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

Cardiomyopathy, Hypertrophic: Follow-up

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

Follow-up

Further Inpatient Care

  • Admit patients with hypertrophic cardiomyopathy (HCM) for testing, electrophysiology procedures, and/or surgical intervention.

Further Outpatient Care

  • Carefully monitor medication dose and adverse effects.
  • Children should be followed by serial echocardiography for development or progression of left ventricular (LV) obstruction.

Inpatient & Outpatient Medications

  • Medications include beta-blockers, calcium channel blockers such as verapamil or diltiazem, and, rarely, amiodarone.
  • Avoid administration of inotropic drugs.
  • Avoid nitrates and sympathomimetic amines except in patients with hypertrophic cardiomyopathy and concomitant coronary artery disease.
  • Avoid digitalis because glycosides are contraindicated, except in patients with uncontrolled atrial fibrillation.
  • Avoid diuretics because of their effect on LV myotomy and ventricular volume.

Transfer

  • Transfer may be required for further diagnostic evaluation and electrophysiologic device or surgical intervention.

Deterrence/Prevention

  • Advise individuals with hypertrophic cardiomyopathy to avoid strenuous activity, anaerobic exercise such as weightlifting, and high-level competitive sports.

Complications

  • Congestive heart failure
  • Arrhythmia
  • Infective mitral endocarditis
  • Atrial fibrillation with mural thrombosis formation
  • Sudden death

Prognosis

  • The mortality rate for children with hypertrophic cardiomyopathy is 1% per year. Sudden death is the most common cause and certain children may be at higher risk.
  • Hypertrophic cardiomyopathy is a chronic illness with lifestyle restrictions.

Patient Education

  • Family members of persons with hypertrophic cardiomyopathy should learn cardiopulmonary resuscitation (CPR).
  • Refer the family and patient with hypertrophic cardiomyopathy for psychosocial counseling.
  • Impose activity restrictions for individuals with hypertrophic cardiomyopathy.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Palpitations.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize associated conditions, such as essential hypertension, mitral regurgitation, mitral valve prolapse, and angina pectoris
  • Failure to inform family and patient with hypertrophic cardiomyopathy (HCM) of exercise restrictions
  • Failure to recognize signs and symptoms of hypertrophic cardiomyopathy
  • Failure to screen (or recommend screening of) first-degree relatives once an index case of hypertrophic cardiomyopathy is identified
  • Failure to effectively treat or prevent malignant arrhythmias
  • Failure to have serious discussions regarding the risks, benefits, and decisions regarding the implantation of a cardioverter defibrillator

Special Concerns

  • Children with hypertrophic cardiomyopathy may not be symptomatic. Careful evaluation of a heart murmur may reveal hypertrophic cardiomyopathy.
  • Careful screening of first-degree relatives should be recommended, including physical examination, ECG, and echocardiography.
 
Acknowledgments

The authors gratefully acknowledge Dr. Steven Colan for his expert advise while updating this topic.



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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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