General Cardiology
HCM management, screening, and emerging therapies
Also called HCM, HOCM, hypertrophic obstructive cardiomyopathy
Sources: 120 guideline recommendations · 4 landmark trials · 12 new papers · 5 key papers · updated Oct 20, 2026
Updated Oct 14, 2026: 6 experts on ACACIA-HCM
What 6 experts said about this trial's result
Expert labels are toward ACACIA-HCM's result, from named public quotes (n=6). As of Oct 14, 2026.
The strongest guideline recommendations for hypertrophic cardiomyopathy (Class I and IIa). Dosing is left to the guideline.
For patients with obstructive HCM who have persistent symptoms attributable to LVOTO despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (adult patients only), or disopyramide (in combination with an atrioventricular nodal blocking agent), or SRT performed at experienced centers, is recommended.
For patients with HCM, mild- to moderate-intensity recreational exercise is beneficial to improve cardiorespiratory fitness, physical functioning, and quality of life, and for overall health in keeping with physical activity guidelines for the general population.
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.
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.
Unverified · drafted by AI from the sources cited below
Hypertrophic cardiomyopathy is a cardiac muscle disease characterized by left and/or right ventricular hypertrophy, frequent asymmetrical involvement of the heart septum, and normal or reduced left ventricular volume.1 Risk factors for the condition include hypertension, aortic stenosis, and gene mutation.1
Hypertrophic cardiomyopathy carries a substantial risk of major adverse cardiovascular events, yet current tools do not fully exploit quantitative myocardial texture from routine echocardiography.1 A study developed a deep survival model integrating transthoracic echocardiographic texture radiomics with clinical and conventional echocardiographic variables, showing that age, RadScore, left atrial diameter, and maximal wall thickness were leading contributors.1
Earlier trials experts reacted to
Expert labels are toward each trial's result, from named public quotes.
How AHA/ACC 2024 and ESC 2023 grade each treatment for hypertrophic cardiomyopathy. Each chip is that guideline's strongest recommendation; open it for the exact wording and who it applies to.
Septal reduction therapy
AHA/ACC 2024 · Class I · LOE B-R
For patients with obstructive HCM who have persistent symptoms attributable to LVOTO despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (adult patients only), or disopyramide (in combination with an atrioventricular nodal blocking agent), or SRT performed at experienced centers, is recommended.
ESC 2023 · Class I · LOE B
Primary results of 4 trials behind the evidence on hypertrophic cardiomyopathy, as each trial’s own publication reports them.
aficamten vs metoprolol · 175 patients · N Engl J Med 2025
Primary outcome: the change in peak oxygen uptake at week 24
“least-squares mean between-group difference, 2.3 ml per kilogram per minute; 95% CI, 1.5 to 3.1; P<0.001”
aficamten vs placebo · 282 patients · N Engl J Med 2024
Primary outcome: the change from baseline to week 24 in the peak oxygen uptake as assessed by cardiopulmonary exercise testing
“least-squares mean between-group difference, 1.7 ml per kilogram per minute; 95% CI, 1.0 to 2.4; P<0.001”
mavacamten vs placebo · 112 patients · J Am Coll Cardiol 2022
Primary outcome: the composite of the proportion of patients proceeding with SRT or who remained guideline-eligible after 16 weeks' treatment
“difference (58.9%; 95% CI: 44.0%-73.9%; P < 0.001)”
Class of recommendation is how strongly the society advises the action (I is strongest, III is not recommended). Level of evidence is the kind of studies behind it (A is randomized trials, C is expert opinion or limited data).
120 recommendations on hypertrophic cardiomyopathy from 2 guidelines, by strength.
Class IClass IIa
2024 AHA/ACC/AMSSM/HRS/PACES/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy
Journal of the American College of Cardiology — Ommen S
387 citations · 2024 · among the most cited on Hypertrophic cardiomyopathy
Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): health status analysis of a randomised, double-blind, placebo-controlled, phase 3 trial
The Lancet — Spertus J
223 citations · 2021 · among the most cited on Hypertrophic cardiomyopathy
Effect of Mavacamten on Echocardiographic Features in Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy
Journal of the American College of Cardiology — Hegde S
198 citations · 2021 · among the most cited on Hypertrophic cardiomyopathy
A clinical question appears here only when its condition matches this topic, an outcome and a comparator were extracted from the studies, at least two studies and a patient count are on file, and the certainty is low or higher.
Supports benefit, no benefit shown, and mixed describe the direction of the measured effect. No benefit shown means the evidence does not support a benefit. It is not a finding of harm. Too little evidence means the question did not clear that bar.
Study count, RCT count, and patient count are separate. The patient total adds only studies that are linked into a study family and whose sample size is not flagged as implausible. A reversal is listed only when at least one randomized trial sits on each side of the change.
The overview is drafted from the guideline recommendations, trials, papers, and questions on this page, and every sentence has to cite one of them. It stays unverified until an NPI-verified clinician or a Synapse admin approves it. Approving records that person's name.
Improving symptoms is a primary treatment goal in patients with obstructive hypertrophic cardiomyopathy.1 Available pharmacological options are not disease-specific and are often inadequate or poorly tolerated.1 Mavacamten, a cardiac myosin inhibitor, has been studied in a phase 3 trial for patients with symptomatic obstructive hypertrophic cardiomyopathy, aiming to assess its effect on patients' health status.1 Another study evaluated the long-term mortality of patients with obstructive hypertrophic cardiomyopathy following alcohol septal ablation or septal myectomy.2 In a cohort of 3,859 patients, 585 (15.2%) underwent alcohol septal ablation and 3,274 (84.8%) underwent septal myectomy.2
The impact of coexisting type 2 diabetes mellitus on the phenotype and prognosis of hypertrophic cardiomyopathy remains insufficiently studied.1 Also, although pathogenic variants in sarcomeric genes are well-recognized contributors, the mechanisms linking these mutations to the heterogeneous phenotypes remain poorly understood.2
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.
Disopyramide
AHA/ACC 2024 · Class I · LOE B-R
For patients with obstructive HCM who have persistent symptoms attributable to LVOTO despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (adult patients only), or disopyramide (in combination with an atrioventricular nodal blocking agent), or SRT performed at experienced centers, is recommended.
ESC 2023 · Class I · LOE B
Disopyramide, 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 provoked LVOTO.
ICD
AHA/ACC 2024 · Class I · LOE B-NR
For patients with HCM and previous documented cardiac arrest or sustained VT, ICD placement is recommended (Figure 3, Table 8).
ESC 2023 · Class I · LOE B
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.
Beta blockers
AHA/ACC 2024 · Class I · LOE B-NR
In patients with obstructive HCM and symptoms attributable to LVOTO, nonvasodilating beta blockers, titrated to effectiveness or maximally tolerated doses, are recommended.
ESC 2023 · Class I · LOE B
Non-vasodilating beta-blockers, titrated to maximum tolerated dose, are recommended as first-line therapy to improve symptoms in patients with resting or provoked LVOTO.
Verapamil
AHA/ACC 2024 · Class I · LOE C-LD
In patients with AF in whom rate control strategy is planned, beta blockers, verapamil, or diltiazem are recommended, with the choice of agents according to patient preferences and comorbid conditions.
ESC 2023 · Class I · LOE B
Verapamil or diltiazem, titrated to maximum tolerated dose, are recommended to improve symptoms in symptomatic patients with resting or provoked LVOTO who are intolerant or have contraindications to beta-blockers.
Diltiazem
AHA/ACC 2024 · Class I · LOE C-LD
In patients with AF in whom rate control strategy is planned, beta blockers, verapamil, or diltiazem are recommended, with the choice of agents according to patient preferences and comorbid conditions.
ESC 2023 · Class I · LOE B
Verapamil or diltiazem, titrated to maximum tolerated dose, are recommended to improve symptoms in symptomatic patients with resting or provoked LVOTO who are intolerant or have contraindications to beta-blockers.
Surgical myectomy
AHA/ACC 2024 · Class I · LOE B-NR
In symptomatic patients with obstructive HCM who have associated cardiac disease requiring surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral leaflet, intrinsic mitral valve disease, multivessel CAD, valvular aortic stenosis), surgical myectomy, performed at experienced HCM centers, is recommended (Tables 4 and 5).
ESC 2023 · Class I · LOE 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).
Cardiac myosin inhibitor
AHA/ACC 2024 · Class I · LOE B-R
For patients with obstructive HCM who have persistent symptoms attributable to LVOTO despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (adult patients only), or disopyramide (in combination with an atrioventricular nodal blocking agent), or SRT performed at experienced centers, is recommended.
ESC 2023 · Class IIa · LOE 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 provoked LVOTO.
Recreational exercise
AHA/ACC 2024 · Class I · LOE B-R
For patients with HCM, mild- to moderate-intensity recreational exercise is beneficial to improve cardiorespiratory fitness, physical functioning, and quality of life, and for overall health in keeping with physical activity guidelines for the general population.
DOAC
AHA/ACC 2024 · Class I · LOE B-NR
In patients with HCM and clinical AF, anticoagulation is recommended with direct-acting oral anticoagulants (DOACs) as first-line option and vitamin K antagonists as second-line option, independent of CHA2DS2-VASc score.
Vitamin K antagonist
AHA/ACC 2024 · Class I · LOE B-NR
In patients with HCM and clinical AF, anticoagulation is recommended with direct-acting oral anticoagulants (DOACs) as first-line option and vitamin K antagonists as second-line option, independent of CHA2DS2-VASc score.
Heart transplantation
AHA/ACC 2024 · Class I · LOE B-NR
In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) or with life-threatening ventricular arrhythmias refractory to maximal GDMT, assessment for heart transplantation in accordance with current listing criteria is recommended.
Weight loss
AHA/ACC 2024 · Class I · LOE B-NR
In patients with HCM who are overweight or obese, counseling and comprehensive lifestyle interventions are recommended for achieving and maintaining weight loss and possibly lowering the risk of developing LVOTO, HF, and AF.
Alcohol septal ablation
AHA/ACC 2024 · Class I · LOE C-LD
In adult patients with obstructive HCM who remain severely symptomatic, despite GDMT and in whom surgery is contraindicated or the risk is considered unacceptable because of serious comorbidities or advanced age, alcohol septal ablation in eligible patients, performed at experienced HCM centers, is recommended (Tables 4 and 5).
Antiarrhythmic drugs
AHA/ACC 2024 · Class I · LOE C-LD
In children with HCM and recurrent ventricular arrhythmias despite beta-blocker use, antiarrhythmic drug therapy (eg, amiodarone, mexiletine, sotalol) is recommended, with the choice of agent guided by age, underlying comorbidities, severity of disease, patient preferences, and balance of efficacy and safety.
Antitachycardia pacing
AHA/ACC 2024 · Class I · LOE C-LD
In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimize risk of shocks.
Blood pressure control
AHA/ACC 2024 · Class I · LOE C-LD
In patients with HCM and hypertension, lifestyle modifications and medical therapy for hypertension are recommended, with preference for beta blockers and nondihydropyridine calcium channel blockers in patients with obstructive HCM.
GDMT for HF with reduced EF
AHA/ACC 2024 · Class I · LOE C-LD
In patients with HCM who develop systolic dysfunction with an LVEF <50%, GDMT for HF with reduced EF is recommended.
Nondihydropyridine calcium channel blockers
AHA/ACC 2024 · Class I · LOE C-LD
In patients with nonobstructive HCM with preserved EF and symptoms of exertional angina or dyspnea, beta blockers or nondihydropyridine calcium channel blockers are recommended.
Phenylephrine
AHA/ACC 2024 · Class I · LOE C-LD
For patients with obstructive HCM and acute hypotension who do not respond to fluid administration, intravenous phenylephrine (or other vasoconstrictors without inotropic activity), alone or in combination with beta-blocking drugs, is recommended.
Rhythm control
AHA/ACC 2024 · Class IIa · LOE B-NR
In patients with HCM and poorly tolerated AF, a rhythm-control strategy with cardioversion or antiarrhythmic drugs can be beneficial with the choice of an agent according to AF symptom severity, patient preferences, and comorbid conditions.
ESC 2023 · Class IIa · LOE 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.
Dual-chamber ICD
AHA/ACC 2024 · Class IIa · LOE B-NR
In patients with HCM who are receiving an ICD, dual-chamber ICDs are reasonable for patients with a need for atrial or atrioventricular sequential pacing for bradycardia/conduction abnormalities, or as an attempt to relieve symptoms of obstructive HCM (most commonly in patients >65 years of age).
ESC 2023 · Class IIb · LOE 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.
Surgical AF ablation
AHA/ACC 2024 · Class IIa · LOE B-NR
In patients with HCM and AF who require surgical myectomy, concomitant surgical AF ablation procedure can be beneficial for AF rhythm control.
ESC 2023 · Class IIb · LOE C
Surgical AF ablation and/or left atrial appendage occlusion procedures during septal myectomy may be considered in patients with HCM and symptomatic AF.
Diuretics
AHA/ACC 2024 · Class IIa · LOE C-EO
In patients with nonobstructive HCM with preserved EF, it is reasonable to add oral diuretics when exertional dyspnea persists despite the use of beta blockers or nondihydropyridine calcium channel blockers.
ESC 2023 · Class IIb · LOE C
The cautious use of low-dose diuretics may be considered in symptomatic LVOTO to improve exertional dyspnoea.
Catheter ablation
AHA/ACC 2024 · Class IIa · LOE B-NR
In patients with HCM and symptomatic AF, as part of an AF rhythm- control strategy, catheter ablation for AF can be effective when drug therapy is ineffective, contraindicated, or not the patient’s preference.
LVAD
AHA/ACC 2024 · Class IIa · LOE B-NR
In patients with nonobstructive HCM and advanced HF (NYHA functional class III to class IV despite GDMT) who are candidates for heart transplantation, continuous-flow LVAD therapy is reasonable as a bridge to heart transplantation.
Cardiac resynchronization therapy
AHA/ACC 2024 · Class IIa · LOE C-LD
In selected adult patients with nonobstructive HCM receiving an ICD who have NYHA class II to ambulatory class IV HF, left bundle branch block (LBBB), and LVEF <50%, cardiac resynchronization therapy (CRT) for symptom reduction is reasonable.
Calcium antagonists
ESC 2023 · Class IIa · LOE 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.
Cardioversion
AHA/ACC 2024 · Class IIa · LOE C-LD
In pregnant women with HCM, cardioversion for new or recurrent AF, particularly if symptomatic, is reasonable.
Mitral valve repair
ESC 2023 · Class IIa · LOE 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.
Mitral valve repair or replacement
ESC 2023 · Class IIa · LOE 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.
Rate control
ESC 2023 · Class IIa · LOE 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.
Vasoconstrictors
ESC 2023 · Class IIa · LOE C
Oral or i.v. beta-blockers and vasoconstrictors should be considered in patients with severe provocable LVOTO presenting with hypotension and acute pulmonary oedema who do not respond to fluid administration.
Mitral valve replacement
AHA/ACC 2024 · Class III · LOE B-NR
For symptomatic patients with obstructive HCM in whom SRT is an option, mitral valve replacement should not be performed for the sole purpose of relief of LVOTO.
ESC 2023 · Class IIb · LOE 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.
Valsartan
AHA/ACC 2024 · Class IIb · LOE B-R
For younger (eg, ≤45 years of age) patients with nonobstructive HCM due to a pathogenic or likely pathogenic cardiac sarcomere genetic variant, and a mild phenotype, valsartan may be beneficial to slow adverse cardiac remodeling.
ACE inhibitors
AHA/ACC 2024 · Class IIb · LOE C-LD
In patients with nonobstructive HCM with preserved EF, the usefulness of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers in the treatment of symptoms (angina and dyspnea) is not well established.
Angiotensin receptor blockers
AHA/ACC 2024 · Class IIb · LOE C-LD
In patients with nonobstructive HCM with preserved EF, the usefulness of angiotensin-converting enzyme inhibitors and angiotensin receptor blockers in the treatment of symptoms (angina and dyspnea) is not well established.
Apical myectomy
AHA/ACC 2024 · Class IIb · LOE C-LD
In highly selected patients with apical HCM with severe dyspnea or angina (NYHA functional class III or class IV) despite maximal medical therapy, and with preserved EF and small LV cavity size (LV end-diastolic volume <50 mL/m2 and LV stroke volume <30 mL/m2), apical myectomy by experienced surgeons at comprehensive centers may be considered to reduce symptoms.
Left atrial appendage occlusion
ESC 2023 · Class IIb · LOE C
Surgical AF ablation and/or left atrial appendage occlusion procedures during septal myectomy may be considered in patients with HCM and symptomatic AF.
Oral nitrates
ESC 2023 · Class IIb · LOE 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.
Ranolazine
ESC 2023 · Class IIb · LOE C
Ranolazine may be considered to improve symptoms in patients with angina-like chest pain even in the absence of LVOTO or obstructive CAD.
Sequential AV pacing
ESC 2023 · Class IIb · LOE 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.
mavacamten vs placebo · 251 patients · Lancet 2020
Primary outcome: a 1·5 mL/kg per min or greater increase in peak oxygen consumption (pVO2) and at least one NYHA class reduction or a 3·0 mL/kg per min or greater pVO2 increase without NYHA class worsening
“difference +19·4%, 95% CI 8·7 to 30·1; p=0·0005”
Results are quoted from each trial’s primary publication abstract (PubMed); the trial name links to the paper. Ratios below 1 favour the intervention.
The strongest 20 recommendations
For patients with HCM or at risk for HCM, shared decision-making is recommended in developing a plan of care (including, but not limited to, decisions regarding genetic evaluation, activity, lifestyle, and therapy choices) that includes a full disclosure of the risks, benefits, and anticipated outcomes of all options, as well the opportunity for the patient and caregivers to express their goals and concerns.
For patients with HCM and previous documented cardiac arrest or sustained VT, ICD placement is recommended (Figure 3, Table 8).
In patients with HCM who are receiving an ICD, either a single-chamber transvenous ICD or a subcutaneous ICD is recommended after a shared decision-making discussion that takes into consideration patient preferences, age, lifestyle, and potential need for pacing for bradycardia or VT termination.
In patients with HCM who are receiving a transvenous ICD, single-coil ICD leads are recommended in preference to dual-coil leads, if defibrillation threshold is deemed adequate.
For patients with obstructive HCM who have persistent symptoms attributable to LVOTO despite beta blockers or nondihydropyridine calcium channel blockers, adding a myosin inhibitor (adult patients only), or disopyramide (in combination with an atrioventricular nodal blocking agent), or SRT performed at experienced centers, is recommended.
In patients with obstructive HCM and symptoms attributable to LVOTO, nonvasodilating beta blockers, titrated to effectiveness or maximally tolerated doses, are recommended.
In patients with obstructive HCM who remain symptomatic despite GDMT, SRT in eligible patients, performed at experienced HCM centers, is recommended for relieving LVOTO (Tables 4 and 5).
In symptomatic patients with obstructive HCM who have associated cardiac disease requiring surgical treatment (eg, associated anomalous papillary muscle, markedly elongated anterior mitral leaflet, intrinsic mitral valve disease, multivessel CAD, valvular aortic stenosis), surgical myectomy, performed at experienced HCM centers, is recommended (Tables 4 and 5).
In patients with HCM who develop persistent systolic dysfunction (LVEF <50%), cardiac myosin inhibitors should be discontinued.
For patients with HCM, mild- to moderate-intensity recreational exercise is beneficial to improve cardiorespiratory fitness, physical functioning, and quality of life, and for overall health in keeping with physical activity guidelines for the general population.
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.
In symptomatic patients with HCM and a resting or provoked 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.
Non-vasodilating beta-blockers, titrated to maximum tolerated dose, are recommended as first-line therapy to improve symptoms in patients with resting or provoked LVOTO.
Verapamil or diltiazem, titrated to maximum tolerated dose, are recommended to improve symptoms in symptomatic patients with resting or provoked LVOTO who are intolerant or have contraindications to beta-blockers.
Disopyramide, 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 provoked LVOTO.
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.
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.
The HCM Risk-SCD calculator is recommended as a method of estimating risk of sudden death at 5 years in patients aged ≥16 years for primary prevention.
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.
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.