Sections

Guidelines

Guidelines Summary

The following organizations have released guidelines for the managment of hypertrophic cardiomyopathy:

American College of Cardiology Foundation (ACCF)/American Heart Association (AHA)
European Society of Cardiology(ESC)
Heart Rhythm Society(HRS)/American College of Cardiology Foundation (ACCF)

Invasive Therapies

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding invasive therapies in patients with hypertrophic cardiomyopathy (HCM) are summarized below.^{[22, 1]}

Class I recommendations

Only experienced operators should perform septal reduction therapy, in the setting of a dedicated HCM. Moreover, septal reduction therapy should be reserved for treatment-eligible patients with severe drug-refractory symptoms and left ventricular outlet tract (LVOT) obstruction. (Level of evidence: C)

Class IIa recommendations

It is reasonable to consult with centers experienced in performing surgical septal myectomy and alcohol septal ablation for treatment-eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction. At these centers, surgical septal myomectomy may be of benefit in symptomatic pediatric patients in whom traditional medical therapy has been ineffective. (Level of evidence: C)

Surgical septal myectomy performed in experienced centers is the first-line option for most treatment-eligible patients with HCM and severe drug-refractory symptoms and LVOT obstruction. Moreover, at these centers, adult patients with HCM and severe drug-refractory symptoms and LVOT obstruction who are not surgical candidates but who are eligible for alcohol septal ablation may benefit from this procedure (usually New York Heart Association [NYHA] class III or IV). (Level of evidence: B)

Class IIb recommendations

In experienced centers, following a detailed discussion with eligible adult patients with HCM and severe drug-refractory symptoms and LVOT obstruction, alcohol septal ablation may be a treatment option to surgical myectomy when the patient indicates a preference for septal ablation. (Level of evidence: B)

In general, alcohol septal ablation is discouraged in patients with HCM and marked (ie, >30 mm) septal hypertrophy owing to uncertainty regarding its efficacy in these patients. (Level of evidence: C)

Precautions (class III recommendations)

Note the following to prevent harm to patients with HCM (C level of evidence for all)^{[22, 1]}:

Septal reduction therapy should only be performed as part of a dedicated HCM program.
Avoid septal reduction therapy in asymptomatic adults with HCM who have normal exercise tolerance or whose symptoms are controlled/minimized on medical therapy.
When septal reduction therapy is a feasible treatment option to relieve LVOT obstruction in eligible patients, do not perform mitral valve replacement as an alternative therapy.
Avoid performing alcohol septal ablation in (1) patients with HCM and comorbid conditions that also require surgical repair, in whom myectomy can be performed concomitantly; (2) pediatric patients with HCM (age < 21 years); and (3) adults younger than 40 years in whom myectomy is a feasible alternative therapy.

The 2014 ESC guidelines recommendations for septal reduction therapy are summarized below.^[9]

Class I recommendations

Septal reduction therapies be performed by experienced operators, working as part of a multidisciplinary team expert in the management of HCM.(Level of evidence: C)

Septal reduction therapy to improve symptoms in patients with a resting or maximum provoked LVOT gradient of ≥50 mm Hg, who are in NYHA functional Class III–IV, despite maximum tolerated medical therapy. (Level of evidence: B)

Septal myectomy, rather than alcohol septal ablationis in patients with an indication for septal reduction therapy and other lesions requiring surgical intervention (e.g. mitral valve repair/replacement, papillary muscle intervention).(Level of evidence: C)

Class IIa recommendations

Septal reduction therapy should be considered in patients with recurrent exertional syncope caused by a resting or maximum provoked LVOTO gradient ≥50 mm Hg despite optimal medical therapy. (Level of evidence: C)

Mitral valve repair or replacement should be considered in symptomatic patients with a resting or maximum provoked LVOTO gradient ≥50 mm Hg and moderate-to-severe mitral regurgitation not caused by systolic anterior motion of the mitral valve alone. (Level of evidence: C)

Class IIb recommendation

Mitral valve repair or replacement may be considered in patients with a resting or maximum provoked LVOTO gradient ≥50 mm Hg and a maximum septal thickness 16 mm at the point of the mitral leaflet–septal contact or when there is moderate-to-severe mitral regurgitation following isolated myectomy.

Pacemaker Implemenation

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding pacing in patients with HCM are summarized below.^{[22, 1]}

Class IIa recommendation

For patients with HCM who previously underwent dual-chamber device implantation for non–HCM-related causes, the ACCF/AHA believe a trial of dual-chamber atrial-ventricular pacing (from the right ventricular apex) for symptomatic relief from LVOT obstruction can be considered. (Level of evidence: B)

Class IIb recommendation

Permanent pacing is an option in symptomatic patients with medically refractory obstructive HCM who are not good candidates for septal reduction therapy. (Level of evidence: B)

Select patients in whom permanent pacemaker implantation is of no benefit (class III recommendations)

The 2011 ACCF/AHA guidelines indicate pacemaker implantation is not beneficial in the following scenarios for patients with HCM^{[22, 1]}:

For reduction of the gradient in those who are asymptomatic or whose symptoms are medically-controlled (Level of evidence: C)
For first-line treatment of symptomatic relief in candidates eligible for septal reduction who have medically refractory disease and LVOT obstruction (Level of evidence: B)

The 2012 HRS/ACCF expert consensus statement on pacemaker device and mode selection offer the following guidance^[37]:

Class IIa recommendation

Dual-chamber pacing can be useful for patients with medically refractory, symptomatic hypertrophic, cardiomyopathy with significant resting, or provoked left ventricular outflow obstruction. (Level of evidence: C)

Class III recommendation

Single-chamber (VVI or AAI) pacing is not recommended for patients with medically refractory, symptomatic hypertrophic cardiomyopathy. (Level of evidence: C)

In its 2013 guidelines for cardiac pacing and cardiac resynchronization therapy^[38]and its 2014 guidelines for management of hypertrophic cardiomyopathy^[9], the European Society of Cardiology (ESC) recommends considering sequential AV pacing, with optimal AV interval to reduce the LV outflow tract gradient or to facilitate medical treatment with ß-blockers and/or verapamil in selected patients who have contraindications for septal alcohol ablation or septal myectomy. (Class IIb; Level of evidence: C)^[9]

Alcohol Septal Ablation

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding alcohol septal ablation in patients with HCM are summarized below.^{[22, 1]}

Class I recommendations

As discussed earlier, it is reasonable to consult with centers experienced in performing alcohol septal ablation and surgical septal myectomy for treatment-eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction.

Use transthoracic (TTE) or transesophageal (TTE) echocardiography for intraoperative guidance of alcohol septal ablation (level of evidence: B) and TTE for assessing the outcomes of alcohol septal ablation (or surgical myectomy) in patients with obstruct HCM (level of evidence: C).

Class IIa recommendations

For clinical decision making and evaluation for the feasibility of alcohol septal ablation, use TEE when TTE findings are unclear.

Class IIb recommendations

Class III recommendations

To prevent harm, avoid performing alcohol septal ablation in (1) patients with HCM and comorbid conditions that also require surgical repair, in whom myectomy can be performed concomitantly; (2) pediatric patients with HCM (age < 21 years); and (3) adults younger than 40 years in whom myectomy is a feasible alternative therapy. (Level of evidence: C)

ICD Placement

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding ICD placement in patients with HCM are summarized below.^{[22, 1]}

Class I recommendations

ICD placement decision making in patients with HCM should involve a comprehensive discussion between clinicians and patients. (Level of evidence: C)

This procedure is recommended for patients with HCM who have documentation of having suffered cardiac arrest, ventricular fibrillation, or hemodynamically significant VT. (Level of evidence: B)

Class IIa recommendations

ICD placement is a reasonable intervention for patients with HCM who also have (1) one or more first-degree relatives who suffered sudden cardiac death likely related to HCM, (2) an LV wall thickness of 30 mm or more, and (3) one or more recent unexplained syncopal events, as well as for (4) high-risk pediatric patients with HCM and a history of unexplained syncope or massive LV hypertrophy, or a family history of sudden cardiac death, with consideration of long-term ICD complication rates. (Level of evidence: C)

ICD placement is an option for select patients with other risk factors for other sudden cardiac death in addition to having (1) nonsustained VT (eg, age < 30 y) or (2) HCM plus exercise-induced blood pressure anomalies. (Level of evidence: C)

For ICD-eligible patients with HCM, it is reasonable to place a single-chamber device in younger patients who do not require atrial or ventricular pacing, or a dual-chamber device (1) in patients with sinus bradycardia and/or paroxysmal atrial fibrillation or (2) predominantly in older patients with high resting outflow gradients (>50 mm Hg) and significant heart failure symptoms in whom right ventricular pacing has the potential beneficial effects. (Level of evidence: C)

Class IIb recommendations

ICD effectiveness remains unclear in patients with HCM who do not have other risk factors for sudden cardiac death but do have either (1) isolated bursts of nonsustained VT or (2) exercise-induced blood pressure anomalies, especially in the setting of significant LVOT. (Level of evidence: C)

Precautions (class III recommendations)

To prevent harm to patients with HCM, do not use ICD placement as either (1) a routine strategy in the absence of high-risk factors or (2) a strategy to allow participation in competitive athletic events. Do not place an ICD in those with a known HCM genotype but who are asymptomatic. (Level of evidence: C)

In its 2013 guidelines for cardiac pacing and cardiac resynchronization therapy^[38]and its 2014 guidelines for management of hypertrophic cardiomyopathy^[9], the European Society of Cardiology (ESC) recommends considering a dual-chamber ICD (instead of a single-lead device) to reduce the LV outflow tract gradient or to facilitate medical treatment with ß-blockers and/or verapamil in patients with resting or provocable LVOTO ≥50 mm Hg, sinus rhythm and drug refractory symptoms. (Class IIb; Level of evidence: C)^[9]

Heart Transplantation

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding heart transplantation in patients with HCM are summarized below.^{[22, 1]}

Class I recommendations

Heart transplantion should not be considered in the setting of advanced heart failure (end stage) and nonobstructive HCM not otherwise amenable to other treatment intervention, in the presence of an ejection fraction (EF) that is at or below 50% (or, occasionally, with preserved EF). (Level of evidence: B)

Pediatric heart transplant candidates include those with symptomatic HCM with a restrictive physiology who are not responsive to or appropriate candidates for other therapeutic interventions. (Level of evidence: C)

Precautions (class III recommendations)

To prevent harm, do not perform heart transplantation in mildy symptomatic patients of any age with HCM. (Level of evidence: C)

The 2014 ESC guidelines recommendations regarding heart transplantation in patients with HCM are summarized below.^[9]

Class I recommendation

For severely symptomatic patients with systolic and/or diastolic LV dysfunction being evaluated for heart transplantation or mechanical support, cardiopulmonary exercise testing, with simultaneous measurement of respiratory gases should be performed. (Level of evidence: B)

Class IIa recommendation

Orthotopic cardiac transplantation should be considered in eligible patients who have an LVEF < 50% and NYHA functional Class III–IV symptoms despite optimal medical therapy or intractable ventricular arrhythmia.(Level of evidence: B)

Class IIb recommendation

Orthotopic cardiac transplantation may be considered in eligible patients with normal LVEF ( 50%) and severe drug refractory symptoms (NYHA functional Class III–IV) caused by diastolic dysfunction.(Level of evidence: B)

Management of Atrial Fibrillation

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding management of atrial fibrillation in patients with HCM are summarized below.^{[22, 1]}

Class I recommendations

Use vitamin K antagonis (ie, warfarin, to an international normalized ratio [INR] of 2-3) for anticoagulation in patients with paroxysmal, persistent, or chronic atrial fibrillation AF and HCM. (Data are not available in the setting of HCM for anticoagulation with direct thrombin inhibitors such as dabigatran in reducing the risk of thromboembolism). (Level of evidence: C)

Control the ventricular rate in patients with HCM and atrial fibrillation who have rapid ventricular rates. High doses of beta antagonists and nondihydropyridine calcium channel blockers may need to be administered. (Level of evidence: C)

Class IIa recommendations

The ACCF/AHA indicates it is reasonable to use disopyramide (with ventricular rate–controlling agents) and amiodarone for atrial fibrillation in patients with HCM. (Level of evidence: B)

Radiofrequency ablation can be beneficial in the setting of HCM with refractory atrial fibrillation or patients unable to take antiarrhythmic agents. (Level of evidence: B)

Maze procedure with closure of the left atrial appendage is a reasonable intervention in patients with HCM and a histroy of atrial fibrillation. The procedure may be performed during septal myectomy or as an isolated procedure in selected patients. (Level of evidence: C)

Class IIb recommendations

Alternative antiarrhthmic agents for patients with HCM and atrial fibrillation include sotalol, dofetilide, and dronedarone, particularly in those with an implantable cardioverter defibrillator. (Level of evidence: C)

Management of Pregnancy

The 2011 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) guidelines recommendations regarding management of pregnancy and/or delivery in women with HCM are summarized below.^{[22, 1]}

Class I recommendations

Pregnancy is not contraindicated in asymptomatic women with HCM; perform a careful evaluation for pregnancy risks. Asymptomatic women or those whose symptoms are controlled with beta-blocking drugs should continue pharmacotherapy during their pregnancy, and close monitoring for fetal bradycardia or other complications is important. (Level of evidence: C)

Male and female patients with HCM should receive genetic counseling before planned conception. (Level of evidence: C)

Management by a high-risk obstetric team is essential for women with HCM and resting or provocable LVOT obstruction of 50 mm Hg or greater and/or cardic symptoms not controlled by medical therapy alone. (Level of evidence: C)

Class IIa recommendations

Management by an expert materal-fetal team is advised for women with HCM whose symptoms are controlled (mild to moderate). Such specialist care includes cardiovascular and prenatal monitoring. (Level of evidence: C)

Precautions (class III recommendations)

Women with advanced heart failure symptoms and HCM have an increased risk of excess morbidity/mortality in pregnancy. (Level of evidence: C)

The 2014 ESC guidelines recommendations are summarized below.^[9]

Class I recommendations (Level of evidence C for all)

Pre-pregnancy risk assessment and counselling is indicated in all women.
Counselling on safe and effective contraception is indicated in all women of fertile age.
Counselling on the risk of disease transmission is recommended for all men and women before conception.
β-Blockers (preferably metoprolol) should be started in women who develop symptoms during pregnancy.
Whenever β-blockers are prescribed, monitoring of fetal growth and of the condition of the neonate is recommended.
Scheduled (induced) vaginal delivery is recommended as first choice in most patients.
For atrial fibrillation, therapeutic anticoagulation with LMWH or vitamin K antagonists depending on the stage of pregnancy.

Class IIa recommendations

β-Blockers (preferably metoprolol) should be continued in women who used them before pregnancy.
Cardioversion should be considered for persistent atrial fibrillation.

Competitive Athletes

In 2015, the American Heart Association(AHA) and American College of Cardiology (ACC) released a scientific statement containing eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities. The recommendations for athlestes with HCM are summarized below.^[39]

Class IIa recommendation

Participation in competitive athletics for asymptomatic, genotype-positive HCM patients without evidence of LV hypertrophy by 2-dimensional echocardiography and CMR is reasonable, particularly in the absence of a family history of HCM-related sudden death (Level of evidence C).

Class III recommendations

Athletes with a probable or confirmed diagnosis of HCM should not participate in most competitive sports, with the exception of those of low intensity (class IA sports). (Level of evidence C).

Pharmacological agents (eg, β-blockers) to control cardiac-related symptoms or ventricular tachyarrhythmias should not be administered for the sole purpose of permitting participation in high-intensity sports. (Level of evidence C).

Prophylactic ICDs should not be placed in athlete-patients with HCM for the sole or primary purpose of permitting participation in high-intensity sports competition because of the possibility of device-related complications. ICD indications for competitive athletes with HCM should not differ from those in nonathlete patients with HCM.(Level of evidence B).

Medication

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