DOI:10.2214/AJR.07.3997
AJR 2008; 191:1702-1710
© American Roentgen Ray Society
MRI of Cardiomyopathy
Elena Belloni1,
Francesco De Cobelli1,
Antonio Esposito1,
Renata Mellone1,
Gianluca Perseghin2,
Tamara Canu1 and
Alessandro Del Maschio1
1 Department of Radiology, Vita-Salute University, San Raffaele Scientific
Institute, Via Olgettina, 60, 20132, Milan, Italy.
2 Internal Medicine Section of Nutrition and Metabolism, Vita-Salute University,
San Raffaele Scientific Institute, Milan, Italy.
Received March 18, 2008;
accepted after revision July 8, 2008.
Address correspondence to E. Belloni
(belloni.elena{at}hsr.it).
CME
This article is available for CME credit. See
www.arrs.org
for more information.
Abstract
OBJECTIVE. The aims of this article are to present the main features
of MRI of cardiomyopathy and to show selected images of cardiomyopathies.
CONCLUSION. Cardiomyopathy is a frequent reason for cardiac MRI
evaluation, which is now considered the most appropriate imaging technique for
the diagnosis and follow-up of this wide range of myocardial diseases.
Keywords: cardiac imaging • cardiomyopathy • MRI
Introduction
Cardiomyopathies (CMPs) are myocardial diseases associated with cardiac
dysfunction. They are classified as dilated CMP, hypertrophic CMP, restrictive
CMP, arrhythmogenic right ventricular (RV) CMP, specific CMP, and
nonclassified CMP [1].
Cardiac MRI has become an important imaging technique for the diagnosis and
follow-up of CMP. In fact, echocardiography, usually the first step in CMP
evaluation, has some pitfalls, mainly its limited acoustic window. On the
contrary, cardiac MRI allows a reproducible and accurate evaluation of
myocardial morphology, function, perfusion, and tissue damage in a noninvasive
and "one-stop shop" way
[2,
3]. For these reasons, cardiac
MRI has become an important diagnostic tool for CMP and is the new reference
standard for the assessment of cardiac function.
Examples of the use of cardiac MRI are the pre- and posttherapy evaluation
of hypertrophic and dilated CMPs, the differential diagnosis between
restrictive CMP and constrictive pericarditis, the assessment of myocardial
damage in acute and chronic CMP, and the evaluation of myocardial involvement
in systemic diseases such as amyloidosis and sarcoidosis.
Black blood imaging is the first step in a cardiac MRI evaluation because
it allows reliable assessment of morphology as a result of its high spatial
resolution and soft-tissue contrast. Cine imaging is important in the
evaluation of cardiac volumes and kinesis and is now considered the reference
standard for the assessment of cardiac function. Transvalvular flow can be
studied by means of phase-contrast sequences. Late-enhancement imaging is
performed after the IV administration of gadolinium and is fundamental in the
characterization of myocardial tissue abnormalities in CMP. In this article,
we present the main features of cardiac MRI and selected images of CMP.
Cardiac MRI Study
Our cardiac MRI protocol is performed on a 1.5-T scanner (Achieva Nova,
Philips Healthcare) with maximum gradient strength of 33 mT/m and a 5-element
phased-array coil (SENSE [sensitivity encoding] Cardiac, Philips Healthcare).
The study usually includes morphologic fast spin-echo black blood sequences
with and without fat suppression, cine single-shot free-precession
(steady-state free-precession) sequences, phase-contrast sequences, and
late-enhancement 3D T1-weighted fast-field echo inversion recovery sequences
obtained 10–15 minutes after the IV administration of 0.2 mmol/kg of a
gadolinium-based contrast agent. All images are obtained with breath-holding
along the two-chamber plane, the four-chamber long-axis plane, and the
short-axis plane.
Function evaluation is implemented on the cine short-axis images,
encompassing the left ventricle (LV) and RV from base to apex (8–12
contiguous slices) to obtain a volumetric evaluation using a dedicated
workstation (ViewForum, Philips Healthcare)
[3].
Dilated Cardiomyopathy
Dilated CMP is associated with dilatation and dysfunction of the LV or of
both ventricles. Ventricles can have normal or thin walls but always have
increased cavitary volumes and low ejection fractions (EFs). Atrial dilatation
and valvular dysfunction may be associated.
The clinical presentation of dilated CMP is usually characterized by
progressive cardiac failure, and the long-term prognosis is poor
[4]. The cause is not well
understood and, although a number of cases are considered to be idiopathic, it
is now recognized that other cases of the disease may have ischemic, genetic
or familial, viral, immune, or a toxic origin, or can be secondary to
cardiovascular diseases with myocardial dysfunction that is not explained by
ischemic damage or increased volumetric loads
[1,
5].
In black blood images, enlarged cardiac chambers and thin myocardial walls
are evident. Cine images usually show LV hypokinesia and increased volumes.
Roughly, the end-diastolic volumes that constitute a dilated CMP are more than
140 mL for the LV and more than 150 mL for the RV; these data may be more
accurate if indexed to the body surface area
[6]. Phase-contrast sequences
may show impaired diastolic function of one or both ventricles. In particular,
the transvalvular flow may be characterized by a restrictive pattern, with a
narrow blood inflow jet in early diastole and an early peak–atrial peak
ratio > 2, or by an early peak–atrial peak ratio < 1, due to the
early diastolic filling decrease and compensatory atrial contraction
[7,
8].
A cardiac MRI study in dilated CMP should always include late-enhancement
images, which are an important element in tissue characterization and can help
differentiate between dilated CMP secondary to coronary artery disease and
other causes of dilated CMP. In fact, McCrohon et al.
[9] reported that 41% of
patients with dilated CMP showed late-enhancement areas in the myocardial
walls. This 41% consisted of 13% with a pattern (subendocardial and
transmural) that cannot be distinguished from the typical ischemic pattern,
and 28% with a mesocardial distribution of late-enhancement areas; therefore,
the differentiation between these subgroups may be fundamental in the
therapeutic and prognostic approach to the patients. Moreover, De Cobelli et
al. [10] published a study
concerning the role of late-enhancement imaging in the assessment of heart
failure secondary to biopsy-proven chronic myocarditis; the possible
persistence of autoimmune inflammatory processes may give rise to typical
late-enhancement patterns, which again are useful in the diagnostic,
therapeutic, and prognostic management of patients with chronic myocarditis
that may evolve toward dilated CMP.
Cardiac MRI with late-enhancement sequences seems to have a role also in
the evaluation of the degree of fibrosis and of the prognostic significance of
the fibrosis itself in patients with dilated CMP. Testing the hypothesis that
fibrosis in dilated CMP might predict outcome, Assomull et al.
[11] used cardiac MRI—in
particular, late-enhancement imaging—to study a group of patients with
dilated CMP and found that 35% of these patients had midwall myocardial
fibrosis, which is a predictor of the combined end point of all-cause
mortality and cardiovascular hospitalization, and also of sudden cardiac death
and ventricular tachycardia. These results perhaps suggest that cardiac MRI
has a potential role in risk stratification of patients with dilated CMP
(Figs. 1A,
1B, and
1C).
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Fig. 1A —19-year-old woman who has had dilated cardiomyopathy
secondary to viral myocarditis since the age of 10 years. Patient experienced
frequent arrhythmias. Morphologic T2-weighted black blood four-chamber
long-axis image with fat suppression shows enlargement of both left ventricle
(LV) (LV end-diastolic volume, 160 mL) and left atrium (arrows). No
alterations in myocardial wall signal intensity were detected.
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Fig. 1B —19-year-old woman who has had dilated cardiomyopathy
secondary to viral myocarditis since the age of 10 years. Patient experienced
frequent arrhythmias. Late-enhancement short-axis (B) and four-chamber
long-axis (C) images shows mesocardial striae of hyperenhancement
located in basal interventricular septum and posteroinferior and lateral LV
walls (arrows).
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Fig. 1C —19-year-old woman who has had dilated cardiomyopathy
secondary to viral myocarditis since the age of 10 years. Patient experienced
frequent arrhythmias. Late-enhancement short-axis (B) and four-chamber
long-axis (C) images shows mesocardial striae of hyperenhancement
located in basal interventricular septum and posteroinferior and lateral LV
walls (arrows).
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Hypertrophic Cardiomyopathy
The main feature of hypertrophic CMP is LV wall thickening, more often
asymmetric and involving the interventricular septum
[1]. A marker of hypertrophic
CMP may be LV outflow tract obliteration, with the occurrence of systolic
gradients (obstructive form) (Figs.
2A,
2B,
2C,
2D, and
2E and Fig. S2E in supplemental
data at
www.ajronline.org).
LV EF can be normal or increased, whereas cavitary volumes can be normal or
reduced. Diastolic function may also be impaired because of the altered
ventricular compliance. Valve insufficiency may coexist.
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Fig. 2A —60-year-old woman with symmetric hypertrophic cardiomyopathy
who underwent cardiac MRI before surgical intervention for mitral valve
regurgitation and left ventricle (LV) outflow tract obliteration. See also
Figure S2E, cine loop, in supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood two-chamber long-axis image shows diffuse
hypertrophy of left and right ventricle walls (myocardial mass, 307 g)
(arrows).
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Fig. 2B —60-year-old woman with symmetric hypertrophic cardiomyopathy
who underwent cardiac MRI before surgical intervention for mitral valve
regurgitation and left ventricle (LV) outflow tract obliteration. See also
Figure S2E, cine loop, in supplemental data at
www.ajronline.org.
Static cine four-chamber long-axis image shows LV outflow tract obstruction in
systolic phase (arrow).
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Fig. 2C —60-year-old woman with symmetric hypertrophic cardiomyopathy
who underwent cardiac MRI before surgical intervention for mitral valve
regurgitation and left ventricle (LV) outflow tract obliteration. See also
Figure S2E, cine loop, in supplemental data at
www.ajronline.org.
Static cine two-chamber long-axis image shows moderate to severe mitral valve
insufficiency and regurgitant jet (arrow).
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Fig. 2D —60-year-old woman with symmetric hypertrophic cardiomyopathy
who underwent cardiac MRI before surgical intervention for mitral valve
regurgitation and left ventricle (LV) outflow tract obliteration. See also
Figure S2E, cine loop, in supplemental data at
www.ajronline.org.
Analysis of phase-contrast image plot shows altered LV diastolic filling
pattern with early peak–atrial peak ratio < 1.
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Fig. 2E —60-year-old woman with symmetric hypertrophic cardiomyopathy
who underwent cardiac MRI before surgical intervention for mitral valve
regurgitation and left ventricle (LV) outflow tract obliteration. See also
Figure S2E, cine loop, in supplemental data at
www.ajronline.org.
Late-enhancement short-axis image shows diffuse signal hyperintensity after
contrast administration, mainly involving subendocardial and mesocardial
aspects of lateral LV wall (arrows).
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In most cases, the cause of hypertrophic CMP is genetic or familial, with a
50% autosomal-dominant hereditary pattern, even if with variable expression
[12]. Patients are usually
asymptomatic or mildly symptomatic, even if sudden death is a possibility
[13–15].
In black blood images, increased LV wall thickness is usually evident. Cine
imaging allows the assessment of LV systolic function, which can be increased
with reduced volumes, and the evaluation of outflow tract obstruction.
Impaired diastolic function is well investigated using phase-contrast
sequences. In particular, evaluation of transvalvular flow may depict
decreased LV compliance, with a narrow blood inflow jet in early diastole and
an early peak–atrial peak ratio > 2
[7].
The myocardium in patients with hypertrophic CMP is histologically
characterized by fibrotic scars and signs of myocardial microischemia
[13]. These phenomena have a
correlation with cardiac MRI because they are seen in the late-enhancement
areas, the extent of which is associated with the progression and seriousness
of the disease; and an increased risk of sudden death exists because
myocardial scarring can be the substrate for fatal arrhythmia
[13,
15,
16]. Therefore,
late-enhancement imaging may have a fundamental role in risk stratification in
patients with hypertrophic CMP, which has important prognostic and therapeutic
implications [17] (Figs.
3A,
3B,
3C,
3D,
3E, and
3F and Fig. S3G in
supplemental data at
www.ajronline.org).
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Fig. 3A —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood short-axis images show marked thickening
of interventricular septum and of midbasal aspects of left ventricle (LV)
anterior wall (arrows).
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Fig. 3B —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood short-axis images show marked thickening
of interventricular septum and of midbasal aspects of left ventricle (LV)
anterior wall (arrows).
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Fig. 3C —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Static cine short-axis images in end-diastolic (C) and end-systolic
(D) phases in midventricular plane show hypokinesia of septum and of
midbasal anterior LV wall, where hypertrophy is located (arrows).
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Fig. 3D —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Static cine short-axis images in end-diastolic (C) and end-systolic
(D) phases in midventricular plane show hypokinesia of septum and of
midbasal anterior LV wall, where hypertrophy is located (arrows).
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Fig. 3E —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Late-enhancement short-axis images show diffuse moderate hyperintensity of
midbasal septum (arrows) and two well-evident hyperenhancement foci
in midbasal aspect of anterior and posterior LV walls
(arrowheads).
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Fig. 3F —44-year-old man with asymmetric hypertrophic cardiomyopathy
and chest pain on minor exertion. See also Figure S3G, cine loop, in
supplemental data at
www.ajronline.org.
Late-enhancement short-axis images show diffuse moderate hyperintensity of
midbasal septum (arrows) and two well-evident hyperenhancement foci
in midbasal aspect of anterior and posterior LV walls
(arrowheads).
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Restrictive Cardiomyopathy
Restrictive CMP is characterized by reduced ventricular filling and
diastolic volume, leading to atrial dilatation and venous stasis, usually with
preserved systolic function
[1]. Restrictive CMP may be
idiopathic, secondary to infiltrative and storage diseases (such as
amyloidosis and sarcoidosis), or associated with myocardial disorders such as
hypereosinophilic syndrome
[18].
Morphologic images in restrictive CMP may show atrial enlargement. The RV
may also enlarge if pulmonary hypertension coexists. Cine images allow
assessment of the altered diastolic ventricular filling. Restrictive CMP is
characterized by a restrictive diastolic filling pattern, with a narrow blood
inflow jet in early diastole and an early peak–atrial peak ratio > 2
[7]. Systolic function of the
LV is either preserved or reduced.
Cardiac MRI is a fundamental diagnostic tool because it helps in the
differentiation between restrictive CMP and constrictive pericarditis, which
have different therapeutic approaches. Although reduced ventricular filling
and diastolic volumes may be features of both diseases, pericardial thickening
(> 4 mm) is typical of constrictive pericarditis. Pericardial thickening
can be assessed with morphologic T2-weighted black blood images;
unfortunately, the pericardium may be only minimally thickened or even normal
in patients with constrictive pericarditis. New cardiac MRI techniques have
been implemented for the evaluation of dubious cases, such as cine MRI
assessment of diastolic ventricular septal movements and real-time cine MRI
evaluation of septal motion during respiration
[19,
20]. These techniques show
that in restrictive CMP, septal convexity is maintained in all respiratory
phases, whereas in constrictive pericarditis, septal flattening can be
observed in early inspiration.
To our knowledge, the issue of the late-enhancement patterns in idiopathic
restrictive CMP has not been specifically addressed in the literature,
although the late-enhancement patterns in secondary restrictive CMP have been
and will be described further in this article in the section "Specific
Cardiomyopathy" (Figs.
4A,
4B,
4C, and
4D).
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Fig. 4A —59-year-old woman with restrictive cardiomyopathy secondary
to hypereosinophilic Löffler's syndrome. Morphologic T2-weighted black
blood two-chamber long-axis image shows apical and posterior left ventricle
(LV) wall thickening (black arrows). Note signal hyperintensity at
apex, occupied by fibrous elastic thrombus (arrowhead). Left atrium
is slightly enlarged (white arrow).
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Fig. 4B —59-year-old woman with restrictive cardiomyopathy secondary
to hypereosinophilic Löffler's syndrome. Static cine two-chamber
long-axis images in end-diastolic (B) and end-systolic (C)
phases show moderate apical hypokinesia (arrows) with preserved LV
systolic function (ejection fraction, 57%). Analysis of phase-contrast images
shows altered LV diastolic filling pattern, with early peak–atrial peak
ratio < 1 and decreased deceleration time.
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Fig. 4C —59-year-old woman with restrictive cardiomyopathy secondary
to hypereosinophilic Löffler's syndrome. Static cine two-chamber
long-axis images in end-diastolic (B) and end-systolic (C)
phases show moderate apical hypokinesia (arrows) with preserved LV
systolic function (ejection fraction, 57%). Analysis of phase-contrast images
shows altered LV diastolic filling pattern, with early peak–atrial peak
ratio < 1 and decreased deceleration time.
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Fig. 4D —59-year-old woman with restrictive cardiomyopathy secondary
to hypereosinophilic Löffler's syndrome. Late-enhancement two-chamber
long-axis image shows subendocardial enhancement at apex, with hypointense
core corresponding to thrombus (arrowhead). Subtle late-enhancement
areas are also seen in posterior wall (arrows).
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Arrhythmogenic Right Ventricular Cardiomyopathy
Arrhythmogenic RV CMP is characterized by progressive fibrous or fibrofatty
replacement of the myocytes of the RV walls, which can extend to the entire RV
and also to the LV.
The pathogenesis and cause of the disease are still unclear, but many
studies have been implemented and four main hypotheses are now considered:
myocyte apoptosis followed by fibrofatty replacement, dysontogenesis and
subsequent abnormal RV development, degenerative RV disorders with metabolic
causes, and RV myocyte inflammation with fibrofatty replacement seen as a
healing process after myocarditis
[21,
22]. The familial form of the
disease is fairly common, with either autosomal dominant or recessive
inheritance [21]. The clinical
manifestations of arrhythmogenic RV CMP may vary but usually include
ventricular tachycardia with left bundle-branch block, which can lead to
sudden death [21,
22]. Therefore, the diagnosis
of arrhythmogenic RV CMP is fundamental, and cardiac MRI is now considered an
important tool for this purpose
[21].
Black blood images with and without fat suppression may show fibrofatty
replacement of the RV free walls, even if this finding is rarely the only
abnormality in arrhythmogenic RV CMP. Black blood images have recently been
considered less sensitive for the diagnosis of this disease than the detection
of the RV systolic and diastolic dysfunction. Moreover, the estimation of RV
dysfunction should be both qualitative and quantitative
[23]. Cine images usually show
augmented RV volumes (end-diastolic volume > 150 mL, end-systolic volume
> 70 mL). These data may be more accurate if indexed to the body surface
area [6]. Systolic bulging or
even gross dyskinesia and aneurysms of the RV free wall and outflow tract may
be present, leading to an EF < 45% (Figs.
5A,
5B,
5C, and
5D and Figs. S5E and S5F in
supplemental data at
www.ajronline.org).
In our experience, late-enhancement imaging is not always significant because
RV walls in patients with arrhythmogenic RV CMP can be very thin; however,
there is not agreement on this topic in the literature
[24].
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Fig. 5A —51-year-old woman who was admitted to coronary unit for
repeated episodes of syncope of unknown origin. Final diagnosis was
arrhythmogenic right ventricular cardiomyopathy. See also Figure S5E and S5F,
cine loops, in supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood short-axis image with fat suppression
shows marked right ventricle enlargement (end-diastolic volume, 325 mL) and
diffuse wall thinning, with hypointense areas of fatty infiltration of
myocardium (arrows).
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Fig. 5B —51-year-old woman who was admitted to coronary unit for
repeated episodes of syncope of unknown origin. Final diagnosis was
arrhythmogenic right ventricular cardiomyopathy. See also Figure S5E and S5F,
cine loops, in supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood four-chamber long-axis image (B)
shows hyperintense areas in apical posterolateral aspect of left ventricle
(straight arrow) and in right ventricle free wall (curved
arrows) that correspond to hypointense foci in fat-suppression image
(C) (straight and curved arrows), due to fatty
infiltration of myocardial wall. In C, thinning of right ventricle free
wall is also evident (arrowhead).
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Fig. 5C —51-year-old woman who was admitted to coronary unit for
repeated episodes of syncope of unknown origin. Final diagnosis was
arrhythmogenic right ventricular cardiomyopathy. See also Figure S5E and S5F,
cine loops, in supplemental data at
www.ajronline.org.
Morphologic T2-weighted black blood four-chamber long-axis image (B)
shows hyperintense areas in apical posterolateral aspect of left ventricle
(straight arrow) and in right ventricle free wall (curved
arrows) that correspond to hypointense foci in fat-suppression image
(C) (straight and curved arrows), due to fatty
infiltration of myocardial wall. In C, thinning of right ventricle free
wall is also evident (arrowhead).
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Fig. 5D —51-year-old woman who was admitted to coronary unit for
repeated episodes of syncope of unknown origin. Final diagnosis was
arrhythmogenic right ventricular cardiomyopathy. See also Figure S5E and S5F,
cine loops, in supplemental data at
www.ajronline.org.
Static cine end-systolic image in four-chamber long-axis plane shows diffuse
and gross bulging in right ventricle outflow tract and free wall
(arrows). Interventricular septal bowing due to abnormal right
ventricle enlargement is also evident (arrowhead).
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Specific Cardiomyopathy
Specific CMP is a large group of diseases associated with cardiac or
systemic disorders [1]. Our
attention focuses on amyloidosis, sarcoidosis, and Anderson-Fabry disease.
Amyloidosis is characterized by deposition of amyloid—an insoluble
material that results from protein misfolding—in tissue. Cardiac
involvement is common and may be the cause of death.
The main feature of cardiac amyloidosis is myocardial thickening, similar
to that in hypertrophic CMP but associated with depressed systolic ventricular
function and reduced wall compliance, which in later stages can evolve to
overt restrictive CMP. Moreover, atria may be dilated and transvalvular flow
patterns altered. The late-enhancement pattern seems to be specific for this
disorder and is characterized by a diffuse, heterogeneous subendocardial
distribution that may resemble an incorrect myocardial signal suppression due
to an inappropriate TI choice
[25] (Figs.
6A,
6B, and
6C).
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Fig. 6A —76-year-old woman with histologic diagnosis of amyloidosis of
periumbilical adipose tissue. Morphologic T2-weighted black blood four-chamber
long-axis image shows symmetric hypertrophy of both left and right ventricles
(arrows). Bilateral pleural effusion is also present
(arrowheads).
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Fig. 6B —76-year-old woman with histologic diagnosis of amyloidosis of
periumbilical adipose tissue. Late-enhancement two-chamber long-axis
(B) and short-axis (C) images show intense subendocardial
enhancement involving all left ventricle walls (arrows).
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Fig. 6C —76-year-old woman with histologic diagnosis of amyloidosis of
periumbilical adipose tissue. Late-enhancement two-chamber long-axis
(B) and short-axis (C) images show intense subendocardial
enhancement involving all left ventricle walls (arrows).
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Sarcoidosis is a systemic granulomatous disease that can involve the heart
with noncaseating granulomatous infiltration. Cardiac involvement is one of
the main factors determining the prognosis, even if cardiac symptoms are
experienced by only approximately 5% of subjects with the disorder
[26].
At black blood imaging, myocardial thickening may be present and may
resemble hypertrophic CMP. Diffuse and focal sarcoid infiltrates may lead to
hyperintensity on T2-weighted fat-saturated or STIR images because of
myocardial edema. On cine images, LV altered filling patterns may be seen and
may be consistent with secondary restrictive CMP. Moreover, contraction
abnormalities are a frequent finding, with a segmental distribution that often
overlaps the late-enhancement areas. These findings suggest that in cardiac
sarcoidosis, late-enhancement areas may also represent fibrotic replacement of
the myocardium. The late-enhancement areas seem to have specific locations
(basal interventricular septum, lateral LV wall) and distribution patterns
(patchy or with striae that do not involve the subendocardium; diffuse and
transmural if the disease is advanced), which can help in the differentiation
between cardiac sarcoidosis and postischemic myocardial injury
[27] (Figs.
7A,
7B, and
7C).
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Fig. 7A —56-year-old woman with pulmonary sarcoidosis. Morphologic
T2-weighted black blood short-axis image with fat suppression shows
hyperintense spot located in posterior aspect of mid left ventricle wall
(arrow) that is caused by myocardial edema and corresponds to subtle
late-enhancement focus described in C (arrow).
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Fig. 7B —56-year-old woman with pulmonary sarcoidosis. Morphologic
T2-weighted black blood STIR four-chamber long-axis image shows small
hyperintense foci in lateral left ventricle wall (arrows) caused by
myocardial edema.
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Fig. 7C —56-year-old woman with pulmonary sarcoidosis.
Late-enhancement short-axis image shows hyperintense focus in posterior aspect
of mid left ventricle wall (arrow) that corresponds to hyperintense
spot described in A.
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Anderson-Fabry disease is an X-linked disorder characterized by
-galactosidase A enzyme deficiency, which leads to an abnormal
accumulation of sphingolipid catabolites in various organs, including the
heart. Cardiac involvement is a severe feature of Anderson-Fabry disease
because it can cause arrhythmia, valvulopathy, myocardial infiltration with
development of secondary restrictive CMP, and myocardial hypertrophy
[28]. This aspect of
Anderson-Fabry disease is well studied with morphologic cardiac MRI images,
which usually depict symmetric myocardial wall thickening. Moreover, cine
imaging may show an increased LV EF and reduced volume.
The most interesting part of the cardiac MRI examination in patients with
Anderson-Fabry disease is the late-enhancement study. In fact, recent articles
by Moon et al. [28,
29] suggest that the
late-enhancement phenomenon may be the expression of both intramyocyte
accumulation of sphingolipids and interstitial expansion, probably caused by
fibrosis and collagen deposition, with the latter hypothesis considered the
most reliable. These possible histopathologic correlations need further study
but may point out the clinical significance of late-enhancement findings in
the patients with Anderson-Fabry disease. Moreover, findings by Moon et al.
[28] suggest that
late-enhancement areas seem to have a specific location (midbasal lateral LV
wall) and distribution pattern (subepicardial and mesocardial) in patients
with Anderson-Fabry disease who have cardiac symmetric hypertrophy. This
finding may be helpful in the differential diagnosis between hypertrophic CMP
and symmetric hypertrophy due to Anderson-Fabry disease (Figs.
8A,
8B, and
8C).
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Fig. 8A —47-year-old man with Anderson-Fabry disease and arrhythmias.
Morphologic T2-weighted black blood four-chamber long-axis image shows
symmetric hypertrophy of left ventricle (LV) (arrows). Right
ventricle walls also are moderately hypertrophied (arrowhead).
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Fig. 8B —47-year-old man with Anderson-Fabry disease and arrhythmias.
Late-enhancement four-chamber long-axis image shows thick mesocardial
hyperenhancement stria located in midbasal lateral LV wall
(arrows).
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Conclusion
CMPs are a group of fairly rare diseases, but they are a frequent reason
for cardiac MRI evaluation. CMPs are different in cause, clinical
presentation, histologic features, and expected outcome; for these reasons,
they must be diagnosed and studied with imaging techniques that warrant
precise and reliable assessment of the many aspects of the disease, such as
morphology, function, and tissue characterization of the damaged myocardium.
Cardiac MRI fulfills all these requirements and may be considered the most
important imaging technique for CMP.
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Abstract
Introduction
