eMedicine Specialties > Radiology > Cardiac
Cardiomyopathy, Hypertrophic: Imaging
Updated: Apr 7, 2009
Radiography
Findings
Chest radiographic findings of hypertrophic cardiomyopathy are variable and nonspecific. The cardiac silhouette can be normal or enlarged. In most cases, cardiomegaly is due to left ventricular hypertrophy and/or left atrial enlargement.7 Significant mitral regurgitation leads to left atrial enlargement.
Degree of Confidence
Cardiomegaly is a nonspecific finding on the chest radiographs. The clinical context, however, may suggest HCM as the cause of cardiomegaly.
Computed Tomography
Findings
Electron-beam CT (EBCT) is an excellent method for observing irregular wall hypertrophy, apical morphology, and wall motion dynamics.38 This modality is seldom used, however, because it entails exposure to radiation and contrast medium and provides less information than MRI. The criterion for LV wall hypertrophy is an LV wall thicker than 13 mm. Right ventricular hypertrophy is considered when the right ventricular wall is thicker than 6 mm.
Wall thickening during systole can be calculated with EBCT. Most patients (71%) have decreased wall thickening at the hypertrophic site and normal or increased thickening at the nonhypertrophic site.38 Late enhancement of the myocardium on EBCT has been reported in approximately 47% of HCM patients39 ; this finding suggests the presence of abnormal tissue with a capillary architecture different from that of normal myocardium. The degree of regional wall thickening also is significantly less in areas of late enhancement, which reflects the abnormal myocardial architecture.40
Magnetic Resonance Imaging
Findings
Spin-echo MRI and cine MRA
Cardiac morphology can be evaluated by using either ECG-gated spin-echo MRI (see Image 1) or cine MRA (see Image 2). The 2 most common views are 4 chamber (see Images 1-2) and short axis (see Images 3-4).41
Cardiomyopathy, hypertrophic. Axial electrocardiographically (ECG) gated spin-echo MRI in a patient shows marked septal (S) and less-prominent posterior wall thickening.
Cardiomyopathy, hypertrophic. Oblique axial cine magnetic resonance angiogram in the same patient as in Image above shows a spade-shaped left ventricle with relative sparing of the apical myocardium (arrow).
Cardiomyopathy, hypertrophic. Short-axis cine end-diastolic magnetic resonance angiogram shows asymmetric hypertrophy with septal thickening (S).
Cardiomyopathy, hypertrophic. Short-axis cine end-systolic, magnetic resonance angiogram obtained in the same patient as in Image above shows marked myocardial thickening that affects the entire myocardium.
Spin-echo MRI can be used to accurately characterize the distribution and degree of myocardial hypertrophy. MRI correlates well with 2-dimensional echocardiography in demonstrating asymmetric septal hypertrophy, and MRI can visualize apical and posterolateral myocardial hypertrophy that is not always evident on 2D echograms.42 . MRI reliably provides accurate, comprehensive data that can be used to calculate hypertrophic scores.43
The hypertrophy in HCM is usually asymmetric and is typically most evident in the anteroseptal myocardium.37 In patients with asymmetric septal hypertrophy, the basal IVS at end diastole is disproportionately thickened (see Images 3-4), and the ratio of IVS thickness to posterolateral wall thickness is significantly increased. Patients with asymmetric septal hypertrophy also have decreased systolic myocardial thickening, probably due to disarray and disorganization of myocardial fibers.5 Long-axis MRIs accurately show the typical spade-shaped deformity of the LV cavity and the apical distribution of myocardial hypertrophy in patients with asymmetric septal hypertrophy (see Image 2).
With MRI, assessment of the thickness of the free wall of the right ventricle and measurement of right ventricular mass are possible; patients with HCM tend to have diffuse hypertrophy of the right ventricular wall and an increased right ventricular wall index.44 LV mass can be reliably estimated with spin-echo MRI, ECG-gated MRI, or multilevel cine MRA; however, LV mass, indexed to body surface area, is normal in about 20% of patients with HCM.45
LVH in HCM often decreases the LV volume and increases the ejection fraction, without significantly changing stroke volume. Cine MRA can be used to calculate these parameters. If volume calculation is performed throughout the cardiac cycle, a time-volume curve can be obtained for more detailed functional analysis.
An obstruction of the LV outflow tract (LVOT) resulting in a subaortic pressure gradient can be detected on cine MRA as signal void (ie, an area of low signal intensity in regions where normal cardiac blood flow produces high signal intensity) (see Image 5). Although areas of physiologic signal void can be seen on scans in healthy individuals, signal voids are larger and persist longer in the cardiac cycle in patients with pathologic conditions that cause obstruction.46 Differentiation of physiologic voids from pathologic ones is rarely difficult.
Cardiomyopathy, hypertrophic. Oblique cine magnetic resonance angiogram (outflow 2-chamber view equivalent to a long-axis echocardiogram) shows an area of signal intensity loss (white arrow) in the left ventricular outflow tract, where an obstruction between the hypertrophied septum and anterior mitral leaflet is present. The obstruction is well below the aortic valve ring (between black arrows).
Cardiomyopathy, hypertrophic. Oblique cine magnetic resonance angiogram (outflow 2-chamber view) shows prolapse (arrow) of the posterior mitral leaflet in early systole.
Cardiomyopathy, hypertrophic. Oblique cine magnetic resonance angiogram (outflow 2-chamber view obtained during the same study as Image above) shows mitral prolapse of the posterior mitral leaflet with a small signal intensity loss due to mitral regurgitation (arrow).
Systolic anterior motion (SAM) of anterior mitral leaflet toward the IVS can be recognized on cine MRAs as a cause of LVOT obstruction. Mitral regurgitation is a common finding in patients with HCM and appears on cine MRAs as a signal void in the left atrium during ventricular systole. It may be associated with mitral valve prolapse (see Images 6-7). Although healthy individuals may have a small round area of physiologic signal void within the left atrium immediately behind and between the 2 mitral leaflets, in patients with pathologic conditions the signal void is larger and persists longer through ventricular systole. The area and extent of the signal void correlates closely with the grade of mitral regurgitation, as estimated with angiography and echocardiography.35,47,48
Myocardial structural abnormality from fiber disarray and disorganization can result in abnormal signal intensity. Fattori et al reported areas of reduced signal intensity, probably due to myocardial fibrosis, in 16 (43%) of 37 unselected patients with HCM.49 This group also had higher maximum septal thickness (25 mm ± 7 vs 21 mm ± 6) and maximum posterior left wall thickness (15 mm ± 9 vs 7 mm ± 8). In patients with HCM, cine MRA also can be used to demonstrate nonuniform regional LV function (ie, LV asynchrony resulting in abnormal diastolic relaxation).
Cardiovascular MRI with gadolinium enhancement can detect myocardial fibrosis.50 Gadolinium hyperenhancement may correlate with progressive ventricular dilation and markers of sudden death.51
Cardiac amyloidosis can resemble HCM; symmetric LV thickening is typical of amyloidosis but also occurs in HCM, and restrictive physiology and poor compliance may be present in both diseases.52 However, amyloidosis tissue may be characterized by its diffuse high signal intensity on T2-weighted spin-echo and short–inversion time inversion recovery (STIR) MRI. The signal intensity with echo times of 20 ms and 60 ms is significantly lower in cardiac amyloidosis than in HCM and in normal tissue.53 Poorer ventricular wall contractility and lower ECG voltages suggest amyloidosis, and a right atrial free wall > 6 mm thick is a specific marker for the disease.53
Magnetic resonance spectroscopy
Contractile dysfunction in HCM is thought to result from alterations in myocardial metabolism. Proton-decoupled phosphorus-31 nuclear magnetic resonance spectroscopy depicts alterations of myocardial metabolism in asymptomatic patients with HCM.54 The ratio of phosphocreatine (PCr) to adenosine triphosphate (ATP) is significantly lower in HCM patients than in healthy control subjects.54 In addition, patients with severe hypertrophy of the IVS have a significantly increased inorganic phosphate (Pi)–to-PCr ratio compared with that of control subjects.54,55,45 Both abnormalities are similar to those found in ischemic myocardia. Also, significantly increased phosphomonoester (PME)-to-PCr ratios are present in patients with HCM; this finding indicates altered glucose metabolism.54 Myocardial pH is lower in patients with HCM relative to that of control subjects.45
MRI myocardial tagging
MRI myocardial tagging can be used to quantify the severity and extent of subtle regional heart wall motion abnormalities. The 3 stages of myocardial tagging are: (1) placement of a saturation band pattern (either a grid or parallel tag lines) over the myocardium with spatially selective RF pulses; (2) MRI acquisition, during which tag motion is observed; and (3) detection of myocardial tag motion.
The motion of the saturation pattern is then used to compute the regional myocardial function. MR tagging techniques offer 2 fundamental improvements over echocardiography and nontagged MRI in regional function assessment: (1) The same volume of myocardium can be tracked throughout the heart cycle to map function in a specific region, and (2) precise quantitative estimates of myocardial shortening and wall thickening can be computed from the images. The position of a myocardial tag can be estimated within approximately 150 µm.
Maier et al found, with myocardial tagging, that the wall motion of the hypertrophied septum was significantly reduced in HCM,56 and Kramer et al showed depressed circumferential myocardial segment shortening in the septum and in the anterior and inferior regions.57 Three-dimensional analysis of tagged images showed that although circumferential and longitudinal ventricular strains were reduced in patients with HCM, the magnitude of the maximal contraction strain was reduced only in the basal septum and anterior walls.58 This finding suggests that a major portion of the mechanical work in HCM contributes to wall shearing and not cavity reduction. Dong et al reported that the myocardium in patients with HCM is heterogeneously thickened and that the fractional thickening and circumferential shortening of the abnormally thickened myocardium are reduced.59
Ultrasonography
Findings
Echocardiography
A major criterion for the echocardiographic diagnosis of HCM is LV wall thickness of ³ 13 mm in the anterior septum or posterior wall or ³ 15 mm in the posterior septum or free wall, in the absence of LV dilatation or other cardiac and systemic causes of increased mass.31 However, no definitive criterion or single echocardiographic feature is pathognomonic for HCM.
The typical echocardiographic feature in HCM is hypertrophy of the septum and LV anterolateral free wall (see Images 8-9); however, the degree and pattern of hypertrophy vary. Maximum hypertrophy of the septum often occurs midway between the base and the apex. On echocardiograms, asymmetric septal hypertrophy is defined as a ratio of septal thickness to posterior wall thickness of at least 1.3-1.5. Although the average LV wall is thicker than 20 mm (ie, almost twice the normal thickness), it can vary from 13-15 mm in mild hypertrophy to 50 mm in massive hypertrophy.8
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).
Cardiomyopathy, hypertrophic. Axial 2-dimensional echocardiogram obtained in the same patient as in Image 8 shows asymmetric septal thickening (23 mm) and a small left ventricular cavity (LV).
Often, the echocardiographic feature of a ground-glass appearance is noted either visually or by using quantitative texture analysis in both hypertrophied and nonhypertrophied regions of the ventricle.60 This feature can be used to distinguish HCM from other causes of secondary hypertrophy.61
Another common echocardiographic feature in HCM is narrowing or obstruction of the LVOT caused by IVS and the anterior leaflet of the mitral valve. The abnormal geometry of LVOT results in a dynamic pressure gradient. Abnormal systolic anterior motion (SAM) of the anterior leaflet (see Images 10-11) and, occasionally, the posterior leaflet of the mitral valve may be present; severe SAM, with septal-leaflet contact, has been proposed as a major diagnostic criterion.31 Mitral valve abnormalities in HCM patients include increased leaflet area, elongation of the leaflet, and anomalous insertion of papillary muscle directly into the anterior mitral leaflet.62 Recognition of these anatomic abnormalities during the preoperative assessment is important.
Cardiomyopathy, hypertrophic. M-mode echocardiogram recorded at the level of the mitral valve shows a small ventricular cavity (arrow) and systolic anterior motion of the anterior mitral valve leaflet (*).
Cardiomyopathy, hypertrophic. M-mode echocardiogram recorded at the level of the mitral valve in a patient with extreme septal hypertrophy (>40 mm) shows a small ventricular cavity (3 cm) and systolic anterior motion of the anterior mitral valve leaflet (*).
Other echocardiographic findings may include a small ventricular cavity, reduced septal motion and systolic thickening, normal or increased motion of the posterior wall, abnormal rate of closure of mitral valve in middiastole (secondary to decreased LV compliance or abnormal transmitral diastolic flow), mitral valve prolapse, and partial systolic closure or coarse systolic fluttering of the aortic valve (see Image 12).
Cardiomyopathy, hypertrophic. M-mode echocardiogram recorded at the level of the aortic valve in the same patient as in Image 8 shows high-frequency flutter on the aortic leaflets (arrows).
Cardiomyopathy, hypertrophic. Continuous-wave Doppler image shows a typical concave profile (arrows) compared with the systolic waveform recorded from the left ventricular outflow; this finding represents subvalvular dynamic outflow obstruction.
Approximately 70% of HCM patients have an LV outflow gradient of ³ 30 mm Hg (2.7 m/s by Doppler).2 Doppler ultrasonography can be used to accurately measure the gradient and to demonstrate the characteristic velocity profile due to dynamic outflow obstruction (see Image 13). Distinguishing obstructive from nonobstructive forms of HCM, on the basis of the presence or absence of an LV outflow gradient, may be critical for the selection of management strategies.2 Although the gradient may be evident when the patient is at rest, in many cases it is latent and can be identified only with exercise.63
Athlete's heart
In the vast majority of competitive athletes, the LV wall is £ 12 mm in thickness. Athletes with an LV wall thicker than 16 mm are likely to have pathologic hypertrophy such as HCM. For the minority of athletes whose LV thickness is in the "gray zone" of 13-15 mm, differentiation of physiologic from pathologic hypertrophy can be problematic. Maron et al published criteria that can help in this distinction.8 Echocardiographic features that suggest HCM are an unusual pattern of LVH, asymmetry, end-diastolic LV dimension <45 mm, left atrial enlargement, and abnormal Doppler diastolic indices of LV filling. Other associated features suggestive of HCM are bizarre ECG findings, female sex, and family history of HCM. An end-diastolic LV dimension >55 mm suggests athlete's heart, as does regression of hypertrophy within 3 months after cessation of exercise.8
Dilation of the LV chamber
LV chamber dilatation and systolic dysfunction occur in about 1.5% of patients of HCM per year.64 This dilatation can evolve into a phase resembling dilated cardiomyopathy.
False Positives/Negatives
Prasad et al have reviewed the pitfalls in the echocardiographic diagnosis of HCM, which include the potential for both false-positive and false-negative readings.65
Nuclear Imaging
Findings
201 TI myocardial tests
201 TI myocardial tests, particularly those with SPECT, permit direct determination of the relative thickness of septum and free wall, and they may be useful in cases in which echocardiography is technically limited. Typically, Tl-enhanced images demonstrate a small LV cavity with marked Tl uptake in the hypertrophied myocardium.
Cardiomyopathy, hypertrophic. Stress (top row) and rest (bottom row) technetium-99m Sesta-2-methoxy-isobutyl-isonitrile (MIBI) perfusion images of hypertrophic cardiomyopathy shows a reversible septal perfusion defect that is not related to coronary obstruction. The septum is markedly thickened (4 cm on the echocardiogram).
Reversible perfusion defects (see Image 14), which presumably reflect myocardial ischemia, are common in HCM without coronary artery disease.66 These defects are common in adult patients with HCM and in young patients with a history of syncope and sudden death.24 Fixed defects occur in patients with impaired systolic function and likely represent myocardial scarring. Also, technetium-99m–labeled perfusion agents can be used, with similar results.
Gated radionuclide ventriculography
Gated radionuclide ventriculography with bloodpool labeling permits evaluation of the size and diastolic filling of the ventricular cavity and of the motion of the septum and ventricular wall.
Myocardial scintigraphy
Myocardial scintigraphy with iodine-123– m -iodobenzylguanidine (123 I-MIBG) demonstrates decreased uptake and increased clearance in the hypertrophied myocardium, and it has shown that cardiac sympathetic activity correlates with the degree of hypertrophy function in HCM patients.67 Scintigraphy results have proved useful for predicting prognosis in HCM.68
Positron emission tomography
In Japanese patients, PET studies performed with123 I-labeled 15-(p -iodophenyl)-3-R,S -methylpentadecanoic acid (BMIPP) suggest that fatty acid metabolism is impaired in areas of myocardium affected by HCM and that BMIPP studies may be useful in classifying HCM and assessing its severity.69
Angiography
Cardiomyopathy, hypertrophic. Pressure tracing obtained as the catheter is pulled back from the center of the left ventricle to the aortic root shows a reduction in systolic pressure (arrow 1) in the left ventricle; this finding indicates a subaortic gradient. The waveform changes at the level of the aortic valve, but the systolic pressure does not change (arrow 2). Note the spike-and-dome configuration of the left ventricular pressure tracing.
Cardiomyopathy, hypertrophic. End-diastolic right anterior oblique digital subtraction left ventriculogram shows the normal size and shape of the left ventricle in a patient with hypertrophic cardiomyopathy.
Cardiomyopathy, hypertrophic. End-diastolic right anterior oblique digital subtraction left ventriculogram obtained in the same study as Image above shows a small cavity, with prominent papillary muscles (arrows) projecting into the remains of the ventricular cavity.
Cardiomyopathy, hypertrophic. The 2 images above are used to calculate function and left ventricular dimensions. The outline of the end-diastolic image (Image 16 in Multimedia) has been superimposed on the systolic image (Image 17 in Multimedia). Ejection fractions were calculated by using the area-length method (ejection fraction, 86%) and the Simpson rule (ejection fraction, 84%). The videodensitometric technique shown is inaccurate because of incorrect background registration.
Cardiomyopathy, hypertrophic. Conventional end-diastolic right anterior oblique left ventriculogram acquired during cardiac catheterization shows the normal size and shape of the left ventricle in a patient with hypertrophic cardiomyopathy. Note the distance between the ventricular cavity and the coronary arteries (arrows), which define the epicardial surface of the heart. This distance indicates considerable thickening of the myocardium.
Cardiomyopathy, hypertrophic. Conventional end-systolic right anterior oblique left ventriculogram acquired during the same cardiac catheterization study as in Image above shows a small left-ventricular cavity with mild mitral regurgitation (M). Note the increased distance between the ventricular cavity and the coronary arteries (arrows), which define the epicardial surface of the heart as the myocardium becomes thickened in systole.
Cardiomyopathy, hypertrophic. Conventional right anterior oblique aortogram acquired during cardiac catheterization in the same patient as in Image above shows unobstructed coronary arteries.
Findings
Cardiac catheterization demonstrates decreased LV compliance and, in some patients, a subaortic systolic pressure gradient (see Image 15). The pressure gradient may be labile, varying 0-175 mm Hg in the same patient under different conditions. Increased myocardial contractility can worsen the gradient, particularly in patients with midventricular gradient, because of a direct muscular sphincteric action. Conversely, reduction in contractility or increases in preload or afterload (which increase the LV cavity size) reduce or eliminate the outflow gradient. This dynamic characteristic of HCM distinguishes it from other forms of ventricular outflow obstruction.
The arterial pressure tracing may demonstrate a spike-and-dome configuration (see Image 15). Approximately 25% patients have pulmonary hypertension, at least partly due to decreased LV compliance and elevated left atrial pressure. A right ventricular outflow tract pressure gradient occurs in 15% of patients who have LVOT obstruction, and this likely results from a markedly hypertrophied right ventricle.70
Left ventriculography reveals a hypertrophied ventricle with vigorous ejection (see Images 16-20). The papillary muscles often are prominent, filling the LV cavity at the end of systole. In patients with apical involvement, extensive hypertrophy may result in a spadelike configuration of the LV cavity.71 Associated mitral regurgitation may be present (see Image 18, Image 20). Simultaneous right ventriculography in cranially angulated left anterior oblique projections can be performed for optimal evaluation of the IVS. The left septal surface is flat or it bulges into the LV cavity at its middle or lower portion, in contrast to the normal curve toward the right ventricle.
Coronary angiographic findings usually are normal, but images may show myocardial bridging. The distance between the coronary arteries on the epicardial surface and the ventricular cavity is increased, indicating myocardial hypertrophy (see Images 19-21).
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Further Reading
Related eMedicine topics
Cardiomyopathy, Hypertrophic (Cardiology)
Cardiomyopathy, Hypertrophic (Pediatrics)
Atrial Fibrillation
Sudden Cardiac Death
Aortic Stenosis
Clinical guidelines
ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices).
American College of Cardiology Foundation
American Heart Association
Heart Rhythm Society. 1998 Apr (revised 2008 May 27). 62 pages. NGC:006498
Clinical trials
Comparison of Data Obtained by Echocardiography and Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy
Use of Magnetic Field Mapping in the Evaluation of Patients With Hypertrophic Heart Disease (Thick Heart Muscle)
Antiarrhythmic Therapy Versus Catheter Ablation for Atrial Fibrillation in Hypertrophic Cardiomyopathy
Genetic Predictors of Outcome in HCM Patients
Keywords
hypertrophic cardiomyopathy, idiopathic hypertrophic subaortic stenosis, IHSS, asymmetric septal hypertrophy, muscular subaortic stenosis, hypertrophic obstructive cardiomyopathy, HOCM, HCM
