eMedicine Specialties > Cardiology > Myocardial Disease and Cardiomyopathies

Cardiomyopathy, Hypertrophic: Treatment & Medication

Author: Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice
Contributor Information and Disclosures

Updated: Aug 27, 2009

Treatment

Medical Care

  • Evaluation usually can be conducted on an outpatient basis. Inpatient studies and surgical treatment also may be necessary.
  • Medical and surgical therapy is used to reduce ventricular contractility or increase ventricular volume, increase ventricular compliance and outflow tract dimensions, and, in the case of obstructive HCM, reduce the pressure gradient across the LV outflow tract. Paramount to any therapy is reduction in the risk of sudden death by identification of these patients early on and effective medical and/or surgical implantation of an automatic defibrillator.13

Surgical Care

  • Left ventricular myomectomy
    • LV myomectomy is used for patients with severe symptoms refractory to therapy and an outflow gradient of more than 50 mm Hg, either with provocation or with rest.
    • The procedure typically is successful in abolishing the outflow gradient; most patients have symptomatic improvement for at least 5 years.
    • The reduction in LV outflow gradient may not correlate with a risk reduction for sudden death or overall mortality. Furthermore, the outflow gradient may increase gradually over time and return to the same level as before, requiring a repeat procedure or additional medical therapy.
  • Mitral valve replacement: Mitral valve replacement is reserved for those patients with severe mitral regurgitation due to systolic anterior movement of the mitral valve, particularly when mitral regurgitation (large regurgitant fraction) is associated with the development congestive heart failure, largely due to), or pulmonary hypertension.
  • Pacemaker implantation
    • The ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities recommend permanent pacing for SND or AV block in patients with HCM and may be considered in medically refractory symptomatic patients with hypertrophic cardiomyopathy and significant resting or provoked LV outflow tract obstruction.14
    • Transvenous dual-chamber pacing has been used for patients with HCM. The right ventricular septal preexcitation induced by right ventricular 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.15
    • Many patients feel an improvement in symptoms and can have a reduction in prescribed medication.
    • Note again that a reduction in LV outflow tract gradient does not necessarily mean a reduction in vulnerability to ventricular arrhythmias and sudden death. Therefore, permanent pacing in patients with HCM has been used as adjunctive therapy by some investigators rather than as primary treatment. The reported results are widely variable, with a significant placebo effect and variability in patient outcomes.16,17  
  • Catheter septal ablation 
    • Transvenous catheter ablation of the septal region has been performed using selective arterial ethanol infusion to destroy myocardial tissue.18
    • The procedure involves infusing 96% ethanol down the first septal branch of the left anterior descending artery and inducing a therapeutic infarction of the proximal interventricular septal myocardium. This leads to a remodeling of the septum, which decreases the marked septal thickening characteristic of HCM and results in a decrease of the gradient across the LV outflow tract. In this manner, the procedure is analogous to a surgical myomectomy, in attempting to decrease the amount of septal ventricular myocardium and thereby reducing the LV outflow tract gradient.
    • The procedure has been used in clinical practice since the early 1990s and the reported results have been excellent, with significant reduction symptoms, particularly in the incidence of heart failure.19,20
    • In many centers, it is the surgical procedure of choice for HCM.
  • Implantable cardioverter defibrillator
    • The implantable cardioverter defibrillator (ICD) has been used for the prevention of sudden arrhythmic death.
    • Transvenous placement is similar in technique to permanent pacemaker implantation and can be performed in either the electrophysiology laboratory or operating room.
    • An ICD automatically detects, recognizes, and treats tachyarrhythmias and bradyarrhythmias using tiered therapy (ie, bradycardia pacing, overdrive tachycardia pacing, low-energy cardioversion, and high-energy shock defibrillation).
    • ICD therapy has been shown to be life saving. In recent, large, well-designed prospective studies in adults with coronary artery disease and low ejection fraction surviving myocardial infarction, the ICD has been demonstrated to be superior to antiarrhythmic drug therapy.21
    • Ongoing studies are being performed to assess the value of ICD therapy in cardiomyopathy. Smaller studies in children and personal and anecdotal experience appear to strongly favor utilization of the ICD in patients with HCM and arrhythmias, aborted sudden death, malignant genotype or family history, and other factors that may increase mortality and, particularly, sudden arrhythmic death risk.

Consultations

  • Cardiologist
  • Cardiothoracic surgeon
  • Cardiac electrophysiologist
  • Geneticist

Diet

  • No special diet is required.
  • The patient should avoid excessive weight gain.

Activity

  • Avoid strenuous exercise.
  • Competitive level sports should not be permitted if any of the following is present:
    • Significant outflow gradient
    • Significant ventricular or supraventricular arrhythmia
    • Marked LV hypertrophy
    • History of sudden death in relatives with HCM
    • Identified malignant genotype
    • Young Age (<30 years)
    • Abnormal blood pressure response to exercise
    • History of syncope, particularly in children
  • Although avoidance of intense physical exertion is probably appropriate, participation in noncompetitive level recreational sports activities is not believed to be contraindicated.
  • Cardiovascular screening before participation in competitive sports appears to reduce the frequency of unexpected sudden death from hypertrophic cardiomyopathy, although whether large scale screening of athletes is administratively feasible or cost-effective remains to be determined.22,23
  • Sudden death often occurs during exercise, but also demonstrates a circadian distribution, with clustering of deaths in the morning and early evening.

Medication

The purpose of pharmacologic therapy is to reduce the pressure gradient across the LV outflow tract by reducing the inotropic state of the left ventricle, improving compliance of the left ventricle, and reducing diastolic dysfunction. To date, only one pharmacologic agent, amiodarone (Cordarone), has been shown to reduce the incidence of arrhythmogenic sudden cardiac death.24,25

Beta-adrenergic blocking agents

Reduce inotropic state of left ventricle. Decrease diastolic dysfunction and increase LV compliance, thereby reducing pressure gradient across LV outflow tract. Decrease myocardial oxygen consumption, thereby reducing myocardial ischemia potential. Decrease heart rate, thus reducing myocardial oxygen consumption and reducing myocardial ischemia potential.


Metoprolol (Lopressor)

First-line therapy in treatment of both obstructive and nonobstructive HCM. Rarely, patients may require up to 200 mg PO bid to achieve desired effect. Dose titrated to heart rate between 50 and 60 bpm.

Adult

25-100 mg PO bid

Pediatric

Not established

Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels, possibly resulting in decreased pharmacologic effects; toxicity may increase with coadministration of sparfloxacin, phenothiazines, astemizole (recalled from US market), calcium channel blockers, quinidine, flecainide, and contraceptives; may increase toxicity of digoxin, flecainide, clonidine, epinephrine, nifedipine, prazosin, verapamil, and lidocaine

Documented hypersensitivity; uncompensated congestive heart failure, bradycardia, asthma, cardiogenic shock, AV conduction abnormalities

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

Beta-adrenergic blockade may reduce signs and symptoms of acute hypoglycemia and may decrease clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; monitor patient closely and withdraw drug slowly; during IV administration, carefully monitor BP, heart rate, and ECG


Atenolol (Tenormin)

Selectively blocks beta1 receptors with little or no effect on beta2 types.

Adult

25-100 mg/d PO in AM or divided bid

Pediatric

Not established

Coadministration with aluminum salts, barbiturates, calcium salts, cholestyramine, NSAIDs, penicillins, and rifampin may decrease effects; haloperidol, hydralazine, loop diuretics, and MAOIs may increase toxicity

Documented hypersensitivity; congestive heart failure, pulmonary edema, cardiogenic shock, AV conduction abnormalities, heart block (without a pacemaker)

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

Beta-adrenergic blockade 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; during IV administration, carefully monitor BP, heart rate, and ECG


Sotalol (Betapace)

Class III antiarrhythmic agent that blocks K+ channels, prolongs action potential duration, and lengthens QT interval. Noncardiac selective beta-adrenergic blocker that may be helpful in the use of conversion from and suppression of atrial fibrillation and flutter.

Adult

80-320 mg PO bid

Pediatric

Not established

Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels, possibly resulting in decreased pharmacologic effect; cardiotoxicity may increase when administered concurrently with sparfloxacin, astemizole (recalled from US market), calcium channel blockers, quinidine, flecainide, and contraceptives; toxicity increases when administered concurrently with digoxin, flecainide, acetaminophen, clonidine, epinephrine, nifedipine, prazosin, haloperidol, phenothiazines, and catecholamine-depleting agents

Documented hypersensitivity; sinus bradycardia, second-degree and third-degree AV block

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Beta-adrenergic blockade may decrease signs and symptoms of acute hypoglycemia and clinical signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw drug slowly, and monitor patient closely; caution in hypokalemia, peripheral vascular disease, hypomagnesemia, congestive heart failure, and congestive heart failure


Propranolol (Inderal)

Class II antiarrhythmic nonselective beta-adrenergic receptor blocker with membrane-stabilizing activity that decreases automaticity of contractions.
Dose titrated to heart rate between 50-60 bpm.

Adult

20-80 mg PO qid

Pediatric

0.01-0.1 mg/kg/dose IV over 10 min; 1-2 mg/kg bid

Coadministration with aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity; toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines may increase

Documented hypersensitivity; uncompensated congestive heart failure; bradycardia, cardiogenic shock; AV conduction abnormalities

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

Beta-adrenergic blockade may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw drug slowly and monitor closely

Antiarrhythmics

These agents alter the electrophysiologic mechanisms responsible for arrhythmia.


Disopyramide (Norpace)

Decreases inotropic state of left ventricle. Decreases ventricular and supraventricular arrhythmias. Decreases diastolic dysfunction and increases LV compliance, thereby reducing the pressure gradient across the LV outflow tract. Raises threshold for both atrial and ventricular ectopy.

Adult

150-300 mg PO q12h

Pediatric

Not established

Cardiotoxicity may increase when administered concurrently with sparfloxacin, beta-blockers, cimetidine, macrolides, and quinidine; phenytoin and rifampin may decrease levels

Documented hypersensitivity; second-degree or third-degree AV block; acute MI; severe mitral or aortic regurgitation

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

Caution with concomitant use of beta-blockers or verapamil, may precipitate acute congestive heart failure; caution in angle-closure glaucoma, urinary retention, and myasthenia gravis; adjust dose in renal and hepatic impairment


Amiodarone (Cordarone)

Only agent proven to reduce the incidence and risk of cardiac sudden death, with or without obstruction to LV outflow. Very efficacious in converting atrial fibrillation and flutter to sinus rhythm and in suppressing the recurrence of these arrhythmias.

Adult

800-1600 mg PO qd or divided bid/tid for 2 wk, then 200-400 mg qd

Pediatric

Not established

Increases effect and blood levels of theophylline, quinidine, procainamide, phenytoin, methotrexate, flecainide, digoxin, cyclosporine, beta-blockers, and anticoagulants; toxicity of the cardiotoxicity of amiodarone is increased by ritonavir, sparfloxacin, and disopyramide; coadministration with calcium channel blockers may cause an additive effect and further decrease myocardial contractility; cimetidine may increase levels

Documented hypersensitivity; complete AV block, intraventricular conduction defects; patients taking ritonavir or sparfloxacin; pulmonary fibrosis

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

Caution in thyroid or liver disease

Calcium channel blockers

Alternative to beta-blockers, they decrease inotropic state of the left ventricle, decrease gradient across the LV outflow tract, decrease diastolic dysfunction, and increase diastolic filling of the left ventricle by improving LV diastolic relaxation. May have a better effect on exercise performance.


Verapamil (Calan, Isoptin)

During depolarization, inhibits calcium ion from entering slow channels or voltage-sensitive areas of the vascular smooth muscle and myocardium. Alternative to beta-blocker therapy. Useful in patients with moderate-to-severe COPD.
Use of short-acting calcium channel blockers is being discouraged because of numerous reports of adverse cardiac and hemodynamic events associated with their use, particularly in patients with known coronary artery disease.

Adult

SR dosage form: 120-720 mg PO qd
IR dosage form: 80-240 mg PO tid

Pediatric

0.1-0.2 mg/kg/dose IV over 2 min; repeat in 10-30 min prn

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 levels; may increase theophylline levels

Documented hypersensitivity; severe CHF, sick sinus syndrome, second-degree or third-degree AV block, hypotension (<90 mm Hg systolic)

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

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

Natriuretic Peptides

Dilate veins and arteries.


Nesiritide (Natrecor)

Recombinant DNA form of human B-type natriuretic peptides (hBNP), which dilate veins and arteries.
Human BNP binds to particulate guanylate cyclase receptor of vascular smooth muscle and endothelial cells.
Binding to receptor causes increase in cyclic GMP, which serves as second messenger to dilate veins and arteries. Reduces pulmonary capillary wedge pressure and improves dyspnea in patients with acutely decompensated congestive heart failure.

Adult

2 mcg/kg IV bolus over 60 s; follow by 0.01 mcg/kg/min continuous infusion; bolus volume (mL) = 0.33 x patient weight (kg); infusion flow rate of bolus (mL/h) = 0.1 x patient wt (kg)

Pediatric

Not established

Concurrent administration with ACE inhibitors and other vasodilators may cause hypotension

Documented hypersensitivity; systolic blood pressure <90 mm Hg; patients suspected of having or known to have low cardiac filling pressures, significant valvular stenosis, restrictive or obstructive cardiomyopathy, constrictive pericarditis, pericardial tamponade, or conditions in which cardiac output is dependent upon venous return

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

Do not initiate at dose higher than recommended; may affect renal function in patients whose renal function may depend on activity of renin-angiotensin-aldosterone system; may cause hypotension (administer in settings where blood pressure can be monitored closely); discontinue drug if hypotension develops; ventricular tachycardia, nonsustained VT, headache, abdominal pain, back pain, insomnia, anxiety, angina pectoris, nausea, and vomiting may occur

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References
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Further Reading

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Keywords

hypertrophic cardiomyopathy, HCM, hypertrophic obstructive cardiomyopathy, idiopathic hypertrophic subaortic stenosis, IHSS, muscular subaortic stenosis, asymmetric septal hypertrophy, ASH, sudden death, sudden cardiac death, SCD, arrhythmogenic sudden death, myocardial hypertrophy, cardiomyopathy

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Contributor Information and Disclosures

Author

Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice
Michael E Zevitz, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, and Michigan State Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Gary E Sander, MD, PhD, Professor, Department of Internal Medicine, Division of Cardiology, Tulane University Health Sciences Center
Gary E Sander, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Hypertension, Heart Failure Society of America, Louisiana State Medical Society, and Southern Society for Clinical Investigation
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center
Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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