eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Cardiomyopathy, Hypertrophic: Treatment & Medication

Author: Charles I Berul, MD, Professor of Pediatrics, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center
Coauthor(s): Christina Y Miyake, MD, Senior Fellow in Electrophysiology and Pacing, Children's Hospital Boston
Contributor Information and Disclosures

Updated: Aug 25, 2009

Treatment

Medical Care

Medical management of hypertrophic cardiomyopathy (HCM) in children should focus on (1) ruling out secondary causes, (2) following for progression of disease and identifying those with obstruction, (3) controlling symptoms and restricting activity (with avoidance of volume depletion), (4) identifying those at risk for sudden cardiac death, and (5) screening of family members. Evaluation of the patient with hypertrophic cardiomyopathy can usually be conducted on an outpatient basis. Inpatient studies and surgical treatment may be necessary as well.

  • Evaluation especially in children should be performed to rule out secondary causes of cardiac hypertrophy including the following:
    • Athlete's heart: Long-term athletic conditioning can result in ventricular hypertrophy but typically results in a concentric hypertrophy with an associated increase in left ventricular diastolic dimension, unlike primary hypertrophic cardiomyopathy.
    • Inborn errors of metabolism
    • Mitochondrial disorders
    • Neuromuscular disorders
  • Children are at particular risk for development or progression of outflow tract obstruction and should be followed yearly with serial echocardiography during puberty.
    • Medical and surgical therapy are used to reduce ventricular contractility or increase ventricular volume, to increase ventricular compliance and outflow tract dimensions, and, in obstructive hypertrophic cardiomyopathy, to reduce the pressure gradient across the left ventricular (LV) outflow tract. Patients with symptoms or evidence of outflow tract obstruction are generally started on a calcium channel or beta blocker therapy. Disopyramide has been used in adults, but has potential proarrhythmic effects and is not typically used in children.
    • Patients with severe outflow tract obstruction may be candidates for surgical myomectomy. Alternative therapies, such as alcohol septal ablation or pacemaker insertion, are less commonly performed in children.
  • Reduction of the risk of sudden death is paramount to any therapy for hypertrophic cardiomyopathy. Although no strict guidelines are available, studies have suggested the risk factors for sudden cardiac death include a history of previous arrest, unexplained syncope, ventricular arrhythmias, family history of sudden cardiac death, abnormal blood pressure response during exercise stress testing, or a markedly enlarged septum (>3 cm in adult studies). The amount of outflow obstruction has not been shown to be a risk factor for sudden death.
  • Patients should be advised not to participate in competitive sports or strenuous activity.
  • All first-degree family members of the patient need to be informed and screened for hypertrophic cardiomyopathy. This entails a detailed history, physical examination, ECG, and echocardiography. If a genetic defect is known, asymptomatic family members should be gene tested.
  • Subacute bacterial endocarditis prophylaxis is not required.

Surgical Care

  • Left ventricular myomectomy
    • Left ventricular myomectomy is sometimes used for patients who have severe symptoms refractory to therapy and an outflow gradient of more than 50 mm Hg either with provocation or at rest.
    • The procedure is typically successful in abolishing the outflow gradient; most patients have symptomatic improvement for at least 5 years. However, the reduction in LV outflow gradient may not correlate with a reduction in the risk of sudden death or overall mortality. Furthermore, the outflow gradient may gradually increase over time and return to the same level as before, requiring a repeat procedure or additional medical therapy.
  • Pacemaker implantation
    • Transvenous dual-chamber pacing has been used for patients with hypertrophic cardiomyopathy, although this is not current clinical practice. The right ventricular (RV) septal preexcitation induced by RV apical pacing leads to a "pulling away" of the septum from the outflow region, allowing for an increase in flow with a decrease in LV outflow tract obstruction. Many patients with hypertrophic cardiomyopathy and pacemaker implantation feel that their symptoms improve, allowing a reduction in prescribed medication. However, a reduction in LV outflow tract gradient does not mean necessarily a reduction in vulnerability to ventricular arrhythmias and sudden death.
    • Some investigators have previously used permanent pacing in some patients with hypertrophic cardiomyopathy as adjunctive rather than primary treatment. The reported results widely vary, with a significant placebo effect and variability in patient outcomes. Currently, implantable defibrillators have essentially replaced pacemakers as cardiac rhythm management devices for hypertrophic cardiomyopathy.5,6,7,8
    • Recommendations for pacing in patients with hypertrophic cardiomyopathy have been made by the American College of Cardiology and American Heart Association.9
  • Catheter septal ablation
    • Transvenous catheter ablation of the septal region has been performed using selective arterial ethanol infusion to destroy myocardial tissue in patients with hypertrophic cardiomyopathy.
    • The procedure is analogous to a surgical myomectomy in attempting to decrease the amount of septal ventricular myocardium, thereby reducing the LV outflow tract gradient. Complications may be more frequent than observed with myomectomy and include heart block, ventricular arrhythmias, and myocardial infarction.
  • Implantable cardioverter defibrillator
    • The implantable cardioverter defibrillator (ICD) has been used for prevention of sudden arrhythmic death. Transvenous placement is similar in technique to permanent pacemaker implantation. An ICD automatically detects, recognizes, and treats tachyarrhythmias and bradyarrhythmias using tiered therapy (ie, bradycardia pacing, overdrive tachycardia pacing, low-energy cardioversion, high-energy shock defibrillation).
    • ICD therapy has been demonstrated to be lifesaving in children with hypertrophic cardiomyopathy who receive appropriate shocks for ventricular tachycardia and ventricular fibrillation, even among those on appropriate antiarrhythmic drug therapy.
    • Smaller studies in children as well as personal and anecdotal experience appear strongly to favor using the ICD in patients with hypertrophic cardiomyopathy and arrhythmias, aborted sudden death, malignant genotype or family history, and other factors that may increase mortality, particularly sudden arrhythmic death risk.5,6,7,8
    • Clearly, in patients who have had an aborted sudden death event or documented sustained ventricular tachyarrhythmias, the ICD is indicated as secondary prevention. 
    • In adults, teenagers, and children, primary prevention therapy is also used for patients with hypertrophic cardiomyopathy but without a documented ventricular tachyarrhythmia or aborted sudden death event. Although this is a reasonable indication, the appropriate shock rate is significantly lower in these primary prevention patients.
    • Additional markers of higher risk (eg, LV wall thickness, nonsustained ventricular tachycardia, abnormal exercise blood pressure response, malignant family history, other stratifying tests) are useful in identifying patients who have greater ventricular arrhythmia vulnerability.
    • The main drawbacks to implanting an ICD include the relatively high rate of inappropriate shocks (for sinus tachycardia, supraventricular tachycardia, or lead problems) and a high lead fracture rate, particularly in younger patients.
    • The devices last approximately 4-5 years because of either battery depletion or lead failure, and these young patients require multiple ICD device replacements and lead extraction procedures, which carry additional surgical risks. For more information, see Pacemaker Therapy.

Consultations

  • Cardiologist
  • Cardiothoracic surgeon
  • Cardiac electrophysiologist
  • Geneticist

Diet

  • No special diet is required in individuals with hypertrophic cardiomyopathy; however, patients should be instructed to avoid volume depletion as this can increase pressure gradients across the left ventricular outflow tract.
  • Advise patients to avoid excessive weight gain.

Activity

  • Advise patients to avoid strenuous and anaerobic exercise. 
  • Competitive level sports are not advised if any of the following are present:
    • Significant outflow gradient
    • Significant ventricular or supraventricular arrhythmia
    • Marked LV hypertrophy
    • History of sudden death in relatives with hypertrophic cardiomyopathy

Medication

Beta-blockers and calcium channel blockers are used to treat children with hypertrophic cardiomyopathy (HCM). In individuals with significant tachyarrhythmias, amiodarone and other class III-type antiarrhythmic agents have also been used.

Beta-adrenergic blocking agents

These agents may decrease outflow obstruction and increase ventricular compliance. No clear evidence indicates that they decrease sudden death. Approximately one half of patients who use beta-blockers feel improvement in symptoms.


Propranolol (Inderal)

Nonselective beta-blocker with long record of use and relative safety. Treatment dose titrated to produce clinical effect (ie, reduction in perceived symptoms). Blunting of maximal heart rate during exercise testing is a good marker for beta-blocker effect. Although generally a short-acting agent, long-acting preparations are available. A stable liquid preparation is available and can be used to treat infants.

Adult

1-3 mg IV (under careful monitoring); not to exceed 1 mg/min to avoid lowering blood pressure and causing cardiac standstill
Allow time for drug to reach site of action (particularly if slow circulation); administer second dose after 2 min prn; thereafter, do not give additional drug in <4 h
Discontinue IV doses after desired alteration in rate or rhythm is achieved; switch to PO as soon as possible; typical oral dosage range is 10-30 mg PO tid/qid

Pediatric

Initial: 0.01-0.1 mg/kg/dose IV over 10 min
Maintenance: 1-4 mg/kg/d PO divided q6-8h

Effects may be decreased by aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin; toxicity may be increased by calcium channel blockers, cimetidine, loop diuretics, and MAOIs; may increase toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines

Documented hypersensitivity; cardiogenic shock; sinus bradycardia; AV block greater than first-degree (without a pacemaker); bronchial asthma; congestive heart failure unless caused by tachyarrhythmia treatable with beta-blockers; diabetes

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Titrate dose carefully to level of patient tolerance and effectiveness; slowly withdraw drug and carefully monitor; asthma and bronchospasm; AV conduction disturbance; depression; bradycardia; hypoglycemia in infants; may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism and cause thyroid storm


Atenolol (Tenormin)

Selectively blocks beta-1 receptors with little or no effect on beta-2 types. May be better tolerated than propranolol (has more favorable pharmacokinetics and frequently has equivalent efficacy).

Adult

25-50 mg/d PO initial; may titrate upward prn; not to exceed 100 mg/d

Pediatric

0.1-0.3 mg/kg/d PO q12-24h

Effects may be decreased by aluminum salts, barbiturates, calcium salts, cholestyramine, NSAIDs, penicillins, and rifampin; toxicity may be increased by haloperidol, hydralazine, loop diuretics, and MAOIs

Documented hypersensitivity; uncompensated congestive heart failure; bradycardia; cardiogenic shock; AV conduction abnormalities; severe ventricular dysfunction

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Asthma and bronchospasm; AV conduction disturbance; depression; bradycardia; hypoglycemia in infants; may reduce symptoms of acute hypoglycemia and mask signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism and cause thyroid storm; monitor patients closely and withdraw drug slowly

Calcium channel blockers

These agents are an alternative to beta-blockers. Calcium channel blockers improve diastolic filling by improving diastolic relaxation and decreasing outflow gradient due to depression of cardiac contractility.


Verapamil (Calan, Isoptin)

During depolarization, inhibits calcium ion from entering slow channels or voltage-sensitive areas of the vascular smooth muscle and myocardium. May have better effect on exercise performance. Sustained release formulations with qd dosing are available.

Adult

240-480 mg/d PO qd (extended release) or divided q6-8h (immediate release)
Alternatively, 5-10 mg IV followed by a second dose 15-30 min later if patient does not satisfactorily respond to initial dose

Pediatric

Neonates: Not recommended
>1 year: 0.1-0.2 mg/kg/dose IV over 2 min; repeat q10-30min prn initial; not to exceed 5 mg/dose (first dose) or 10 mg/dose (second dose)
Maintenance: 3-8 mg/kg/d PO divided qid

Verapamil may increase carbamazepine, digoxin, and cyclosporine levels; coadministration with amiodarone can cause bradycardia and a decrease in cardiac output; when administered concurrently with beta-blockers may increase cardiac depression; cimetidine may increase verapamil levels; verapamil may increase theophylline levels

Documented hypersensitivity; PND; increased LV end-diastolic pressure; orthopnea; AV block; sinus node disease

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Depresses impulse formation, AV block, negative inotropism, and vasodilation, which can result in hypotension, shock, pulmonary edema, and death; hepatocellular injury may occur; transient elevations of transaminases with or without concomitant elevations in alkaline phosphatase and bilirubin have occurred (elevations have been transient and may disappear with continued verapamil treatment); monitor liver function periodically

Antiarrhythmics, miscellaneous

Amiodarone is categorized as a class III antiarrhythmic agent but has antiarrhythmic effects that overlap all 4 Vaughn-Williams antiarrhythmic classes. Its use is generally reserved for potentially life-threatening ventricular arrhythmias.


Amiodarone (Cordarone)

Complex and potent antiarrhythmic agent with multiple effects on cardiac action potential, exceedingly complex pharmacokinetics, and extracardiac pharmacodynamics. PO efficacy may take weeks. With exception of disorders of prolonged repolarization (eg, LQTS), may be DOC for life-threatening ventricular arrhythmias refractory to beta-blockade and initial therapy with other agents.

Adult

Loading dose: 800-1600 mg/d PO divided in 1-2 doses for 1-3 wk, decrease to 600-800 mg/d divided in 1-2 doses for 1 mo
Alternatively, 150 mg IV over first 10 min, followed by 360 mg over next 6 h, and then 540 mg over next 18 h
Maintenance: 400 mg/d PO

Pediatric

Loading dose: 10-15 mg/kg/d PO divided in 1-2 doses for 1-3 wk; decrease to 2-6 mg/kg/d divided in 1-2 doses for 1 mo; alternatively, 2-3 mg/kg IV over 5-10 min; may repeat q10-30min, not to exceed a cumulative dose of 10-15 mg/kg/d

Increases effect and blood levels of theophylline, quinidine, procainamide, phenytoin, methotrexate, flecainide, digoxin, cyclosporine, beta-blockers, and anticoagulants; cardiotoxicity of amiodarone is increased by macrolide antibiotics, ritonavir, sparfloxacin, and disopyramide; coadministration with calcium channel blockers may cause an additive effect and decrease myocardial contractility further; cimetidine may increase amiodarone levels; protease inhibitors (eg, indinavir, ritonavir, amprenavir, nelfinavir) inhibit amiodarone metabolism, resulting in increased serum levels, and may prolong QT interval; coadministration may increase myopathy/rhabdomyolysis risk associated with HMG-CoA reductase inhibitors (eg, simvastatin); other drugs that prolong the QT interval (eg, fluoroquinolones, erythromycin, dofetilide, tricyclic antidepressants, thioridazine) may increase life-threatening arrhythmia risk

Documented hypersensitivity or allergy to iodine; complete AV block (without a pacemaker); intraventricular conduction defects

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

IV preparation may induce hypotension; calcium may reverse this hypotension; carefully monitor pulmonary function, thyroid function, and corneal staining; caution in liver or thyroid disease; CNS and GI toxicity may occur and typically dissipate with dose reduction

More on Cardiomyopathy, Hypertrophic

Overview: Cardiomyopathy, Hypertrophic
Differential Diagnoses & Workup: Cardiomyopathy, Hypertrophic
Treatment & Medication: Cardiomyopathy, Hypertrophic
Follow-up: Cardiomyopathy, Hypertrophic
Multimedia: Cardiomyopathy, Hypertrophic
References
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Further Reading

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Keywords

hypertrophic cardiomyopathy, hypertrophic obstructive cardiomyopathy, idiopathic hypertrophic subaortic stenosis, IHSS, muscular subaortic stenosis, asymmetric septal hypertrophy, ASH, HCM, ventricular hypertrophy, outflow tract obstruction, ventricular tachycardia, ventricular fibrillation, dyspnea, syncope, presyncope, angina, palpitations, orthopnea, paroxysmal nocturnal dyspnea, congestive heart failure, dizziness, atrial flutter, supraventricular tachycardia associated with Wolff-Parkinson-White syndrome, sick sinus syndrome, angina, Pompe disease, Barth syndrome, Friedrich ataxia, Duchenne muscular dystrophy, Noonan syndrome, Danon disease

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Contributor Information and Disclosures

Author

Charles I Berul, MD, Professor of Pediatrics, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congential Electrophysiology Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Christina Y Miyake, MD, Senior Fellow in Electrophysiology and Pacing, Children's Hospital Boston
Christina Y Miyake, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Massachusetts Medical Society, and Pediatric and Congential Electrophysiology Society
Disclosure: Nothing to disclose.

Medical Editor

Christopher Johnsrude, MD, Associate Professor of Pediatrics, Director of Electrophysiology, University of Louisville School of Medicine; Consulting Staff, Pediatric Cardiology Associates, PSC
Christopher Johnsrude, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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