eMedicine Specialties > Radiology > Cardiac

Cardiomyopathy, Hypertrophic

Author: Diwaker Agarwal, MD, Staff Physician, Department of Radiology, Mercy Medical Center
Coauthor(s): George Hartnell, MB, Professor of Radiology, Tufts University School of Medicine, Director of Cardiovascular and Interventional Radiology, Department of Radiology, Baystate Medical Center
Contributor Information and Disclosures

Updated: Apr 7, 2009

Introduction

Background

Hypertrophic cardiomyopathy (HCM) consists of genetically abnormal, usually hypercontractile and asymmetric myocardium that may obstruct output and cause sudden death if the hypertrophy is localized in the upper septum.

Cardiomyopathy, hypertrophic. Axial electrocardio...

Cardiomyopathy, hypertrophic. Axial electrocardiographically (ECG) gated spin-echo MRI in a patient shows marked septal (S) and less-prominent posterior wall thickening.

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Cardiomyopathy, hypertrophic. Axial electrocardio...

Cardiomyopathy, hypertrophic. Axial electrocardiographically (ECG) gated spin-echo MRI in a patient shows marked septal (S) and less-prominent posterior wall thickening.


Cardiomyopathy, hypertrophic. M-mode echocardiogr...

Cardiomyopathy, hypertrophic. M-mode echocardiogram recorded at the level of the tips of the mitral valve (horizontal arrows) to assess left ventricular dimensions shows moderate thickening of both the septum (S) and posterior wall of the left ventricle (PW).

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Cardiomyopathy, hypertrophic. M-mode echocardiogr...

Cardiomyopathy, hypertrophic. M-mode echocardiogram recorded at the level of the tips of the mitral valve (horizontal arrows) to assess left ventricular dimensions shows moderate thickening of both the septum (S) and posterior wall of the left ventricle (PW).


The disease includes asymmetric septal hypertrophy and idiopathic hypertrophic subaortic stenosis (IHSS), but the term HCM is preferred because the majority (75%) of patients do not present with obstruction at rest,1 and 30% do not exhibit asymmetric hypertrophy.

Pathophysiology

Hypertrophic cardiomyopathy (HCM) is usually inherited as an autosomal dominant trait involving genes that encode protein constituents of the cardiac sarcomere.2 Although 450 different mutations have been identified within 13 genes,3 three genes probably account for more than half of genotyped cases: those that encode beta-myosin heavy chain (chromosome 14), myosin-binding protein C (chromosome 11) and cardiac troponin-T (chromosome 1).2  

On pathologic examination of involved myocardium, the myofibrils are abnormally short, broad, and hypertrophied; in addition, they may run in different directions, with complex intercellular bridging resulting in the formation of whorls.4,5,6

The left ventricle (LV) is usually more involved in hypertrophy than is the right ventricle. The atria may be dilated, and they are often hypertrophied. The characteristic feature is disproportionate thickening of the interventricular septum (IVS) and the anterolateral wall of the LV compared with the posterior free wall.7

Other patterns include concentric hypertrophy; this is sometimes difficult to differentiate from physiologic hypertrophy, which occurs in some highly trained athletes.8,9 Some patients have significant hypertrophy in unusual locations, such as the posterior portion of the septum, the posterobasal free wall of the LV, or at the midventricular level.7 One unusual type involves marked posterior wall hypertrophy and virtually no septal hypertrophy. These patients are young and have severe symptoms.10

HCM with predominant involvement of apex is especially common in Japan and China. Hypertensive HCM in elderly patients is characterized by severe concentric LV hypertrophy (LVH), a small LV cavity, and hypertension.11,12 It may look similar to symmetric HCM, but it responds better to beta blockers at doses sufficient to control the hypertension, and patients have a better prognosis.

HCM impairs diastolic relaxation. This impairment in relaxation can result in symptoms of heart failure despite a normal and usually supernormal ejection fraction due to high filling pressures, which result in pulmonary congestion. During systole, approximately 25% of patients have LV outflow obstruction with a dynamic pressure gradient secondary to systolic anterior motion of mitral valve, which further narrows an outflow tract that is already diminished because of septal hypertrophy.

Myocardial ischemia is also common in HCM despite normal epicardial coronary arteries. The causes are multifactorial and include increased muscle mass, inadequate capillary density, elevated diastolic filling pressure, abnormal intramural coronary arteries, impaired vasodilatory reserve, systolic compression of ventricles, and increased myocardial oxygen demand secondary to increased stress.1

Frequency

United States

Hypertrophic cardiomyopathy is perhaps the most common genetic cardiac disease, with a prevalence of 0.1-0.2% (1 in 500 to 1 in 1000 adults).7,13 However, the incidence may be higher in select populations.

International

In Japan, Kibira et al reported a prevalence of 170-574 cases per 100,000 population with mass screening.14  The proportion of hypertrophic cardiomyopathic patients with predominant involvement of the apex can vary depending on the population, but frequencies of apical HCM of 41% in China15 and 23% in Japan16 have been reported.

Mortality/Morbidity

The annual mortality among patients with hypertrophic cardiomyopathy (HCM) is approximately 1% when all patients are included, although it is about 3% in large referral centers, which tend to have more severe cases.7

  • Clinical progression: The clinical course of HCM is variable. In many patients, symptoms are absent or mild. Other patients experience progressive exertional dyspnea, chest pain, and episodes of impaired consciousness, with preserved LV systolic function but diastolic dysfunction.2
  • Atrial fibrillation (AF): AF has been reported in 20-25% of HCM patients; chronic atrial fibrillation in particular is associated with substantial morbidity and mortality.7,17 In general, the rate of clinical deterioration is slow, and symptoms are poorly related to the severity of hypertrophy or the gradient.7   however, the prevalence of severe symptoms increases in older patients.18
  • End-stage HCM: In a small minority (5-10%) of patients, HCM progresses to advanced congestive heart failure with LV remodeling and systolic dysfunction.7,2  Patients with end-stage HCM have an annual mortality of 11% and increased risk of sudden death.19
  • Sudden death: In young persons—young athletes in particular—HCM is the most common cause of sudden cardiac death.20,21  In children with HCM, the risk of sudden death can be as high as 6% per year.22  Most sudden deaths are thought to be due to complex ventricular tachyarrhythmias generated by electrically unstable myocardium, with ventricular fibrillation probably being the most common.2 Ventricular arrhythmias and inducibility at electrophysiologic testing are less common in children than in older adults, however, which suggests a different mechanism for sudden death in children.23 Ischemia may play a role in these cases.24,25

Race

Apical hypertrophic cardiomyopathy is especially common in Japan and China (see also Frequency above). In one study, African Americans accounted for only 8% of all clinically identified HCM patients but for 55% of sudden cardiac deaths among young competitive athletes.26

Sex

Women with hypertrophic cardiomyopathy have been found to be older and more symptomatic than male patients at initial evaluation, more likely to have left ventricular outflow obstruction, and to be at higher risk of progression to advanced heart failure or death.27,28

Age

Left ventricular hypertrophy (LVH) usually develops in persons 5-15 years of age.23 Sudden death occurs more commonly in those 12-35 years of age or in those older than 65 years. LVH rarely occurs in children 10 years old or younger.

Presentation

Most patients with hypertrophic cardiomyopathy (HCM) are either asymptomatic or only mildly symptomatic. Such cases are often identified during screening of relatives of known patients with HCM.

Clinical presentation may occur at any age.7 Patients may first present with exertional dyspnea, angina, syncope, or atrial fibrillation and systemic embolism. Dyspnea is the most common symptom, occurring in 90% of symptomatic patients.7 Angina pectoris occurs in about 75% of symptomatic patients. Fatigue, syncope, and presyncope (graying-out spell) are also common. Sudden death can be the first clinical manifestation; it is common in children and young adults and often occurs during or after physical exertion.29

Most patients with gradients have a double or triple apical impulse, a rapidly rising carotid arterial pulse, and a fourth heart sound.29  A tall A-wave on venous pulsations reflects impaired diastolic relaxation, as does S3 and/or S4. The apical precordial impulse may be shifted laterally, and it is usually forceful and enlarged.

The auscultatory hallmark of HCM is a harsh midsystolic murmur that is best heard between the apex and left sternal border and that commences well after the first heart sound. The murmur becomes louder with a Valsalva maneuver and standing, unlike most other murmurs (except that of mitral valve prolapse). Likewise, vasodilators, dehydration, and inotropes increase the murmur. The potentiated beat after an extra systole also increases the outflow gradient. The murmur often decreases with a hand-grip exercise.

Mitral regurgitation often accompanies HCM, resulting in a holosystolic apical murmur. The murmur of aortic regurgitation occurs in 10% of patients, although Doppler echocardiography shows mild aortic regurgitation in as many as one third of patients.30

Preferred Examination

  • Echocardiography: 2-dimensional echocardiography is the usual method of diagnosis.  Echocardiography can be used to confirm the size of the heart, the pattern of ventricular hypertrophy, the contractile function of the heart, and the severity of the outflow gradient. It has the advantages of high resolution and no known risk. Criteria for echocardiographic diagnosis of hypertrophic cardiomyopathy (HCM) have been proposed.31 Initial studies of 3-dimensional echocardiography suggest that this technique is superior to 2-dimensional echocardiography for the evaluation of HCM.32,33
  • MRI: The high contrast resolution of ECG-gated MRI provides excellent information about cardiac anatomy. Spin-echo MRI or cine magnetic resonance angiography (MRA) can be used to demonstrate ventricular anatomy and wall thickness. Cine MRA is used to evaluate ventricular function, ventricular end-diastolic and end-systolic volumes, valvular dysfunction, and outflow tract obstruction. In some cases, the signal intensity through the thickened myocardium varies. A major development in MRI is myocardial tagging, which involves localized radiofrequency (RF) saturation of myocardial tissue before image acquisition to permit monitoring of the progressive distortion of the myocardial wall during the cardiac cycle.34 It can provide unique information about regional myocardial strain and function, and it is particularly useful in diseases with regional heterogeneity such as HCM.
  • Electrocardiography (ECG): Findings on 12-lead ECG are abnormal in 75-95% of HCM patients.35 Common abnormalities are LVH and widespread, deep, Q waves, which suggest an old myocardial infarction. Many patients have arrhythmias, both atrial and ventricular. ECGs are useful principally for suggesting the possibility of HCM in relatives of HCM patients and in athletes undergoing preparticipation screening.
  • Chest radiography: The cardiac silhouette can vary from normal to markedly enlarged in rare cases.
  • Thallium-201 myocardial imaging: This test, particularly with single photon emission CT (SPECT) for cross-sectional imaging, can be used to assess myocardial perfusion and the relative thickness of the IVS and free ventricular walls. Gated radionuclide ventriculography permits evaluation of ventricular size, ejection fraction, and septal and wall motion.
  • Positron emission tomography (PET): This test can be used as an early diagnostic tool.
  • ECG-gated CT: This test can be used to evaluate the patterns of LVH and wall motion in HCM.
  • Cardiac catheterization and angiography: These can be performed to evaluate hemodynamic and morphologic abnormalities associated with HCM, along with associated coronary artery anomalies. However, these are invasive procedures and should be used only if other tests cannot provide adequate information or if alcohol ablation of septal branches is planned (see Intervention).
  • Electrophysiologic studies (EPS): The role of EPS in identifying HCM patients at risk of sudden death is controversial.7 The predictive value of inducible sustained ventricular arrhythmias during EPS is low.21
  • Magnetic resonance spectroscopy: This is a tool for the evaluation of cardiac metabolism with direct measurement of ischemia-induced changes of high-energy phosphates and intracellular pH.36 The technique is still in the research phase.

Limitations Of Techniques

Echocardiography may at times be limited by poor acoustic windows, incomplete visualization of the left ventricular wall, and inaccurate evaluation of left ventricular mass. Echocardiography is less accurate than MRI in  evaluating wall thickness, especially of the anterolateral LV; it is also less accurate in assessing regional wall motion abnormalities, aneurysms, and delayed enhancement.37

Differential Diagnoses

Amyloidosis, Overview
Aortic Stenosis

Other Problems to Be Considered

Hypertensive heart disease
Subaortic membrane

More on Cardiomyopathy, Hypertrophic

Overview: Cardiomyopathy, Hypertrophic
Imaging: Cardiomyopathy, Hypertrophic
Follow-up: Cardiomyopathy, Hypertrophic
Multimedia: Cardiomyopathy, Hypertrophic
References
Further Reading
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Keywords

hypertrophic cardiomyopathy, idiopathic hypertrophic subaortic stenosis, IHSS, asymmetric septal hypertrophy, muscular subaortic stenosis, hypertrophic obstructive cardiomyopathy, HOCM, HCM

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Contributor Information and Disclosures

Author

Diwaker Agarwal, MD, Staff Physician, Department of Radiology, Mercy Medical Center
Diwaker Agarwal, MD is a member of the following medical societies: American College of Radiology, American Medical Association, and Radiological Society of North America
Disclosure: Nothing to disclose.

Coauthor(s)

George Hartnell, MB, Professor of Radiology, Tufts University School of Medicine, Director of Cardiovascular and Interventional Radiology, Department of Radiology, Baystate Medical Center
George Hartnell, MB is a member of the following medical societies: American College of Cardiology, American College of Radiology, American Heart Association, Association of University Radiologists, British Institute of Radiology, British Medical Association, Massachusetts Medical Society, Radiological Society of North America, Royal College of Physicians, Royal College of Radiologists, and Society of Cardiovascular and Interventional Radiology
Disclosure: Nothing to disclose.

Medical Editor

Justin D Pearlman, MD, PhD, ME, MA, Director of Advanced Cardiovascular Imaging, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Justin D Pearlman, MD, PhD, ME, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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