Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

asymptomatic

1st line – observation/monitoring

Back
ONGOING
|
asymptomatic
1st line – 

observation/monitoring

Asymptomatic patients (genotype-positive/phenotype-negative) require ongoing monitoring for development of clinical hypertrophic cardiomyopathy.

US guidelines recommend serial clinical assessment, ECG, and cardiac imaging every 1-2 years in children and adolescents and every 3-5 years in adults.[2]

Genotype-positive/phenotype-negative patients are not considered at high risk of sudden cardiac death and implantable cardioverter-defibrillator placement is not required.[2]

Plus – lifestyle and risk factor modification

Back
ONGOING
|
asymptomatic
Plus – 

lifestyle and risk factor modification

Treatment recommended for ALL patients in selected patient group

​Primary prevention of cardiovascular disease (as per published guidelines) and management of risk factors is recommended in all asymptomatic patients.[1][2][101]​​​

Comorbidities such as hypertension, obesity, diabetes, hyperlipidaemia, and sleep-disordered breathing, and lifestyle factors, such as smoking and inactivity, may increase symptom burden and risk of heart failure and/or atrial fibrillation.[2][100]​​​

US guidelines suggest that weight loss interventions in patients with hypertrophic cardiomyopathy (HCM) and obesity have the potential to reduce symptoms and adverse outcomes.[2]

Assessment for sleep-disordered breathing is recommended by US guidelines, with referral to specialist if symptoms are present.[2] The guidelines suggest that management of obstructive sleep apnoea could reduce symptoms and arrhythmic complications in patients with HCM, but evidence is needed.

Participation in competitive sport of any intensity is reasonable in genotype-positive/phenotype-negative patients; they should be regularly assessed for change in clinical status.[2][65]

symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)

1st line – beta-blocker or non-dihydropyridine calcium-channel blocker

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
1st line – 

beta-blocker or non-dihydropyridine calcium-channel blocker

Helps alleviate obstruction.[1][2]​​ Non-vasodilating beta-blockers are considered first-line therapy for symptomatic hypertrophic cardiomyopathy (HCM) due to LVOTO.[1][2]​​ In standard doses, they are usually well tolerated. A beta-blocker may be of benefit in patients with HCM and symptoms suggestive of ischaemia.

Non-dihydropyridine calcium-channel blockers (diltiazem, verapamil) are alternatives to beta-blockers.[1][2]​ Verapamil and diltiazem have vasodilating properties as well as negative inotropic and chronotropic effects.[2] Short-term oral administration may increase exercise capacity, improve symptoms, and normalise or improve LV diastolic filling without altering systolic function.[1] Verapamil can be used when beta-blockers are contraindicated or ineffective, but it is potentially harmful in patients with obstructive HCM and severe dyspnoea at rest, hypotension, and very high resting gradients (e.g., >100 mmHg), and infants <6 weeks.[2] Verapamil has been reported to cause death in a few patients with HCM and severe LVOTO or elevated pulmonary arterial pressure as it may provoke pulmonary oedema.[1] It should therefore be used with caution in these patients.[1] Diltiazem should be considered in patients who are intolerant or have contraindications to beta-blockers and verapamil.[1] ​

Tachyphylaxis to drugs is common, and the dosage must be adjusted over time. In the absence of many randomised controlled trials, pharmacological therapy is mostly administered on an empirical basis to improve functional capacity and reduce symptoms.[1]

Primary options

atenolol: 50-100 mg orally once daily

OR

propranolol: 80-160 mg orally (sustained-release) once daily

OR

metoprolol: 100-450 mg/day orally (immediate-release) given in 2-3 divided doses

OR

nadolol: 40 mg orally once daily initially, increase by 40-80 mg/day increments every 3-7 days according to response, maximum 240 mg/day

Secondary options

verapamil: consult specialist for guidance on dose

OR

diltiazem: consult specialist for guidance on dose

Plus – lifestyle and risk factor modification

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
Plus – 

lifestyle and risk factor modification

Treatment recommended for ALL patients in selected patient group

​Comorbidities such as hypertension, obesity, diabetes, hyperlipidaemia, and sleep-disordered breathing, and lifestyle factors, such as smoking and inactivity, may increase symptom burden and risk of heart failure and/or atrial fibrillation.[2][100]​​ Primary prevention of cardiovascular disease (as per published guidelines) and management of risk factors is recommended in all patients.[1][2][101]

US guidelines suggest that weight loss interventions in patients with hypertrophic cardiomyopathy (HCM) and obesity have the potential to reduce symptoms and adverse outcomes.[2]

Assessment for sleep-disordered breathing is recommended by US guidelines, with referral to specialist if symptoms are present.[2] The guidelines suggest that management of obstructive sleep apnoea could reduce symptoms and arrhythmic complications in patients with HCM, but evidence is needed.

Consensus recommendations have previously restricted all athletes with HCM from all competitive sports; however, US and European guidelines now advise that participation in high-intensity exercise/competitive sports may be considered for some individuals after comprehensive evaluation and shared discussion.[2]​​[65]

Consider – implantable cardioverter-defibrillator (ICD)

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
Consider – 

implantable cardioverter-defibrillator (ICD)

Additional treatment recommended for SOME patients in selected patient group

Patients should be considered for an ICD if at any stage during therapy they are found to be at a higher risk level, or develop new symptomatic or important asymptomatic ventricular arrhythmias.

Guidelines recommend ICD placement for patients with hypertrophic cardiomyopathy (HCM) and previous documented cardiac arrest or sustained ventricular tachycardia.[1][2]​​​ Comprehensive sudden cardiac death risk stratification is recommended in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1][2]

A single marker of high risk for sudden cardiac arrest may also be sufficient to consider ICD placement in selected patients.[1][2][68]​ Patients in whom this would apply include those with one or more first-degree or close relatives 50 years or less with sudden death presumably caused by HCM, patients with a maximum LV wall thickness greater than or equal to 30 mm, patients with one or more recent, unexplained episodes of syncope suspected to be arrhythmic, LV apical aneurysm, LV systolic dysfunction with ejection fraction <50%, and late gadolinium enhancement >15% on cardiac magnetic resonance imaging.[1][2]

No randomised controlled trials studying the effect of ICD placement have been performed in patients with HCM, although there is evidence from observational studies.[2][68]

Complications following ICD placement have been reported to occur at a rate of 3.4% per year.[69] Contact sports should be avoided after ICD implant.[70] Patients and carers should be fully informed and participate in decision-making regarding ICD placement.[2]​ They should be counselled on the risk of inappropriate shocks, implant complications, and the social, occupational, and driving implications of the device. Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good-quality survival >1 year.[1]

Consider – mavacamten

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ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
Consider – 

mavacamten

Additional treatment recommended for SOME patients in selected patient group

Consider addition of mavacamten (a cardiac myosin inhibitor) for patients with LVOTO and persistent severe symptoms despite therapy with beta-blockers or non-dihydropyridine calcium-channel blockers.[1][2]​​

In the US, mavacamten is available through a Risk Evaluation and Mitigation Strategy (REMS) programme, designed to monitor patients periodically with echocardiograms for early detection of systolic dysfunction and to screen for drug interactions prior to each prescription.[76]

European guidelines stipulate that in the absence of evidence to the contrary, mavacamten should not be used with disopyramide, but may be coadministered with beta-blockers or non-dihydropyridine calcium-channel blockers.[1] UK guidelines differ, stating that it can be added‑on to individually optimised standard care that includes beta‑blockers, non-dihydropyridine calcium-channel blockers, or disopyramide, unless these are contraindicated.[75]

In patients with contraindications or known sensitivity to beta-blockers, non-dihydropyridine calcium-channel blockers, and disopyramide, mavacamten may be considered as monotherapy.[1]

Up-titration of drug treatment to a maximum tolerated dose should be monitored in accordance with licensed recommendations using echocardiographic surveillance of LV ejection fraction.[1]

Primary options

mavacamten: 2.5 to 15 mg orally once daily

More

Consider – disopyramide

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ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
Consider – 

disopyramide

Additional treatment recommended for SOME patients in selected patient group

​Consider addition of disopyramide (a negative inotrope and a type Ia anti-arrhythmic agent) for patients with LVOTO and persistent severe symptoms despite therapy with beta-blockers or non-dihydropyridine calcium-channel blockers.[1][2]

US guidelines recommend that disopyramide is used in combination with an agent that has atrioventricular nodal blocking properties (i.e., beta-blocker or non-dihydropyridine calcium-channel blocker), as it may enhance conduction through the atrioventricular node, which could lead to rapid conduction increase the ventricular rate in patients with atrial fibrillation.[2]

European guidelines also advise that disopyramide may be considered as monotherapy in patients who are intolerant of or have contraindications to beta-blockers and non-dihydropyridine calcium-channel blockers.[1]​​

Dose-limiting anticholinergic side effects include dry eyes and mouth, urinary hesitancy or retention, and constipation. The ECG QT interval should be monitored for prolongation.[1]

Primary options

disopyramide: 200-250 mg orally (controlled-release) twice daily initially, increase gradually according to response, maximum 600 mg/day

Consider – diuretic

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
Consider – 

diuretic

Additional treatment recommended for SOME patients in selected patient group

​Low-dose diuretics (e.g., furosemide, hydrochlorothiazide) may be used with caution in patients who have persistent dyspnoea with clinical evidence of volume overload and high left-sided filling pressures despite other hypertrophic cardiomyopathy guideline-directed medical therapy. Aggressive diuresis can worsen LVOTO.[1][2]

Primary options

furosemide: 20-80 mg orally initially, may increase by 20-40 mg every 6-8 hours according to response, maximum 600 mg/day

OR

hydrochlorothiazide: 25 mg orally once or twice daily initially, increase gradually according to response, maximum 200 mg/day

2nd line – septal reduction therapy

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
2nd line – 

septal reduction therapy

If severe symptoms persist despite optimal medical therapy, consideration should be given to surgical myectomy, which reduces septal mass, thereby relieving obstruction.[2] Patients are generally considered to be eligible for septal reduction therapy when they have severe dyspnoea or chest pain (New York Heart Association class III or IV) or exertional recurrent syncope, resting or provocable outflow tract gradient of ≥50 mmHg, and appropriate anatomy.[1][2] Myectomy abolishes or substantially reduces LV outflow tract gradients in over 90% of cases, reduces systolic anterior motion-related mitral regurgitation, and improves exercise capacity and symptoms. Long-term symptomatic benefit is achieved in >80% of patients, with a long-term survival comparable to that of the general population.

Preoperative determinants of a good long-term outcome are: age <50 years; left atrial size <46 mm; absence of AF; and male sex.[1] Older age and increased severity of comorbidities are predictive of poor surgical outcomes.[83]

The rate of postoperative complications is estimated at 5.9% in most experienced centres. The most common complications are complete heart block in patients without previous conduction abnormality (3% to 10%), left bundle branch block (40% to 56%), and ventricular septal defect (1%).[83][84]

Alcohol septal ablation (ASA) may be performed as an alternative to surgical myectomy. ASA involves the delivery of alcohol into a target septal perforator branch of the left anterior descending coronary artery, for the purpose of producing a myocardial infarction and reducing septal thickness.[1] Septal remodelling and relief of obstruction after ASA occurs over several months, resulting in a smaller reduction in resting gradient compared with surgical myectomy, but a similar reduction in patient symptoms.[85][86] Complications include ventricular arrhythmias (2.2%), coronary dissection (1.8%), and complete heart block (>10%) necessitating permanent pacemaker placement.[87] ​ There is an increased need for permanent pacemaker implantation post-procedure compared with surgical myectomy.[88] Mortality from all-cause or sudden cardiac death is low after ASA.[89]

While data comparing the later outcomes of ASA and surgical myectomy are lacking, a retrospective, observational study compared long-term mortality of patients with obstructive hypertrophic cardiomyopathy following both procedures. It concluded that ASA was associated with increased long-term all-cause mortality compared with septal myectomy. This finding remained after adjustment for confounding factors (patients undergoing ASA tend to be older with more comorbidities and reduced septal thickness, compared with patients undergoing septal myectomy), but may still be influenced by unmeasured confounders.[90]

Following septal reduction therapy, medical therapy may be continued or adjusted, depending on the individual patient.

Consider – surgery or percutaneous coronary intervention

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
|
with ischaemia
Consider – 

surgery or percutaneous coronary intervention

Additional treatment recommended for SOME patients in selected patient group

​Patients may develop symptoms or signs of ischaemia. Ischaemia in hypertrophic cardiomyopathy (HCM) is multifactorial and thus not easily treated. Decreasing myocardial oxygen demand with negative inotropic and chronotropic agents may prove beneficial.

Aetiology of the ischaemia should be identified (i.e., increased LV outflow tract obstruction, coronary artery disease, or myocardial bridging).

For patients with anomalous coronary artery, surgical unroofing of myocardial bridge (tunnelling of coronary arteries into heart muscle) has been reported to yield symptomatic improvement in select patients, but data are limited.[30][91]​​​​​​ Moreover, myocardial bridging is frequently identified in HCM and has not been conclusively linked to sudden cardiac death.[92][93] Therefore, the risks of the procedure need to be considered when advising surgical intervention.

For patients with concomitant epicardial coronary artery disease consider PCI or CABG. See Chronic coronary disease.

Plus – management of arrhythmia

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
|
with ventricular arrhythmia
Plus – 

management of arrhythmia

Treatment recommended for ALL patients in selected patient group

Implantation of an implantable cardioverter-defibrillator (ICD) is recommended for secondary prevention in patients with hypertrophic cardiomyopathy (HCM) 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. It should also be considered in patients presenting with haemodynamically tolerated VT, in the absence of reversible causes.[1] In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2]

Although data are lacking, anti-arrhythmics such as beta-blockers (e.g., sotalol) and amiodarone should be considered for patients with recurrent, symptomatic ventricular arrhythmia, or recurrent ICD shocks.[1]

Catheter ablation in specialised centres may be considered in select patients with recurrent, symptomatic sustained monomorphic VT (SMVT), or recurrent ICD shocks for SMVT, in whom anti-arrhythmics are ineffective, contraindicated, or not tolerated.[2][94]

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96][70]

Plus – anticoagulation plus management of arrhythmia

Back
ONGOING
|
symptomatic: left ventricular outflow tract obstruction (LVOTO) with preserved systolic function (ejection fraction ≥50%)
|
with atrial fibrillation
Plus – 

anticoagulation plus management of arrhythmia

Treatment recommended for ALL patients in selected patient group

​Atrial fibrillation (AF) is often poorly tolerated in patients with hypertrophic cardiomyopathy (HCM).[2] As a result, an aggressive strategy for maintaining sinus rhythm may be warranted. Paroxysmal or persistent AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, and occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms. Management of AF is as per patients without HCM. However, digoxin is not typically used for atrial rate control if the patient has significant hypertrophy, as there is a theoretical concern that it could exacerbate LVOTO due to a positive inotropic effect.[2] In addition, traditional stroke risk scoring systems used in the general population, such as CHA2DS2-VASc (congestive heart failure or left ventricular dysfunction, hypertension, age ≥75 [doubled], diabetes, stroke [doubled]-vascular disease, ages 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2][95] ​ For this reason, although there are no randomised controlled trials evaluating the role of anticoagulation in patients with HCM, given the high incidence of stroke, prophylactic anticoagulation is recommended in all patients with HCM and AF (if no contraindication).[1] A direct oral anticoagulant (DOAC) is recommended first-line option, and a vitamin K antagonist (usually warfarin) second-line option.[1][2][95] See New-onset atrial fibrillation and Chronic atrial fibrillation.

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96][70]​

symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)

1st line – beta-blocker or non-dihydropyridine calcium-channel blocker

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
1st line – 

beta-blocker or non-dihydropyridine calcium-channel blocker

Patients with non-obstructive hypertrophic cardiomyopathy (HCM) commonly have dyspnoea and exertional angina.[2] Symptoms are related to diastolic dysfunction, with impaired filling resulting in reduced output and pulmonary congestion. Presence of obstructive coronary artery disease should be excluded.[2] Patients are more symptomatic when heart rate is higher, as diastolic filling is further compromised; a negative chronotropic agent may therefore be beneficial in this setting.[4]

First-line options for patients with non-obstructive HCM and preserved ejection fraction are beta-blockers or non-dihydropyridine calcium-channel blockers (diltiazem, verapamil).[1][2] 

Non-dihydropyridine calcium-channel blockers are thought to improve symptoms, secondary to the beneficial effect on myocardial relaxation and ventricular filling. They are also negative inotropes, which may aid in relief of symptoms.

Beta-blockers may be used, as they may improve diastolic filling due to their negative chronotropic effect.

Primary options

atenolol: 50-100 mg orally once daily

OR

propranolol: 80-160 mg orally (sustained-release) once daily

OR

metoprolol: 100-450 mg/day orally (immediate-release) given in 2-3 divided doses

OR

nadolol: 40 mg orally once daily initially, increase by 40-80 mg/day increments every 3-7 days according to response, maximum 240 mg/day

OR

verapamil: consult specialist for guidance on dose

OR

diltiazem: consult specialist for guidance on dose

Plus – lifestyle and risk factor modification

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
Plus – 

lifestyle and risk factor modification

Treatment recommended for ALL patients in selected patient group

​Comorbidities such as hypertension, obesity, diabetes, hyperlipidaemia, and sleep-disordered breathing, and lifestyle factors, such as smoking and inactivity, may increase symptom burden and risk of heart failure and/or atrial fibrillation.[2][100]​​​ Primary prevention of cardiovascular disease (as per published guidelines) and management of risk factors is recommended in all patients.[1][2][101]

US guidelines suggest that weight loss interventions in patients with hypertrophic cardiomyopathy (HCM) and obesity have the potential to reduce symptoms and adverse outcomes.[2]

Assessment for sleep-disordered breathing is recommended by US guidelines, with referral to specialist if symptoms are present.[2] The guidelines suggest that management of obstructive sleep apnoea could reduce symptoms and arrhythmic complications in patients with HCM, but evidence is needed.

Consensus recommendations have previously restricted all athletes with HCM from all competitive sports; however, US and European guidelines now advise that participation in high-intensity exercise/competitive sports may be considered for some individuals after comprehensive evaluation and shared discussion.[2]​​[65]

Consider – diuretic

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
Consider – 

diuretic

Additional treatment recommended for SOME patients in selected patient group

​Oral diuretics (e.g., furosemide, hydrochlorothiazide) may be added to treatment in patients who have persistent dyspnoea despite beta-blockers or non-dihydropyridine calcium-channel blockers or volume overload; diuretics should be used with caution to avoid hypotension or hypovolemia.[2]

Primary options

furosemide: 20-80 mg orally initially, may increase by 20-40 mg every 6-8 hours according to response, maximum 600 mg/day

OR

hydrochlorothiazide: 25 mg orally once or twice daily initially, increase gradually according to response, maximum 200 mg/day

Consider – implantable cardioverter-defibrillator (ICD)

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
Consider – 

implantable cardioverter-defibrillator (ICD)

Additional treatment recommended for SOME patients in selected patient group

Patients should be considered for an ICD if at any stage during therapy they are found to be at a higher risk level, or develop new symptomatic or important asymptomatic ventricular arrhythmias.

Guidelines recommend ICD placement for patients with hypertrophic cardiomyopathy (HCM) and previous documented cardiac arrest or sustained ventricular tachycardia.[1][2]​​​​ Comprehensive sudden cardiac death risk stratification is recommended in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1][2]

A single marker of high risk for sudden cardiac arrest may also be sufficient to consider ICD placement in selected patients.[1][2]​​​​​​​​[68]​ Patients in whom this would apply include those with one or more first-degree or close relatives 50 years or less with sudden death presumably caused by HCM, patients with a maximum LV wall thickness greater than or equal to 30 mm, patients with one or more recent, unexplained episodes of syncope suspected to be arrhythmic, LV apical aneurysm, LV systolic dysfunction with ejection fraction <50%, and late gadolinium enhancement >15% on cardiac magnetic resonance imaging.[1][2]

No randomised controlled trials studying the effect of ICD placement have been performed in patients with HCM, although there is evidence from observational studies.[2][68]

Complications following ICD placement have been reported to occur at a rate of 3.4% per year.[69] Contact sports should be avoided after ICD implant.[70] Patients and carers should be fully informed and participate in decision-making regarding ICD placement.[2] They should be counselled on the risk of inappropriate shocks, implant complications, and the social, occupational, and driving implications of the device. Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good-quality survival >1 year.[1]

Consider – antianginal therapy

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
|
with ischaemia
Consider – 

antianginal therapy

Additional treatment recommended for SOME patients in selected patient group

Oral nitrates can be used cautiously for relief of angina.[1] Ranolazine can be considered to improve symptoms in patients with angina-like chest pain and no evidence of left ventricular outflow tract obstruction, even in the absence of obstructive coronary artery disease.[1]

See Chronic coronary disease.

Consider – surgery or percutaneous coronary intervention

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
|
with ischaemia
Consider – 

surgery or percutaneous coronary intervention

Additional treatment recommended for SOME patients in selected patient group

​Patients may develop symptoms or signs of ischaemia. Ischaemia in hypertrophic cardiomyopathy (HCM) is multifactorial and thus not easily treated. Decreasing myocardial oxygen demand with negative inotropic and chronotropic agents may prove beneficial.

Aetiology of the ischaemia should be identified (i.e., increased LV outflow tract obstruction, coronary artery disease, or myocardial bridging).

For patients with anomalous coronary artery, surgical unroofing of myocardial bridge (tunnelling of coronary arteries into heart muscle) has been reported to yield symptomatic improvement in select patients, but data are limited.[30][91]​​​​​​​ Moreover, myocardial bridging is frequently identified in HCM and has not been conclusively linked to sudden cardiac death.[92][93]​ Therefore, the risks of the procedure need to be considered when advising surgical intervention.

For patients with concomitant epicardial coronary artery disease consider PCI or CABG. See Chronic coronary disease.

Plus – management of arrhythmia

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
|
with ventricular arrhythmia
Plus – 

management of arrhythmia

Treatment recommended for ALL patients in selected patient group

Implantation of an implantable cardioverter-defibrillator (ICD) is recommended for secondary prevention in patients with hypertrophic cardiomyopathy (HCM) 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. It should also be considered in patients presenting with haemodynamically tolerated VT, in the absence of reversible causes.[1] In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2]

Although data are lacking, anti-arrhythmics such as beta-blockers (e.g., sotalol) and amiodarone should be considered for patients with recurrent, symptomatic ventricular arrhythmia, or recurrent ICD shocks.[1]

Catheter ablation in specialised centres may be considered in select patients with recurrent, symptomatic sustained monomorphic VT (SMVT), or recurrent ICD shocks for SMVT, in whom anti-arrhythmics are ineffective, contraindicated, or not tolerated.[2][94]

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96][70]

Plus – anticoagulation plus management of arrhythmia

Back
ONGOING
|
symptomatic: non-obstructive with preserved systolic function (ejection fraction ≥50%)
|
with atrial fibrillation
Plus – 

anticoagulation plus management of arrhythmia

Treatment recommended for ALL patients in selected patient group

​Atrial fibrillation (AF) is often poorly tolerated in patients with hypertrophic cardiomyopathy (HCM).[2] As a result, an aggressive strategy for maintaining sinus rhythm may be warranted. Paroxysmal or persistent AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, and occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms. Management of AF is as per patients without HCM. However, digoxin is not typically used for atrial rate control if the patient has significant hypertrophy, as there is a theoretical concern that it could exacerbate LVOTO due to a positive inotropic effect.[2] In addition, traditional stroke risk scoring systems used in the general population, such as CHA2DS2-VASc (congestive heart failure or left ventricular dysfunction, hypertension, age ≥75 [doubled], diabetes, stroke [doubled]-vascular disease, ages 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2][95]​​ ​ For this reason, although there are no randomised controlled trials evaluating the role of anticoagulation in patients with HCM, given the high incidence of stroke, prophylactic anticoagulation is recommended in all patients with HCM and AF (if no contraindication).[1] A direct oral anticoagulant (DOAC) is recommended first-line option, and a vitamin K antagonist (usually warfarin) second-line option.[1][2][95]​​ See New-onset atrial fibrillation and Chronic atrial fibrillation.

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96][70]​

symptomatic: with systolic dysfunction (ejection fraction <50%)

1st line – guideline-directed medical therapy for heart failure with reduced ejection fraction

Back
ONGOING
|
symptomatic: with systolic dysfunction (ejection fraction <50%)
1st line – 

guideline-directed medical therapy for heart failure with reduced ejection fraction

The average duration from onset of symptoms to end-stage disease is 14 years.[97] Systolic function deteriorates, and the left ventricle remodels and becomes dilated. The mechanism of end-stage hypertrophic cardiomyopathy (HCM) is likely to be diffuse ischaemic injury. Risk factors for end-stage disease include younger age at diagnosis, more severe symptoms, larger left ventricular cavity size, and family history of end-stage disease. Mortality is high once this complication develops, with mean time to death or cardiac transplantation of 2.7 ± 2.1 years.[97]

Patients with systolic dysfunction with ejection fraction <50% are treated with guideline-directed medical therapy for heart failure with reduced ejection fraction.[2][98]

Diuretics should be used cautiously in these patients compared with patients with other causes of heart failure, due to possible impairment in preload.

Reduced ejection fraction is uncommon in patients with HCM, and the patient should therefore be evaluated for other causes of systolic dysfunction.[2]

If the patient is receiving mavacamten and develops left ventricular ejection fraction <50%, it should be interrupted or discontinued. Previously indicated negative inotropic agents (verapamil, diltiazem, or disopyramide) should also be discontinued.[2] See Heart failure with reduced ejection fraction.

Plus – lifestyle and risk factor modification

Back
ONGOING
|
symptomatic: with systolic dysfunction (ejection fraction <50%)
Plus – 

lifestyle and risk factor modification

Treatment recommended for ALL patients in selected patient group

​Comorbidities such as hypertension, obesity, diabetes, hyperlipidaemia, and sleep-disordered breathing, and lifestyle factors, such as smoking and inactivity, may increase symptom burden and risk of heart failure and/or atrial fibrillation.[2][100]​​​ Primary prevention of cardiovascular disease (as per published guidelines) and management of risk factors is recommended in all patients.[1][2][101]

US guidelines suggest that weight loss interventions in patients with hypertrophic cardiomyopathy (HCM) and obesity have the potential to reduce symptoms and adverse outcomes.[2]

Assessment for sleep-disordered breathing is recommended by US guidelines, with referral to specialist if symptoms are present.[2] The guidelines suggest that management of obstructive sleep apnoea could reduce symptoms and arrhythmic complications in patients with HCM, but evidence is needed.

Patients should refrain from high-intensity athletics.

Consider – implantable cardioverter-defibrillator (ICD)

Back
ONGOING
|
symptomatic: with systolic dysfunction (ejection fraction <50%)
Consider – 

implantable cardioverter-defibrillator (ICD)

Additional treatment recommended for SOME patients in selected patient group

Patients should be considered for an ICD if at any stage during therapy they are found to be at a higher risk level, or develop new symptomatic or important asymptomatic ventricular arrhythmias.

Guidelines recommend ICD placement for patients with hypertrophic cardiomyopathy (HCM) and previous documented cardiac arrest or sustained ventricular tachycardia.[1][2]​​​​ Comprehensive sudden cardiac death risk stratification is recommended in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1][2]

A single marker of high risk for sudden cardiac arrest may also be sufficient to consider ICD placement in selected patients.[1][2]​​​​​​​​[68]​ Patients in whom this would apply include those with one or more first-degree or close relatives 50 years or less with sudden death presumably caused by HCM, patients with a maximum LV wall thickness greater than or equal to 30 mm, patients with one or more recent, unexplained episodes of syncope suspected to be arrhythmic, LV apical aneurysm, LV systolic dysfunction with ejection fraction <50%, and extensive late gadolinium enhancement >15% on cardiac magnetic resonance imaging.[1][2]

No randomised controlled trials studying the effect of ICD placement have been performed in patients with HCM, although there is evidence from observational studies.[2][68]

Complications following ICD placement have been reported to occur at a rate of 3.4% per year.[69] Contact sports should be avoided after ICD implant.[70] Patients and carers should be fully informed and participate in decision-making regarding ICD placement.[2] They should be counselled on the risk of inappropriate shocks, implant complications, and the social, occupational, and driving implications of the device. Implantation of a cardioverter defibrillator is only recommended in patients who have an expectation of good-quality survival >1 year.[1]

Consider – cardiac resynchronisation therapy (CRT)

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symptomatic: with systolic dysfunction (ejection fraction <50%)
Consider – 

cardiac resynchronisation therapy (CRT)

Additional treatment recommended for SOME patients in selected patient group

​US guidelines note that in patients with New York Heart Association functional class II to class IV symptoms despite guideline-directed medical therapy, and left bundle branch block, CRT can be beneficial to improve symptoms, reduce HF hospitalisations, and increase survival in patients. The benefit in patients with hypertrophic cardiomyopathy is not established, but use of CRT may be considered in select patients.[2]

2nd line – evaluation for heart transplant and/or mechanical circulatory support

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symptomatic: with systolic dysfunction (ejection fraction <50%)
2nd line – 

evaluation for heart transplant and/or mechanical circulatory support

If patients remain refractory to medical therapy, they should be referred for consideration for heart transplant.[2][98]

Heart transplants have been shown to improve survival and quality of life for patients with end-stage heart failure secondary to hypertrophic cardiomyopathy.​[98]​ Presence of comorbidities, caretaker status, and goals of care should all be taken into account when considering patient eligibility for transplant.​[98]

Left ventricular assist device therapy may be considered as a bridge to transplantation.[2]

Consider – antianginal therapy

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symptomatic: with systolic dysfunction (ejection fraction <50%)
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with ischaemia
Consider – 

antianginal therapy

Additional treatment recommended for SOME patients in selected patient group

Oral nitrates can be used cautiously for relief of angina.[1] ​Ranolazine can be considered to improve symptoms in patients with angina-like chest pain and no evidence of left ventricular outflow tract obstruction, even in the absence of obstructive coronary artery disease.[1]

See Chronic coronary disease.

Consider – surgery or percutaneous coronary intervention

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symptomatic: with systolic dysfunction (ejection fraction <50%)
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with ischaemia
Consider – 

surgery or percutaneous coronary intervention

Additional treatment recommended for SOME patients in selected patient group

Patients may develop symptoms or signs of ischaemia. Ischaemia in hypertrophic cardiomyopathy (HCM) is multifactorial and thus not easily treated. Decreasing myocardial oxygen demand with negative inotropic and chronotropic agents may prove beneficial.

Aetiology of the ischaemia should be identified (i.e., increased LV outflow tract obstruction, coronary artery disease, or myocardial bridging).

For patients with anomalous coronary artery, surgical unroofing of myocardial bridge (tunnelling of coronary arteries into heart muscle) has been reported to yield symptomatic improvement in select patients, but data are limited.[30][91]​​​​​​​ Moreover, myocardial bridging is frequently identified in HCM and has not been conclusively linked to sudden cardiac death.[92][93]​ Therefore, the risks of the procedure need to be considered when advising surgical intervention.

For patients with concomitant epicardial coronary artery disease consider PCI or CABG. See Chronic coronary disease.

Plus – management of arrhythmia

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symptomatic: with systolic dysfunction (ejection fraction <50%)
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with ventricular arrhythmia
Plus – 

management of arrhythmia

Treatment recommended for ALL patients in selected patient group

​As described above, implantation of an implantable cardioverter-defibrillator (ICD) is recommended for secondary prevention in patients with hypertrophic cardiomyopathy (HCM) 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. It should also be considered in patients presenting with haemodynamically tolerated VT, in the absence of reversible causes.[1] In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2]

Although data are lacking, anti-arrhythmics such as beta-blockers (e.g., sotalol) and amiodarone should be considered for patients with recurrent, symptomatic ventricular arrhythmia, or recurrent ICD shocks.[1]

Catheter ablation in specialised centres may be considered in select patients with recurrent, symptomatic sustained monomorphic VT (SMVT), or recurrent ICD shocks for SMVT, in whom anti-arrhythmics are ineffective, contraindicated, or not tolerated.[2][94]

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96][70]​

Plus – anticoagulation plus management of arrhythmia

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symptomatic: with systolic dysfunction (ejection fraction <50%)
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with atrial fibrillation
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anticoagulation plus management of arrhythmia

Treatment recommended for ALL patients in selected patient group

Atrial fibrillation (AF) is often poorly tolerated in patients with hypertrophic cardiomyopathy (HCM).[2] As a result, an aggressive strategy for maintaining sinus rhythm may be warranted. Paroxysmal or persistent AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, and occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms. Management of AF is as per patients without HCM. However, digoxin is not typically used for atrial rate control if the patient has significant hypertrophy, as there is a theoretical concern that it could exacerbate LV outflow tract obstruction due to a positive inotropic effect.[2] In addition, traditional stroke risk scoring systems used in the general population, such as CHA2DS2-VASc (congestive heart failure or left ventricular dysfunction, hypertension, age ≥75 [doubled], diabetes, stroke [doubled]-vascular disease, ages 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2][95]​​​ ​ For this reason, although there are no randomised controlled trials evaluating the role of anticoagulation in patients with HCM, given the high incidence of stroke, prophylactic anticoagulation is recommended in all patients with HCM and AF (if no contraindication).[1] A direct oral anticoagulant (DOAC) is recommended first-line option, and a vitamin K antagonist (usually warfarin) second-line option.[1][2][95]​​​ See New-onset atrial fibrillation and Chronic atrial fibrillation.

Indications for permanent pacemaker implantation: permanent pacemaker implantation is indicated in patients with symptomatic sinus node dysfunction and HCM, and in patients with high-grade atrioventricular block who are symptomatic, or who have arrhythmias such as AF or ventricular arrhythmias that are worsened by bradycardia or prolonged pauses.[96]

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