown significance, typically in parents and/or affected relatives, to determine if the variant segregates with the cardiomyopathy phenotype should be considered if this might allow the variant to | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.35 | III | C | be interpreted with confidence. Diagnostic genetic testing is not recommended in a phenotype-negative relative of a patient with cardiomyopathy in the absence of a confident genetic diagnosis (i.e. a P/LP variant) in the family. P/LP, pathogenic/likely pathogenic. aClass of recommendation. bLevel of evidence. | Recommendation Table 8 — Recommendations for genetic counselling and testing in cardiomyopathies |
| p.38 | I | C | 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.317–319 NYHA, New York Heart Association. aClass of recommendation. bLevel of evidence. | Recommendation Table 9 — Recommendations for cardiac transplantation in patients with cardiomyopathy |
| p.38 | IIa | B | Mechanical circulatory support therapy should be considered in selected cardiomyopathy patients with advanced heart failure (NYHA class III–IV) despite optimal pharmacological and device treatment, who are otherwise suitable for heart transplantation, to improve symptoms and reduce the risk of heart failure hospitalization from worsening heart failure and premature death while awaiting a transplant.320–324 | Recommendation Table 10 — Recommendation for left ventricular assist device therapy in patients with cardiomyopathy |
| p.41 | I | B | Anticoagulation Oral anticoagulation in order to reduce the risk of stroke and thrombo-embolic events is recommended in all patients with HCM or cardiac amyloidosis and AF or atrial flutter (unless contraindicated).332,365,369,371,373,378,413,427,428,456–464 | Recommendation Table 11 — Recommendations for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy |
| p.41 | I | B | Oral anticoagulation to reduce the risk of stroke and thrombo-embolic events is recommended in patients with DCM, NDLVC, or ARVC, and AF or atrial flutter with a CHA2DS2-VASc score ≥2 in men or ≥3 in women.465–469 | Recommendation Table 11 — Recommendations for management of atrial fibrillation and atrial flutter in patients with cardiomyopathy |
| p.42 | IIa | C | Oral anticoagulation to reduce the risk of stroke and thrombo-embolic events should be considered in patients with RCM and AF or atrial flutter (unless contraindicated). | |
| p.42 | IIa | B | Oral anticoagulation to reduce the risk of stroke and thrombo-embolic events should be considered in patients with DCM, NDLVC, or ARVC, and AF or atrial flutter with a CHA2DS2-VASc score of 1 in men or of 2 in women.470–472 Control of symptoms and heart failure | |
| p.42 | I | B | 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.335,397–399,412,415–420,430– 435,447,451,473–498 | |
| p.42 | I | B | Atrial fibrillation catheter ablation is recommended to reverse LV dysfunction in AF patients with cardiomyopathy when tachycardia-induced component is highly probable, independent of their symptom status.405,407,408,499–501 | |
| p.42 | IIa | C | Maintenance of sinus rhythm rather than rate control should be considered at an early stage for patients with a cardiomyopathy and AF without major risk factors for recurrence, regardless of symptoms.402 Atrial fibrillation catheter ablation should be | |
| p.42 | IIa | C | considered as first-line rhythm control therapy to improve symptoms in selected patients with cardiomyopathy and paroxysmal or persistent AF without major risk factors for recurrences as an alternative to class I or III AADs, considering patient choice, benefit, and risk.392,393,480,502–506 | |
| p.42 | IIa | B | Atrial fibrillation catheter ablation should be considered in selected patients with cardiomyopathy, AF, and heart failure and/or reduced LVEF to prevent AF recurrences and improve QoL, LVEF, and survival and reduce heart failure hospitalization.399–401,403– 408,499–501,507 | |
| p.42 | I | B | Modification of unhealthy lifestyle and targeted therapy of intercurrent conditions is recommended to reduce AF burden and symptom severity in patients with cardiomyopathy.347,508–513 | |
| p.43 | I | B | • is recommended in patients with HCM.81,525,535 | |
| p.43 | IIa | B | • should be considered in patients with DCM, NDLVC, and ARVC.185,186,524,526,536–542 | |
| p.43 | I | C | General recommendations Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good quality survival >1 year. It is recommended that ICD implantation be guided | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | I | C | 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, harms, and possible consequences of different | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | I | C | treatment options.c It is recommended that prior to ICD implantation, patients are counselled on the risk of inappropriate shocks, implant complications, and the social, occupational, and driving implications of the device. | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | III | C | It is not recommended to implant an ICD in patients with incessant ventricular arrhythmias until the ventricular arrhythmia is controlled. | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | I | B | Secondary prevention Implantation of an ICD is recommended:d • in patients with HCM, DCM, and ARVC who have survived a cardiac arrest due to VT or VF, or who have spontaneous sustained ventricular arrhythmia causing syncope or haemodynamic compromise in the absence of reversible causes.528–534 | |
| p.43 | I | C | • in patients with NDLVC and RCM who have survived a cardiac arrest due to VT or VF, or who have spontaneous sustained ventricular arrhythmia causing syncope or haemodynamic compromise in | |
| p.43 | IIa | C | the absence of reversible causes. ICD implantation should be considered in patients with cardiomyopathy presenting with haemodynamically tolerated VT, in the absence of reversible causes. Primary prevention | |
| p.43 | I | C | Comprehensive SCD risk stratification is recommended in all cardiomyopathy patients who have not suffered a previous cardiac arrest/sustained ventricular arrhythmia at initial evaluation and at 1–2 year intervals, or whenever there is a change in clinical status. | |
| p.43 | IIa | C | If a patient with cardiomyopathy requires pacemaker implantation, comprehensive SCD risk stratification to evaluate the need for ICD implantation should be considered. | |
| p.43 | I | A | When an ICD is indicated, it is recommended to evaluate whether the patient could benefit from CRT.533 | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | IIa | B | Subcutaneous defibrillators should be considered as an alternative to transvenous defibrillators in patients with an indication for an ICD when pacing therapy for bradycardia, cardiac resynchronization, or antitachycardia pacing is not anticipated.543 | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.43 | IIa | C | The wearable cardioverter defibrillator should be considered for adult patients with a secondary prevention ICD indication who are temporarily not candidates for ICD implantation. Continued | Recommendation Table 12 — Recommendations for implantable cardioverter defibrillator in patients with cardiomyopathy |
| p.44 | I | C | It is recommended that all clinically stable patients with cardiomyopathy undergo routine follow-up using a multiparametric approach that includes ECG and echocardiography every 1 to 2 years. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | I | C | Clinical evaluation with ECG and multimodality imaging is recommended in patients with cardiomyopathy whenever there is a substantial or unexpected change in symptoms. ECG, electrocardiogram. aClass of recommendation. bLevel of evidence. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | I | B | Following cascade genetic testing, clinical evaluation using a multiparametric approach that includes ECG and cardiac imaging and long-term follow-up is © ESC 2023 recommended in first-degree relatives who have the same disease-causing variant as the proband.178,544,547 | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | I | C | Following cascade genetic testing, it is recommended that first-degree relatives without a phenotype who do not have the same disease-causing variant as the proband are discharged from further follow-up but advised to seek re-assessment if they develop symptoms or when new clinically relevant data emerge in the family. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | I | C | It is recommended that when no P/LP variant is identified in the proband or genetic testing is not performed, an initial clinical evaluation using a multiparametric approach that includes ECG and cardiac imaging is performed in first-degree relatives. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | IIa | C | When no P/LP variant is identified in the proband or genetic testing is not performed, regular, long-term clinical evaluation using a multiparametric approach that includes ECG and cardiac imaging should be considered in first-degree relatives. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.44 | IIa | C | During cascade screening, where a first-degree relative has died, clinical evaluation of close relatives of the deceased individual (i.e. second-degree relatives of the index patient) should be considered. ECG, electrocardiogram, P/LP, pathogenic/likely pathogenic. aClass of recommendation. bLevel of evidence. | Recommendation Table 14 — Recommendations for family screening and follow-up evaluation of relatives |
| p.47 | I | B | It is recommended that psychological support by an appropriately trained health professional be offered to all individuals who have experienced the premature sudden cardiac death of a family member with cardiomyopathy.558,570,571,576,577 | Recommendation Table 15 — Recommendations for psychological support in patients and family members with cardiomyopathies |
| p.47 | IIa | C | Psychological support by an appropriately trained health professional should be considered in all patients and families with an inherited cardiomyopathy and in particular for those issues described in the text.c aClass of recommendation. bLevel of evidence. cSee Table 16. | Recommendation Table 15 — Recommendations for psychological support in patients and family members with cardiomyopathies |
| p.47 | I | B | In all patients with HCM, at initial evaluation, transthoracic 2D and Doppler echocardiography are recommended, at rest and during Valsalva manoeuvre in the sitting and semi-supine positions— and then on standing if no gradient is provoked—to detect LVOTO.84,86,365,525,584,587,589–594 | Recommendation Table 16 — Recommendation for evaluation of left ventricular outflow tract obstruction |
| p.47 | I | B | In symptomatic patients with HCM and a resting or provokedc peak instantaneous LV outflow tract gradient <50 mmHg, 2D and Doppler echocardiography during exercise in the standing, sitting (when possible), or semi-supine position are recommended to detect provocable LVOTO and exercise-induced mitral regurgitation.588,595–598 | Recommendation Table 16 — Recommendation for evaluation of left ventricular outflow tract obstruction |
| p.47 | IIa | C | Transoesophageal echocardiography should be considered in patients with HCM and LVOTO if the mechanism of obstruction is unclear or when assessing the mitral valve apparatus before a septal reduction procedure, or when severe mitral regurgitation caused by intrinsic valve abnormalities is suspected.599–602 | Recommendation Table 16 — Recommendation for evaluation of left ventricular outflow tract obstruction |
| p.48 | IIb | C | In symptomatic patients with HCM and inconclusive non-invasive cardiac imaging, left and right heart catheterization may be considered to assess the severity of LVOTO and to measure LV filling pressures.603 | |
| p.48 | IIb | C | Contrast-enhanced CMR may be considered before ASA or myectomy to assess the extent and distribution of hypertrophy and myocardial fibrosis.606,607 ASA, alcohol septal ablation; CMR, cardiac magnetic resonance. aClass of recommendation. bLevel of evidence. Comments | Recommendation Table 17 — Additional recommendation for cardiovascular magnetic resonance evaluation in hypertrophic cardiomyopathy |
| p.51 | IIa | C | Avoidance of digoxin and arterial and venous dilators, including nitrates and phosphodiesterase inhibitors, should be considered, if possible, in patients with resting or provocable LVOTO.626,627 | Recommendation Table 18 — Recommendations for treatment of left ventricular outflow tract obstruction (general measures) |
| p.51 | IIa | C | Restoration of sinus rhythm or appropriate rate control should be considered before invasive management of LVOTO in patients with new-onset or poorly controlled AF.629,630 | Recommendation Table 18 — Recommendations for treatment of left ventricular outflow tract obstruction (general measures) |
| p.52 | I | B | Non-vasodilating beta-blockers, titrated to maximum tolerated dose, are recommended as first-line therapy to improve symptoms in patients with resting or provokedc LVOTO.631–633,648–650 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | I | B | Verapamil or diltiazem, titrated to maximum tolerated dose, are recommended to improve symptoms in symptomatic patients with resting or provokedc LVOTO who are intolerant or have contraindications to beta-blockers.633,637–641 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | I | B | Disopyramide,d titrated to maximum tolerated dose, is recommended in addition to a beta-blocker (or, if this is not possible, with verapamil or diltiazem) to improve symptoms in patients with resting or provokedc LVOTO.632–634 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIa | A | Cardiac myosin ATPase inhibitor (mavacamten), titrated to maximum tolerated dose with echocardiographic surveillance of LVEF, should be considered in addition to a beta-blocker (or, if this is not possible, with verapamil or diltiazem) to improve symptoms in adult patients with resting or provokedc LVOTO.622,642–646 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIa | B | echocardiographic surveillance of LVEF, should be considered as monotherapy in symptomatic adult patients with resting or provokedc LVOTO (exercise or Valsalva manoeuvre) who are intolerant or have contraindications to beta-blockers, verapamil/ | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIa | C | diltiazem, or disopyramide.622,644–646 Oral or i.v. beta-blockers and vasoconstrictors should be considered in patients with severe provocablec LVOTO presenting with hypotension and acute pulmonary oedema who do not respond to fluid administration.627 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIb | C | Disopyramide, titrated to maximum tolerated dose, may be considered as monotherapy in patients who are intolerant to or have contraindications to beta-blockers and verapamil/diltiazem to improve symptoms in patients with resting or provokedc | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIb | C | LVOTO.632 Beta-blockers or verapamil may be considered in selected cases in asymptomatic patients with resting or provokedc LVOTO to reduce LV pressures.623,639 | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.52 | IIb | C | The cautious use of low-dose diuretics may be considered in symptomatic LVOTO to improve exertional dyspnoea. Continued | Recommendation Table 19 — Recommendations for medical treatment of left ventricular outflow tract obstruction |
| p.54 | I | C | It is recommended that SRT be performed by experienced operators working as part of a multidisciplinary team expert in the management of HCM.664,665,687,695,696,708–710 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | I | B | SRT to improve symptoms is recommended in patients with a resting or maximum provoked LVOT gradient of ≥50 mmHg who are in NYHA/Ross functional class III–IV, despite maximum tolerated medical therapy.697–702 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | I | C | Septal myectomy, rather than ASA, is recommended in children with an indication for SRT, as well as in adult patients with an indication for SRT and other lesions requiring surgical intervention (e.g. mitral valve abnormalities).673 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | IIa | C | SRT should be considered in patients with recurrent exertional syncope caused by a resting or maximum provoked LVOTO gradient ≥50 mmHg despite optimal medical therapy.686,711–713 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | IIa | C | Mitral valve repair or replacement should be considered in symptomatic patients with a resting or maximum provoked LVOTO gradient ≥50 mmHg and moderate-to-severe mitral regurgitation that cannot be corrected by SRT alone.661,669–672,714 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | IIa | C | Mitral valve repair should be considered in patients with a resting or maximum provoked LVOTO gradient ≥50 mmHg when there is moderate-to-severe mitral regurgitation following isolated myectomy. SRT may be considered in expert centres with | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.54 | IIb | C | demonstrable low procedural complication rates in patients with mild symptoms (NYHA class II) refractory to medical therapy who have a resting or maximum provoked (exercise or Valsalva) gradient of ≥50 mmHg and: • moderate-to-severe SAM-related mitral regurgitation; or • AF; or • moderate-to-severe left atrial dilatation.653,715 | Recommendation Table 20 — Recommendations for septal reduction therapy |
| p.55 | IIb | C | Mitral valve replacement may be considered in patients with a resting or maximum provoked LVOTO gradient ≥50 mmHg when there is moderate-to-severe mitral regurgitation following isolated myectomy.661,674,714,716 | |
| p.55 | IIb | C | Surgical AF ablation and/or left atrial appendage occlusion procedures during septal myectomy may be considered in patients with HCM and symptomatic AF.717,718 | |
| p.55 | IIb | C | Sequential AV pacing, with optimal AV interval to reduce the LV outflow tract gradient or to facilitate medical treatment with beta-blockers and/or verapamil, may be considered in selected patients with resting or provocable LVOTO ≥50 mmHg, sinus rhythm, and drug-refractory symptoms, who have contraindications for ASA or septal myectomy or are at high risk of developing heart block following ASA or septal myectomy.633,719–724 | Recommendation Table 21 — Recommendations for indications for cardiac pacing in patients with obstruction |
| p.55 | IIb | C | In patients with resting or provocable LVOTO ≥50 mmHg, sinus rhythm, and drug-refractory symptoms, in whom there is an indication for an ICD, a dual-chamber ICD (instead of a single-lead device) may be considered, to reduce the LV outflow tract gradient or to facilitate medical treatment with beta-blockers and/or verapamil.633,719–724,726 | Recommendation Table 21 — Recommendations for indications for cardiac pacing in patients with obstruction |
| p.55 | IIa | C | Beta-blockers and calcium antagonists (verapamil or diltiazem) should be considered to improve symptoms in patients with angina-like chest pain even in the absence of LVOTO or obstructive CAD.740–744 | Recommendation Table 22 — Recommendations for chest pain on exertion in patients without left ventricular outflow tract obstruction |
| p.55 | IIb | C | Oral nitrates may be considered to improve symptoms in patients with angina-like chest pain, even in the absence of obstructive CAD, if there is no LVOTO. | Recommendation Table 22 — Recommendations for chest pain on exertion in patients without left ventricular outflow tract obstruction |
| p.55 | IIb | C | Ranolazine may be considered to improve symptoms in patients with angina-like chest pain even in the © ESC 2023 absence of LVOTO or obstructive CAD. 738,739 | Recommendation Table 22 — Recommendations for chest pain on exertion in patients without left ventricular outflow tract obstruction |
| p.59 | I | B | Secondary prevention Implantation of an ICD is recommended in patients who have survived a cardiac arrest due to VT or VF, or who have spontaneous sustained VT with haemodynamic compromise.532,534,726,831,832 | Recommendation Table 23 — Additional recommendations for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy |
| p.59 | I | B | method of estimating risk of sudden death at 5 years in patients aged ≥16 years for primary prevention.525,821–824 | Recommendation Table 23 — Additional recommendations for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy |
| p.59 | I | B | Validated paediatric-specific risk prediction models (e.g. HCM Risk-Kids) are recommended as a method of estimating risk of sudden death at 5 years in patients aged <16 years for primary prevention.81,833 Continued | Recommendation Table 23 — Additional recommendations for prevention of sudden cardiac death in patients with hypertrophic cardiomyopathy |
| p.60 | I | B | It is recommended that the 5-year risk of SCD be assessed at first evaluation and re-evaluated at 1–2 year intervals or whenever there is a change in clinical status.525 Implantation of an ICD should be considered in patients with an estimated 5-year risk of sudden | |
| p.60 | IIa | B | death of ≥6%, following detailed clinical assessment that considers: (i) the lifelong risk of complications; (ii) competing mortality risk from the disease and comorbidities; AND (iii) the impact of an ICD on lifestyle, | |
| p.60 | IIa | B | socio-economic status, and psychological health.81,521,525,726,832,833 In patients with LV apical aneurysms, decisions about primary prevention ICD based on an assessment of risk using the HCM Risk-SCD or a validated paediatric risk-prediction (e.g. HCM Risk-Kids) tool and not solely on the presence of the aneurysm should be considered.580,728,737,791,792 | |
| p.60 | IIb | B | Implantation of an ICD may be considered in individual patients with an estimated 5-year risk of SCD of between ≥4% and <6%, following detailed clinical assessment that takes into account the lifelong risk of complications and the impact of an ICD on lifestyle, socio-economic status, and psychological health.81,521,525,726,832,833 For patients who are in the low-risk category (<4% estimated 5-year risk of SCD), the presence of extensive LGE (≥15%) on CMR may be considered in shared decision-making with patients about prophylactic ICD implantation, acknowledging the | |
| p.60 | IIb | B | lack of robust data on the impact of scar quantification on the personalized risk estimates generated by HCM Risk-SCD or a validated paediatric model (e.g. HCM Risk-Kids).141,796,797,834– For patients who are in the low-risk category (<4% estimated 5-year risk of SCD), the presence of LVEF <50% may be considered in shared decision-making with patients about prophylactic ICD implantation, acknowledging the lack of robust data on the impact of systolic dysfunction on the personalized risk estimates generated by HCM Risk-SCD or a validated paediatric model (e.g. HCM Risk-Kids).89,315,841–844 | |
| p.64 | IIa | B | The patient’s genotype should be considered in the estimation of SCD risk in DCM.185,186,869,886 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.64 | I | B | Secondary prevention An ICD is recommended to reduce the risk of sudden death and all-cause mortality in patients with DCM who have survived a cardiac arrest or have recovered from a ventricular arrhythmia causing haemodynamic instability.530,531,884 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.64 | IIa | A | Primary prevention An ICD should be considered to reduce the risk of sudden death and all-cause mortality in patients with DCM, symptomatic heart failure, and LVEF ≤35% despite >3 months of OMT.861,885 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.64 | IIa | C | An ICD should be considered in patients with DCM with a genotype associated with high SCD risk and LVEF >35% in the presence of additional risk factors (see Table 21).541,542,867,869,873,878,881,886 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.64 | IIb | C | An ICD may be considered in selected patients with DCM with a genotype associated with high SCD risk and LVEF >35% without additional risk factors (see Table 21).869,873,881,886 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.64 | IIb | C | An ICD may be considered in patients with DCM without a genotype associated with high SCD risk and LVEF >35% in the presence of additional risk factors.c,138,873,874 | Recommendation Table 24 — Recommendations for an implantable cardioverter defibrillator in patients with dilated cardiomyopathy |
| p.65 | I | C | Ambulatory ECG monitoring is recommended in patients with NDLVC annually or when there is a change in clinical status, to aid in management and risk stratification. | Recommendation Table 25 — Recommendation for resting and ambulatory electrocardiogram monitoring in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | I | C | Secondary prevention An ICD is recommended to reduce the risk of sudden death and all-cause mortality in patients with NDLVC who have survived a cardiac arrest or have recovered from a ventricular arrhythmia causing haemodynamic instability. | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | IIa | A | Primary prevention An ICD should be considered to reduce the risk of sudden death and all-cause mortality in patients with NDLVC, heart failure symptoms, and LVEF ≤35% despite >3 months of OMT.861,885 | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | IIa | C | The patient’s genotype should be considered in the estimation of SCD risk in NDLVC. | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | IIa | C | An ICD should be considered in patients with NDLVC with a genotype associated with high SCD risk and LVEF >35% in the presence of additional risk factors (see Table 21).185,186,438,541,542,865–869,878–883 | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | IIb | C | An ICD may be considered in selected patients with NDLVC with a genotype associated with high SCD risk and LVEF >35% without additional risk factors (see Table 21). | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.66 | IIb | C | An ICD may be considered in patients with NDLVC without a genotype associated with high SCD risk and LVEF >35% in the presence of additional risk factors.c | Recommendation Table 26 — Recommendations for an implantable cardioverter defibrillator in patients with non-dilated left ventricular cardiomyopathy |
| p.67 | I | C | Annual ambulatory ECG monitoring is recommended in patients with ARVC to aid in diagnosis, management, and risk stratification.902 | Recommendation Table 27 — Recommendation for resting and ambulatory electrocardiogram monitoring in patients with arrhythmogenic right ventricular cardiomyopathy |
| p.68 | I | C | patients with VE, NSVT, and VT.920–922 | Recommendation Table 28 — Recommendations for the antiarrhythmic management of patients with arrhythmogenic right ventricular cardiomyopathy |
| p.68 | IIa | C | Beta-blocker therapy is recommended in ARVC Amiodarone should be considered when regular beta-blocker therapy fails to control arrhythmia-related symptoms in patients with ARVC.921,922 | Recommendation Table 28 — Recommendations for the antiarrhythmic management of patients with arrhythmogenic right ventricular cardiomyopathy |
| p.68 | IIa | C | Flecainide in addition to beta-blockers should be considered when single agent treatment has failed to control arrhythmia-related symptoms in patients with ARVC.923,924 | Recommendation Table 28 — Recommendations for the antiarrhythmic management of patients with arrhythmogenic right ventricular cardiomyopathy |
| p.68 | IIa | C | Catheter ablation with availability for epicardial approach guided by 3D electroanatomical mapping of VT should be considered in ARVC patients with incessant VT or frequent appropriate ICD interventions for VT despite pharmacological therapy with beta-blockers.925,929–934 | Recommendation Table 28 — Recommendations for the antiarrhythmic management of patients with arrhythmogenic right ventricular cardiomyopathy |
| p.69 | I | A | Secondary prevention An ICD is recommended to reduce the risk of sudden death and all-cause mortality in patients with ARVC who have survived a cardiac arrest or have recovered from a ventricular arrhythmia causing haemodynamic instability.939,943,944,948,949 | Recommendation Table 29 — Recommendations for sudden cardiac death prevention in patients with arrhythmogenic right ventricular cardiomyopathy |
| p.69 | IIa | B | An ICD should be considered in ARVC patients who have suffered a haemodynamically tolerated VT.522,939,943–945,948–950 | Recommendation Table 29 — Recommendations for sudden cardiac death prevention in patients with arrhythmogenic right ventricular cardiomyopathy |
| p.69 | IIa | B | Primary prevention High-risk featuresc should be considered to aid individualized decision-making for ICD implantation in patients with ARVC.538,939 The updated 2019 ARVC risk calculator should be considered to aid individualized decision-making for ICD implantation in patients with ARVC.d,524,526,536– | Recommendation Table 29 — Recommendations for sudden cardiac death prevention in patients with arrhythmogenic right ventricular cardiomyopathy |
| p.72 | I | C | It is recommended that multimodality imaging be used to differentiate RCM from HCM or DCM with restrictive physiology. | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.72 | I | C | It is recommended that baseline cardiac and non-cardiac investigations are performed to assess involvement of the neuromuscular system or other syndromic disorders. | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.72 | I | B | Cardiac catheterization is recommended in all children with RCM to measure pulmonary artery pressures and PVR at diagnosis and at 6–12 monthly intervals to assess change in PVR.953 | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.72 | I | C | ICD implantation is recommended to reduce the risk of sudden death and all-cause mortality in patients with RCM who have survived a cardiac arrest or have recovered from a ventricular arrhythmia causing haemodynamic instability. | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.72 | IIa | C | Endomyocardial biopsy should be considered in patients with RCM to exclude specific diagnoses (including iron overload, storage disorders, mitochondrial cytopathies, amyloidosis, and granulomatous myocardial diseases) and to diagnose | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.72 | IIb | C | restrictive myofibrillar disease caused by desmin variants. ICD implantation may be considered in children with RCM who have evidence of myocardial ischaemia and syncope.969 | Recommendation Table 30 — Recommendations for the management of patients with restrictive cardiomyopathy |
| p.80 | I | C | All cardiomyopathies Regular low- to moderate-intensity exercise is recommended in all able individuals with cardiomyopathy. | Recommendation Table 31 — Exercise recommendations for patients with cardiomyopathy |
| p.81 | I | C | An individualized risk assessment for exercise prescription is recommended in all patients with cardiomyopathy. HCM | |
| p.81 | IIa | C | High-intensity exercise and competitive sport should be considered in genotype-positive/ phenotype-negative individuals who seek to do so.1124 | |
| p.81 | IIb | B | High-intensity exercise and competitive sport may be considered in asymptomatic low-riskc individuals with morphologically mild hypertrophic cardiomyopathy in the absence of resting or inducible left ventricular outflow obstruction and exercise-induced complex ventricular | |
| p.81 | III | C | arrhythmias.1107,1113,1125,1126 High-intensity exercise, including competitive sport, is not recommended in high-risk individuals and in individuals with left ventricular outflow tract obstruction and exercise-induced complex ventricular arrhythmias. | |
| p.81 | IIb | C | ARVC Avoidance of high-intensity exercise, including competitive sport, may be considered in genotype-positive/phenotype-negative individuals in families with ARVC.1111,1116,1117 | |
| p.81 | III | B | Moderate- and/or high-intensity exercise, including competitive sport, is not recommended in individuals with ARVC.181,1111–1114 DCM and NDLVC | |
| p.81 | IIa | C | Moderate- and high-intensity exercise should be considered in individuals who are gene positive and phenotype negative (with the exception of pathogenic variants in LMNA and TMEM43) who seek to do so.1123 | |
| p.81 | IIb | C | High-intensity exercise and competitive sport may be considered in a select group of asymptomatic and optimally treated individuals with a left ventricular ejection fraction ≥50% in the absence of exercise-induced complex arrhythmias. | |
| p.81 | IIb | C | Moderate-intensity exercise may be considered in asymptomatic and optimally treated individuals with a left ventricular ejection fraction of 40–49% in the absence of exercise-induced complex arrhythmias. | |
| p.81 | III | C | High-intensity exercise, including competitive sport, is not recommended in symptomatic individuals, those with a left ventricular ejection fraction ≤40%, exercise-induced arrhythmias or pathogenic variants in LMNA or TMEM43. | |
| p.83 | I | C | Pre-pregnancy risk assessment and counselling are recommended in all women using the mWHO classification of maternal risk. | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | Counselling on safe and effective contraception is recommended in all women of fertile age and their partners. | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | Counselling on the risk of disease inheritance is recommended for all men and women before conception. Vaginal delivery is recommended in most women | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | with cardiomyopathies, unless there are obstetric indications for caesarean section, severe heart failure (EF <30% or NYHA class III–IV), or severe outflow tract obstructions, or in women presenting in labour | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | on oral anticoagulants. It is recommended that medication be carefully reviewed for safety in advance of pregnancy and adjusted according to tolerability in pregnancy. | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | Therapeutic anticoagulation with LMWH or VKAs according to the stage of pregnancy is recommended for patients with AF. | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | IIa | C | Continuation of beta-blockers should be considered during pregnancy in women with cardiomyopathies, with close follow-up of foetal growth and of the condition of the neonate, and if benefits outweigh | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | IIa | C | risks. Genetic counselling and testing should be considered in patients with peripartum cardiomyopathy. | Recommendation Table 32 — Recommendations for reproductive issues in patients with cardiomyopathy |
| p.83 | I | C | Peri-operative ECG monitoring is recommended for all patients with cardiomyopathy undergoing surgery. In patients with cardiomyopathy and suspected or | Recommendation Table 33 — Recommendations for non-cardiac surgery in patients with cardiomyopathy |
| p.83 | I | B | known HF scheduled for intermediate or high-risk NCS, it is recommended to re-evaluate LV function with echocardiography (assessing LVOTO in HCM patients) and measurement of NT-proBNP/BNP levels, unless this has recently been performed.1151,1153–1156,1158–1165 | Recommendation Table 33 — Recommendations for non-cardiac surgery in patients with cardiomyopathy |
| p.83 | I | C | It is recommended that cardiomyopathy patients with high-risk genotypes or associated factors for arrhythmic or heart failure complications or severe LVOTO be referred for additional specialized investigations to a cardiomyopathy unit before | Recommendation Table 33 — Recommendations for non-cardiac surgery in patients with cardiomyopathy |
| p.83 | I | C | undergoing elective NCS. In patients aged <65 years with a first-degree relative with a cardiomyopathy, it is recommended to perform © ESC 2023 an ECG and TTE before NCS, regardless of symptoms. | Recommendation Table 33 — Recommendations for non-cardiac surgery in patients with cardiomyopathy |
| p.86 | I | C | Identification and management of risk factors and concomitant diseases is recommended as an integral part of the management of cardiomyopathy patients. aClass of recommendation. bLevel of evidence. | Recommendation Table 34 — Recommendation for management of cardiovascular risk factors in patients with cardiomyopathy |
| p.89 | I | B | Contrast-enhanced CMR is recommended in patients with cardiomyopathy at initial evaluation. | |
| p.89 | I | B | DPD/PYP/HMDP bone-tracer scintigraphy is recommended in patients with suspected ATTR-related cardiac amyloidosis to aid diagnosis. | |
| p.90 | I | B | Pre- and post-test genetic counselling is recommended in all individuals undergoing genetic testing for cardiomyopathy. | |
| p.90 | I | C | Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good quality survival >1 year. | |
| p.90 | III | C | It is not recommended to implant an ICD in patients with incessant ventricular arrhythmias until the ventricular arrhythmia is controlled. | |
| p.91 | I | A | When an ICD is indicated, it is recommended to evaluate whether the patient could benefit from CRT. | |
| p.92 | I | C | Annual ambulatory ECG monitoring is recommended in patients with ARVC to aid in diagnosis, management, and risk stratification. | |
| p.92 | I | C | Beta-blocker therapy is recommended in ARVC patients with VE, NSVT, and VT. | |
| p.92 | I | C | It is recommended that multimodality imaging be used to differentiate RCM from HCM or DCM with restrictive physiology. | |
| p.92 | I | C | Regular low- to moderate-intensity exercise is recommended in all able individuals with cardiomyopathy. | |
| p.92 | I | C | An individualized risk assessment for exercise prescription is recommended in all patients with cardiomyopathy. | |
| p.92 | III | B | Moderate- and/or high-intensity exercise, including competitive sport, is not recommended in individuals with ARVC. | |
| p.92 | I | C | Pre-pregnancy risk assessment and counselling are recommended in all women using the mWHO classification of maternal risk. | |
| p.93 | I | C | Counselling on safe and effective contraception is recommended in all women of fertile age and their partners. | |
| p.93 | I | C | Counselling on the risk of disease inheritance is recommended for all men and women before conception. | |
| p.93 | I | C | Therapeutic anticoagulation with LMWH or VKAs according to the stage of pregnancy is recommended for patients with AF. | |
| p.93 | I | C | Peri-operative ECG monitoring is recommended for all patients with cardiomyopathy undergoing surgery. |