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

INITIAL

asymptomatic

1st line – implantable cardioverter-defibrillator (ICD) + avoidance of high-intensity athletics

Back
INITIAL
|
asymptomatic
|
at high risk of sudden death
1st line – 

implantable cardioverter-defibrillator (ICD) + avoidance of high-intensity athletics

Guidelines recommend ICD placement for patients with hypertrophic cardiomyopathy (HCM) and previous documented cardiac arrest or sustained ventricular arrhythmia causing syncope or haemodynamic compromise in the absence of reversible causes.[1][2]​​ Routine diagnostic testing to evaluate the risk of sudden death is recommended, regardless of symptom status. European guidelines recommend comprehensive sudden cardiac death risk stratification in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.​​[1]

Prophylactic ICD placement should also be considered in selected asymptomatic patients including those with one or more first-degree or close relatives 50 years of age or less with sudden death presumably caused by HCM, patients with a maximum left ventricular (LV) wall thickness greater than or equal to 30 mm, patients with one or more recent 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]​ Sports with high likelihood of bodily collision should also 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]

1st line – observation

Back
INITIAL
|
asymptomatic
|
not at high risk of sudden death
1st line – 

observation

If the patient is not considered at high risk of sudden death, implantable cardioverter-defibrillator placement is not required. Patients in this category who are asymptomatic should be closely observed for the development of hypertrophic cardiomyopathy. Routine diagnostic testing to evaluate the risk of sudden death is recommended, regardless of symptom status. European guidelines recommend comprehensive sudden cardiac death risk stratification in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1]

US and European guidelines now advise that for those who are genotype-positive and phenotype-negative (asymptomatic without evidence of left ventricular hypertrophy on cardiac imaging), participation in competitive sport of any intensity is reasonable.[2][65]

Patients should be regularly assessed for change in clinical status.

ACUTE

symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function

1st line – negative inotropic and chronotropic agents

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
1st line – 

negative inotropic and chronotropic agents

A symptomatic patient with resting or provocable LVOTO is initially treated with negative inotropic or chronotropic therapy to help alleviate obstruction. Tachyphylaxis to medication is common, and medication 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]

Beta-blockers are beneficial due to their negative inotropic and chronotropic properties. Non-vasodilating beta-blockers are considered first-line therapy for symptomatic HCM due to LVOTO. In standard doses, they are usually well tolerated. Reported side effects include fatigue, impotence, sleep disturbances, and bradycardia. Substantial experience suggests that beta-blockers can mitigate symptoms and reduce outflow tract obstruction in those patients with LVOTO occurring with exercise. There is little evidence to suggest a beneficial effect on resting outflow tract gradients; however, one small RCT found that metoprolol reduced LVOTO obstruction at rest and during exercise, provided symptom relief, and improved quality of life in patients with obstructive HCM. Maximum exercise capacity remained unchanged.[71][72]​​ Beta-blocker therapy may also be of benefit in patients with HCM and symptoms suggestive of ischaemia. Patients with co-existing ischaemia should have their beta-blocker dosing optimised.

Non-dihydropyridine calcium-channel blockers (diltiazem, verapamil) are used for relief of symptoms, including those with a component of chest pain.[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. Diltiazem should be considered in patients who are intolerant or have contraindications to beta-blockers and verapamil.[1] Caution in administration of non-dihydropyridine calcium-channel blockers is advised, because if the vasodilatory properties predominate, outflow tract obstruction may be increased, resulting in pulmonary oedema and shock. They should be avoided if a patient has pronounced obstruction or elevated pulmonary arterial pressure.[1]

Disopyramide is 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, adding disopyramide is recommended.[2] Disopyramide is often used in combination with an agent that has atrioventricular nodal blocking properties, as it may increase the ventricular rate in patients with atrial fibrillation. It may be considered as monotherapy in patients who are intolerant to or have contraindications to beta-blockers and verapamil or diltiazem.[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] 

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]

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

Tertiary 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

-- AND --

disopyramide: <50 kg body weight: 200 mg orally (controlled-release) twice daily; >50 kg body weight: 300 mg orally (controlled-release) twice daily

Consider – mavacamten

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
Consider – 

mavacamten

Additional treatment recommended for SOME patients in selected patient group

Mavacamten is a cardiac myosin inhibitor approved for the treatment of adults with symptomatic New York Heart Association class II-III obstructive hypertrophic cardiomyopathy (HCM) to improve functional capacity and symptoms.[1][74][75]​ It works by inhibiting cardiac myosin adenosine triphosphatase (ATPase), thus reducing actin-myosin cross-bridge formation; this reduces contractility and improves myocardial dynamics.[1]

While mavacamten is approved for this indication in the US, the most recent guidelines from the American Heart Association and American College of Cardiology do not include a cardiac myosin inhibitor in the treatment cascade.[2]​ Mavacamten is currently available in the US 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 and UK guidelines now recommend mavacamten as a second-line treatment for patients with HCM and LVOTO.[1][75]​ It should be considered when optimal medical therapy with beta-blockers, calcium-channel blockers, and/or disopyramide is ineffective or poorly tolerated. 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 calcium-channel blockers.[1] UK guidelines differ, stating that it can be added‑on to individually optimised standard care that includes beta‑blockers, calcium-channel blockers, or disopyramide, unless these are contraindicated.[75]

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

Up-titration of medication 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 – implantable cardioverter-defibrillator (ICD)

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
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]​ Routine diagnostic testing to evaluate the risk of sudden death is recommended, regardless of symptom status. European guidelines recommend comprehensive sudden cardiac death risk stratification in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1]

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 of age 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 – management of arrhythmias

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
Consider – 

management of arrhythmias

Additional treatment recommended for SOME patients in selected patient group

Atrial arrhythmias, including atrial fibrillation (AF), are common, particularly in older patients with hypertrophic cardiomyopathy (HCM). Prevalence of AF among patients with HCM is estimated at 17% to 39%, with an annual incidence of 2.8% to 4.8%.[1] AF is often poorly tolerated in patients with HCM.[2] As a result, an aggressive strategy for maintaining sinus rhythm is warranted.

Paroxysmal and chronic AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms.[2]

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, aged 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2]​​​​[98]​​ 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 is recommended first-line option, and a vitamin K antagonist (e.g., warfarin) second-line option.[1][2][98]​​ See New-onset atrial fibrillation (Management) and  Chronic atrial fibrillation (Management)

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.[2]​

All patients with symptomatic ventricular arrhythmias or important asymptomatic ventricular arrhythmias should receive an implantable cardioverter-defibrillator (ICD).[1][2]​​ In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2] Although data are lacking, anti-arrhythmic drugs 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-arrhythmic drugs are ineffective, contraindicated, or not tolerated.[1][2]

Consider – surgical coronary artery unroofing (selected cases)

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
Consider – 

surgical coronary artery unroofing (selected cases)

Additional treatment recommended for SOME patients in selected patient group

In the presence of ischaemia due to a myocardial bridge (a band of heart muscle lying on top of a coronary artery) surgical coronary artery unroofing of the myocardial bridge has been shown to lead to resolution of ischaemia and of ventricular arrhythmias in some patients, and may lessen the incidence of sudden death.[30][94]​​ However, the evidence for the benefit of this procedure is limited and risks of the procedure need to be considered.[96]

2nd line – myectomy/alcohol septal ablation/dual-chamber pacing

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
2nd line – 

myectomy/alcohol septal ablation/dual-chamber pacing

Surgical myectomy (septal reduction therapy) is the optimal procedure for relief of drug-refractory LVOTO in patients with hypertrophic cardiomyopathy (HCM).[20][30]​​​​ Indicated if severe symptoms persist despite medical therapy, with a resting or provocable outflow tract gradient of ≥50 mmHg.[2]​ European guidelines specify that patients should be in New York Heart Association/Ross functional class III-IV, or be experiencing recurrent exertional syncope due to LVOTO, despite maximum tolerated medical therapy.[1]

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] Data from experienced centres suggest that institutions should aim for mortality rates of <1%.[2]

Alcohol septal ablation (ASA) is an alternative to surgery in adults with HCM. It 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]

Neither ASA or surgical myectomy have been conclusively shown to affect the incidence of sudden death. While randomised controlled trial data comparing the later outcomes of both procedures are lacking, a retrospective, observational study compared long-term mortality of patients with obstructive HCM following septal myectomy or ASA. 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 to patients undergoing septal myectomy), but may still be influenced by unmeasured confounders.[90] Results of each procedure are likely to be dependent on the technical expertise of the centre where the procedure is to be performed.[86]

Dual-chamber pacing may be an option in select patients with medically refractory symptomatic obstruction who are not candidates for, or who do not desire, surgery or ASA. It is not a primary line of therapy; however, as efficacy is unproven in randomised cross-over blinded studies.[91][92]​​ Randomised trials have not demonstrated benefit in objective measures of exercise capacity. Gradient reduction is less than that achieved with surgery.[93]

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
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
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]​​ Routine diagnostic testing to evaluate the risk of sudden death is recommended, regardless of symptom status.​ European guidelines recommend comprehensive sudden cardiac death risk stratification in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1]

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 of age 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 – management of arrhythmias

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
Consider – 

management of arrhythmias

Additional treatment recommended for SOME patients in selected patient group

Atrial arrhythmias, including atrial fibrillation (AF), are common, particularly in older patients with hypertrophic cardiomyopathy (HCM). Prevalence of AF among patients with HCM is estimated at 17% to 39%, with an annual incidence of 2.8% to 4.8%.[1] AF is often poorly tolerated in patients with HCM.[2] As a result, an aggressive strategy for maintaining sinus rhythm is warranted.

Paroxysmal and chronic AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms.[2]

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, aged 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2]​​​​[98]​​ 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 is recommended first-line option, and a vitamin K antagonist (e.g., warfarin) second-line option.[1][2][98]​​ See New-onset atrial fibrillation (Management) and  Chronic atrial fibrillation (Management)

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.[2]​

All patients with symptomatic ventricular arrhythmias or important asymptomatic ventricular arrhythmias should receive an implantable cardioverter-defibrillator (ICD).[1][2]​​ In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2] Although data are lacking, anti-arrhythmic drugs 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-arrhythmic drugs are ineffective, contraindicated, or not tolerated.[1][2]

Consider – surgical coronary artery unroofing (selected cases)

Back
ACUTE
|
symptomatic: predominant left ventricular outflow tract obstruction (LVOTO) with preserved systolic function
Consider – 

surgical coronary artery unroofing (selected cases)

Additional treatment recommended for SOME patients in selected patient group

In the presence of ischaemia due to a myocardial bridge (a band of heart muscle lying on top of a coronary artery) surgical coronary artery unroofing of the myocardial bridge has been shown to lead to resolution of ischaemia and of ventricular arrhythmias in some patients, and may lessen the incidence of sudden death.[30][94]​​ However, the evidence for the benefit of this procedure is limited and risks of the procedure need to be considered.[96]

symptomatic: predominant non-obstructive with preserved systolic function

1st line – negative inotropic and chronotropic agents

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
1st line – 

negative inotropic and chronotropic agents

Symptoms are related to diastolic dysfunction, with impaired filling resulting in reduced output and pulmonary congestion. 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]

Non-dihydropyridine calcium-channel blockers (verapamil and diltiazem) 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. Beta-blocker therapy may also be of benefit in patients with HCM and symptoms suggestive of ischaemia.

Disopyramide is not recommended, as it may decrease cardiac output more than the other therapies in this setting.

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]

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

Consider – management of angina

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
Consider – 

management of angina

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 Stable ischaemic heart disease (Management) for further details of management of angina.

Consider – management of heart failure

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
Consider – 

management of heart failure

Additional treatment recommended for SOME patients in selected patient group

Management of heart failure with reduced ejection fraction is focused on: (1) risk stratification and management of comorbidities, including hypertension, diabetes mellitus, obesity, atrial fibrillation, coronary artery disease, chronic kidney disease, and obstructive sleep apnoea; (2) non-pharmacological management, including exercise and weight loss; and (3) pharmacological treatment, namely disease-modifying medications and medication for symptom management (e.g., relief of congestion with loop diuretics).[99]

For further details of management of heart failure, see  Heart failure with preserved ejection fraction (Management)

Consider – implantable cardioverter-defibrillator (ICD)

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
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]​ Routine diagnostic testing to evaluate the risk of sudden death is recommended, regardless of symptom status. European guidelines recommend comprehensive sudden cardiac death risk stratification in all patients at initial presentation, then at 1-2 year intervals or whenever there is a change in clinical status.[1]

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 of age 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 – management of arrhythmias

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
Consider – 

management of arrhythmias

Additional treatment recommended for SOME patients in selected patient group

Atrial arrhythmias, including atrial fibrillation (AF), are common, particularly in older patients with hypertrophic cardiomyopathy (HCM). Prevalence of AF among patients with HCM is estimated at 17% to 39%, with an annual incidence of 2.8% to 4.8%.[1] AF is often poorly tolerated in patients with HCM.[2] As a result, an aggressive strategy for maintaining sinus rhythm is warranted.

Paroxysmal and chronic AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms.[2]

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, aged 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2]​​​​[98]​​ 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 is recommended first-line option, and a vitamin K antagonist (e.g., warfarin) second-line option.[1][2][98]​​ See New-onset atrial fibrillation (Management) and  Chronic atrial fibrillation (Management)

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.[2]​

All patients with symptomatic ventricular arrhythmias or important asymptomatic ventricular arrhythmias should receive an implantable cardioverter-defibrillator (ICD).[1][2]​​ In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2] Although data are lacking, anti-arrhythmic drugs 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-arrhythmic drugs are ineffective, contraindicated, or not tolerated.[1][2]

Consider – surgical coronary artery unroofing (selected cases)

Back
ACUTE
|
symptomatic: predominant non-obstructive with preserved systolic function
Consider – 

surgical coronary artery unroofing (selected cases)

Additional treatment recommended for SOME patients in selected patient group

In the presence of ischaemia due to a myocardial bridge (a band of heart muscle lying on top of a coronary artery) surgical coronary artery unroofing of the myocardial bridge has been shown to lead to resolution of ischaemia and of ventricular arrhythmias in some patients, and may lessen the incidence of sudden death.[30][94]​​ However, the evidence for the benefit of this procedure is limited and risks of the procedure need to be considered.[96]

ONGOING

symptomatic: end-stage heart failure with systolic dysfunction

1st line – beta-blocker + ACE inhibitor/angiotensin-II receptor antagonist + consideration for implantable cardioverter-defibrillator + avoidance of high-intensity athletics

Back
ONGOING
|
symptomatic: end-stage heart failure with systolic dysfunction
1st line – 

beta-blocker + ACE inhibitor/angiotensin-II receptor antagonist + consideration for implantable cardioverter-defibrillator + avoidance of high-intensity athletics

The average duration from onset of symptoms to end-stage disease is 14 years.[100] Systolic function deteriorates, and the left ventricle remodels and becomes dilated. The mechanism of end-stage 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.[100]

These patients are treated with standard heart failure therapy, including initially a beta-blocker and ACE inhibitor or angiotensin-II receptor antagonist. Other medications may be necessary as adjunctive therapies.

Patients should be considered for an implantable cardioverter-defibrillator 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.

Patients should refrain from high-intensity athletics.

Primary options

metoprolol: 12.5 to 200 mg orally (extended-release) once daily

or

bisoprolol: 1.25 to 10 mg orally once daily

-- AND --

captopril: 6.25 to 50 mg orally three times daily

or

enalapril: 2.5 to 20 mg orally twice daily

or

fosinopril: 5-40 mg orally once daily

or

lisinopril: 2.5 to 40 mg orally once daily

or

perindopril: 2-16 mg orally once daily

or

quinapril: 5-20 mg orally twice daily

or

ramipril: 1.25 to 10 mg orally once daily

or

trandolapril: 1-4 mg orally once daily

Secondary options

metoprolol: 12.5 to 200 mg orally (extended-release) once daily

or

bisoprolol: 1.25 to 10 mg orally once daily

-- AND --

candesartan: 4-32 mg orally once daily

or

losartan: 25-100 mg orally once daily

or

valsartan: 40-160 mg orally twice daily

Consider – digoxin

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

digoxin

Additional treatment recommended for SOME patients in selected patient group

Digoxin can be used in patients with a dilated left ventricle with reduced function. It is not used typically in the setting of severe hypertrophy. Digoxin should not be used if the patient has ventricular pre-excitation through an accessory pathway, as its atrioventricular nodal blocking effect may promote rapid conduction of the atrial arrhythmia across the accessory pathway, precipitating a ventricular arrhythmia or haemodynamic compromise.

Digoxin levels need monitoring. Toxicity may occur especially if there is renal dysfunction, hypokalaemia, hypomagnesaemia, or hypothyroidism.

Primary options

digoxin: 0.125 to 0.5 mg orally once daily

Consider – diuretics

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

diuretics

Additional treatment recommended for SOME patients in selected patient group

Diuretics should be considered for patients who have evidence of, or a prior history of, fluid retention.

Amiloride and triamterene should be used with caution with aldosterone antagonists, because of the increased risk of developing hyperkalaemia. Close monitoring of serum potassium levels is suggested in this situation.

Primary options

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

OR

bumetanide: 0.5 to 1 mg orally once or twice daily initially, increase according to response, maximum 10 mg/day

Secondary options

chlorothiazide: 250-500 mg orally once or twice daily, maximum 1000 mg/day

OR

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

OR

metolazone: 2.5 to 20 mg orally once daily

Consider – aldosterone antagonists

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

aldosterone antagonists

Additional treatment recommended for SOME patients in selected patient group

Aldosterone antagonists may be used in patients with moderate to severe heart failure. These agents should be used with caution in patients with renal dysfunction and hyperkalaemia.

Patients should discontinue potassium repletion, and renal function and serum potassium levels require strict monitoring.

Primary options

spironolactone: 25-100 mg orally once daily

Secondary options

eplerenone: 25-50 mg orally once daily

Consider – management of arrhythmias

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

management of arrhythmias

Additional treatment recommended for SOME patients in selected patient group

Atrial arrhythmias, including atrial fibrillation (AF), are common, particularly in older patients with hypertrophic cardiomyopathy (HCM). Prevalence of AF among patients with HCM is estimated at 17% to 39%, with an annual incidence of 2.8% to 4.8%.[1] AF is often poorly tolerated in patients with HCM.[2] As a result, an aggressive strategy for maintaining sinus rhythm is warranted.

Paroxysmal and chronic AF are linked to left atrial enlargement.[4] AF is independently associated with heart-failure-related death, occurrence of fatal and non-fatal stroke, as well as long-term progression of heart failure symptoms.[2]

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, aged 65-74 years, sex category [female]) are not predictive in patients with HCM, with evidence suggesting that they may perform suboptimally.[1][2]​​​​[98]​​ 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 is recommended first-line option, and a vitamin K antagonist (e.g., warfarin) second-line option.[1][2][98]​​ See New-onset atrial fibrillation (Management) and  Chronic atrial fibrillation (Management)

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.[2]​

All patients with symptomatic ventricular arrhythmias or important asymptomatic ventricular arrhythmias should receive an implantable cardioverter-defibrillator (ICD).[1][2]​​ In patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimise risk of shocks.[2] Although data are lacking, anti-arrhythmic drugs 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-arrhythmic drugs are ineffective, contraindicated, or not tolerated.[1][2]

Consider – surgical coronary artery unroofing (selected cases)

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

surgical coronary artery unroofing (selected cases)

Additional treatment recommended for SOME patients in selected patient group

In the presence of ischaemia due to a myocardial bridge (a band of heart muscle lying on top of a coronary artery) surgical coronary artery unroofing of the myocardial bridge has been shown to lead to resolution of ischaemia and of ventricular arrhythmias in some patients, and may lessen the incidence of sudden death.[30][94]​​ However, the evidence for the benefit of this procedure is limited and risks of the procedure need to be considered.[96]

Consider – referral for heart transplant

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symptomatic: end-stage heart failure with systolic dysfunction
Consider – 

referral for heart transplant

Additional treatment recommended for SOME patients in selected patient group

If patients remain refractory to medical therapy, they should be referred for consideration for heart transplant surgery.[101]

Heart transplants have been shown to improve survival and quality of life for patients with end-stage heart failure secondary to hypertrophic cardiomyopathy.[101] Presence of comorbidities, caretaker status, and goals of care should all be taken into account when considering patient eligibility for transplant.[101]

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