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Cardiac MR Imaging of Nonischemic Cardiomyopathies: Imaging Protocols and Spectra of Appearances

February 2012
Radiology 262(2):403-22

February 2012
262(2):403-22

DOI:10.1148/radiol.11100284

Source
PubMed

Authors:

Suhny Abbara

University of Texas Southwestern Medical Center

Kibar Yared

University of Toronto

Show all 9 authorsHide

Recent technologic advances in cardiac magnetic resonance (MR) imaging have resulted in images with high spatial and temporal resolution and excellent myocardial tissue characterization. Cardiac MR is a valuable imaging technique for detection and assessment of the morphology and functional characteristics of the nonischemic cardiomyopathy. It has gained acceptance as a standalone imaging modality that can provide further information beyond the capabilities of traditional modalities such as echocardiography and angiography. Black-blood fast spin-echo MR images allow morphologic assessment of the heart with high spatial resolution, while T2-weighted MR images can depict acute myocardial edema. Contrast material-enhanced images can depict and be used to quantify myocardial edema, infiltration, and fibrosis. This review presents recommended cardiac MR protocols for and the spectrum of imaging appearances of the nonischemic cardiomyopathies.

(a, b) HCM in a 52-year-old woman. (a) Short-axis SSFP MR image shows mild asymmetric hypertrophy (16 mm thick) of interventricular septum (solid arrows), as compared with 9-mm-thick lateral free wall (open arrow). Note RV dilatation. (b) Short-axis late-gadolinium-enhanced MR image shows enhancement in hypertrophied segment (arrow), consistent with scar. (c, d) HCM in her 16-year-old son. (c) Short-axis SSFP image shows asymmetric hypertrophy of inferoseptal segment (arrow). (d) Short-axis late-enhancement MR image shows enhancement (arrow) in hypertrophied segment, consistent with scar. (e, f) Apical-variant HCM in a 45-year-old man. (e) Vertical-long-axis SSFP MR image shows asymmetric apical hypertrophy, a variant of HCM that occurs in 5%–10% of cases. (f) Vertical-longaxis late-enhancement image shows scar in hypertrophied segments (arrow).

…

ARVD in a 38-year-old woman. (a) Horizontal-long-axis T1-weighted fast spinecho MR image without fat saturation shows extensive fat infi ltration in RV free wall (arrows). Note similar signal intensity characteristics to epicardial fat. (b) Horizontal-long-axis T1-weighted spinecho MR image with fat saturation shows signal drop-out in RV wall, confi rming intramyocardial fat (arrows). (c) Horizontal-long-axis SSFP MR image from a more caudal level shows focal aneurysms (arrow) in RV free wall.

…

Primary Cardiomyopathies Predominantly Involving the Heart

…

Secondary Cardiomyopathies Characterized by Multiorgan Involvement

…

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REVIEWS AND COMMENTARY

n REVIEW

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 403

1 From the Department of Radiology, Beth Israel Deaconess

Medical Center, 330 Brookline Ave, Boston, MA 02215 (D.H.O.);

Cardiac MR-PET-CT Program, Center for Integration of

Medicine and Innovative Technology , Boston, Mass (S.A.,

V.C., K.Y.); Department of Radiology, Massachusetts General

Hospital and Harvard Medical School, Boston, Mass (S.A., K.Y.);

Department of Radiology, Baptist (Miami) Cardiac and Vascular

Institute, Miami, Fla (R.C.C.); and Cardiac CT and MR imaging

Program, St Vincent’s University Hospital and University

College, Dublin, Ireland (R.P.K., R.M., D.K., J.D.D.). Received

March 6, 2010; revision requested April 12; revision received

June 16, accepted July 6; ﬁ nal version accepted July 14.

Final review by J.D.D. October 18, 2011. Address

correspondence to J.D.D. (e-mail: j.dodd@st-vincents.ie ).

q RSNA, 2012

David H. O’Donnell , MD

Suhny Abbara , MD

Vithaya Chaithiraphan , MD

Kibar Yared , MD

Ronan P. Killeen , MD

Ramon Martos , MD

David Keane , PhD

Ricardo C. Cury , MD

Jonathan D. Dodd , MD

Cardiac MR Imaging of

Nonischemic Cardiomyopathies:

Imaging Protocols and Spectra of

Appearances

Recent technologic advances in cardiac magnetic reso-

nance (MR) imaging have resulted in images with high

spatial and temporal resolution and excellent myocardial

tissue characterization. Cardiac MR is a valuable imag-

ing technique for detection and assessment of the mor-

phology and functional characteristics of the nonischemic

cardiomyopathy. It has gained acceptance as a standalone

imaging modality that can provide further information

beyond the capabilities of traditional modalities such as

echocardiography and angiography. Black-blood fast spin-

echo MR images allow morphologic assessment of the

heart with high spatial resolution, while T2-weighted MR

images can depict acute myocardial edema. Contrast

material–enhanced images can depict and be used to

quantify myocardial edema, inﬁ ltration, and ﬁ brosis. This

review presents recommended cardiac MR protocols for

and the spectrum of imaging appearances of the nonis-

chemic cardiomyopathies.

q RSNA, 2012

Supplemental material: http://radiology.rsna.org/lookup

/suppl/doi:10.1148/radiol.11100284/-/DC1

Learning Objectives:

n Review the standard cardiac MR imaging protocol required

to assess for the presence of cardiomyopathy

n Recognize speciﬁ c additional MR sequences that may

increase the diagnostic accuracy of cardiac MR imaging in

certain speciﬁ c nonischemic cardiomyopathies

n Identify the spectrum of MR imaging appearances of

nonischemic cardiomyopathies

Accreditation and Designation Statement

The RSNA is accredited by the Accreditation Council or

Continuing Medical Education (ACCME) to provide continuing

medical education for physicians. The RSNA designates this

journal-based activity for a maximum of 1.0. AMA PRA

Category 1 Credit ™. Physicians should claim only the credit

commensurate with the extent of their participation in the

activity.

Disclosure Statement

The ACCME requires that the RSNA, as an accredited

provider of CME, obtain signed disclosure statements from

the authors, editors, and reviewers for this activity. For this

journal-based CME activity, author disclosures are listed at

the end of this article.

Online CME

See www.rsna.org/education/ry_cme.html

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

404 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Recent technical advances have al-

lowed cardiac magnetic resonance

(MR) imaging to enter mainstream

cardiology imaging practice for cardio-

myopathy assessment ( 1 ). The devel-

opment of increasing magnetic ﬁ eld

strengths and surface coil channels, rapid

k-space sampling, postprocessing tech-

niques, and sophisticated sequences for

myocardial characterization have made

cardiac MR a powerful tool in the work-

up of many complex cardiomyopathies

( 2 ). Consensus statements from several

international cardiac associations include

cardiac MR as a primary imaging tech-

nique ( 1 , 3 , 4 ). In this review, we will out-

line the cardiac MR protocols that allow

optimal characterization of nonischemic

cardiomyopathy and illustrate the spec-

trum of their appearances on cardiac

MR imaging studies.

The prevalence of cardiomyopathy

in the United States is approximately

one in 5439 people, or 0.02% of the

population ( Table 1 ). Prevalence varies

between the most common type, dilated

cardiomyopathy, to the least common,

restrictive cardiomyopathy. It is esti-

mated that cardiomyopathy causes ap-

proximately 25 000 deaths each year in

Published online

10.1148/radiol.11100284 Content codes:

Radiology 2012; 262:403– 422

Abbreviations:

ARVD = arrhythmogenic RV dysplasia

HCM = hypertrophic cardiomyopathy

LV = left ventricle

RV = right ventricle

SSFP = steady-state free precession

Potential conﬂ icts of interest are list at the end

of this article.

Essentials

Cardiac MR is a valuable non- n

invasive imaging technique for

detection and assessment of the

morphologic, functional, and

myocardial contrast-enhancement

characteristics of nonischemic

cardiomyopathy and has become

the noninvasive imaging test of

choice for measuring chamber

size and function.

Cardiac MR complements and n

furthers the information acquired

with traditional modalities such

as echocardiography in nonisch-

emic cardiomyopathy assessment

and in many cases allows accu-

rate determination of their prog-

nostic implications.

Contrast-enhanced MR images n

may depict and help quantify

myocardial inﬂ ammation, inﬁ ltra-

tion, and ﬁ brosis.

the United States and follows coronary

arterial heart disease as the common-

est cause of sudden death ( 5 ). Trans-

thoracic echocardiography remains the

mainstay imaging modality for cardio-

myopathy. It allows accurate assessment

of chamber dynamics, valvular motion,

and real-time Doppler interrogation of

intracardiac blood ﬂ ow and its practical

aspects, such as widespread availability

and portability, make it a valuable im-

aging method for cardiomyopathy eval-

uation ( 6 ). Nevertheless, the accuracy

of transthoracic echocardiography can

be reduced by factors that cause subop-

timal acoustic windows, such as chest

wall or rib deformities , obesity, and ob-

structive lung disease. Transesophageal

echocardiography resolves this by imag-

ing through the esophagus, with often-

improved visualization of the posterior

aspect of the heart, which is sometimes

not possible via the transthoracic win-

dow. Disadvantages are associated with

its invasive nature: Intubation of the

esophagus is required, and there is an

attendant small but documented risk of

esophageal perforation, bleeding, and

aspiration ( 7 ). The patient may also re-

quire care by an anesthesiologist.

Cardiac MR is a noninvasive test

with excellent spatial and myocardial

tissue resolution ( 2 ). A combination of

sequences allows the detection, locali-

zation, and quantiﬁ cation of many path-

ologic myocardial processes. It has be-

come the reference standard test for

accurate quantiﬁ cation of chamber size

and function ( 8 ). In this article, we will

describe the cardiac MR protocols that

allow optimum imaging and provide the

spectrum of appearances of nonisch-

emic cardiomyopathies.

Classiﬁ cation of Cardiomyopathy

An increased understanding of cardio-

myopathy prompted a revision of the

1995 World Health Organization/Inter-

national Society and Federation of Car-

diology Task Force on the Deﬁ nition and

Classiﬁ cation of Cardiomyopathy with

the inclusion of new subgroups of myo-

cardial diseases ( 9 ). This contempo-

rary consensus report revised the def-

inition of cardiomyopathy to include

“mechanical or electrical dysfunction that

usually exhibit inappropriate ventricu-

lar hypertrophy and dilatation due to a

variety of causes that frequently are

genetic.” Under the classiﬁ cation system,

the division of cardiomyopathy is split

into primary and secondary causes

( Ta bl es 2 , 3 ). Primary cardiomyopathies

are those dis ease processes that are

uniquely intrinsic to the myocardium.

They may be genetic, acquired, or mixed

and represent a minority of cardiomy-

opathies. Secondary cardiomyopathies

have multiorgan involvement and are

responsible for the majority of cases.

There are other classiﬁ cation systems

such as the classiﬁ cation by Elliott et al

( 11 ) published by the European Soci-

ety of Cardiology Working Group on

Myocardial and Pericardial Diseases,

which places an emphasis on pheno-

typic classiﬁ cation.

Basic Cardiac MR Protocols for

Cardiomyopathy Assessment

The cardiac MR imaging protocol used

in cases of cardiomyopathy should be

tailored speciﬁ cally to the suspected

type of cardiomyopathy. In this regard,

it is important that the radiologist be

present to review the images, so that

subsequent additional sequences can

be determined. There is a tremendous

degree of diversity in cardiac MR se-

quences, but all follow a basic generic

protocol ( Table 4 ) ( 11 ). Speciﬁ c addi-

tional sequences may be added, de-

pending on the cardiomyopathy. Table 4

outlines the general basic set of car-

diac MR imaging sequences for car-

diomyopathy evaluation (see also Fig E1

[online]).

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 405

Scout images (axial, coronal, sagit-

tal) are useful, particularly sagittal scout

images, which represent an underuti-

lized and valuable opportunity to ensure

that the heart is positioned at the cen-

ter of the surface coils, which improves

signal to noise. A stack of axial sections

of the thorax (most commonly, half-

Fourier rapid acquisition with relaxa-

tion enhancement or SSFP sequences)

is acquired next, to depict major non-

cardiac pathologic processes and to

provide planning information for the sub-

sequent sequences (Fig E1a [online]).

Typically, a set of cine SSFP sections

are acquired next in the vertical long axis

(so named because this imaging plane

depicts the atrium and ventricle; Fig E1b

[online], Movie 1a [online]) and hori-

zontal long axis (so named because it

depicts all four cardiac chambers; Fig

E1c [online], Movie 1b [online]) planes.

These and all subsequent sequences

are acquired by using cardiac gating.

This is an essential component to over-

come image blurring secondary to car-

diac contraction. The optimal method

is to use electrocardiographic gating, in

which image acquisition is triggered

from the QRS complex of a three-lead

electrocardiogram. For cine SSFP sec-

tions, portions of k-space data that

compose the image are acquired at the

same time point of the QRS complex over

several heartbeats, which eliminates im-

age blurring. These sequences usually

require a breath hold of 8–12 seconds

and, typically, acquisition of two paral-

lel sections (because one may be of sub-

optimal image quality or miss the ideal

image plane). These are followed by a

stack of short-axis sections from the

annulus to the apex (Fig E1d [online],

Movie 1c [online]). These are used to al-

low quantiﬁ cation of chamber volumes

and myocardial function. It is prudent

to plan to acquire the ﬁ rst section of the

stack on the atrial side of the annulus,

because the annulus of the heart con-

tracts toward the apex during systole.

Once acquired, the stack of short-axis

sections can be interrogated with spe-

ciﬁ c software programs to enable accu-

rate measurements of end diastolic and

systolic volumes and record functional

cardiac parameters, such as left and

right ventricular end-systolic and dia-

stolic volumes, cardiac mass, stroke vol-

ume and ejection fraction. A qualitative

analysis of global and regional ventricu-

lar function may be provided using a

ﬁ ve-point scale (score of 1 for normal;

2 for mild hypokinesia, 3 for severe

hypokinesia, 4 for akinesia, 5 for

dyskinesia) ( 12 ). Although chamber vol-

umes are more meaningful, we also con-

sider it useful to supply an end-diastolic

diameter of the LV in our reports, which

we measure at the midventricular level.

This should mea sure a mean of 50.2 mm

(upper bound of 95% conﬁ dence inter-

val, 58.5 mm) for men and 45.6 mm

(upper bound, 51.1 mm) for women

( 13 ).

For all cine acquisitions, parallel im-

aging techniques can be used to shorten

the breath hold. In brief, parallel imag-

ing incorporates the signal and its loca-

tion from multiple independent cardiac

receiver coils to encode multiple MR

echoes simultaneously, which speeds up

image acquisition ( 14 ).

Several additional sequences can

be performed at this point, depending

on the cardiomyopathy in question.

These are described below in more

detail for each cardiomyopathy. After

these sequences have been performed,

late-gadolinium-enhanced images are

acquired. The optimum time for late-

enhancement image acquisition is usu-

ally 10–30 minutes after contrast agent

injection ( 15 ). For late-enhancement im-

ages, optimal contrast differentiation

between viable and nonviable myocar-

dium is individually evaluated for each

patient by determining the exact time

the signal of the normal myocardium is

nulled or black (Fig E1e [online]). The

inversion time will vary considerably

from patient to patient and must be de-

termined on an individual basis. If the

myocardium has a conﬂ uent gray ap-

pearance, then the inversion time is

usually too early (Fig E1f); if it has a

mixed appearance, then it is usually too

late (Fig E1g). Normal inversion times

vary but generally are between 200 and

350 msec, depending on the manufac-

turer of the MR unit, the cardiac out-

put, the time of image acquisition after

contrast agent injection, and the dose

of contrast agent administered ( 15 ). In

contrast-enhanced imaging, inversion

time is adjusted as the examination pro-

gresses, to allow for contrast agent wash-

out from the myocardium (nulling time

will change as this is happening). In-

creasing the time by 10–20 mse c every

one to three sections is usually sufﬁ cient.

The correct inversion time is very im-

portant for accurate image interpreta-

tion and to prevent polarity artifacts. A

useful alternative is acquisition of phase-

sensitive images by using an inversion-

recovery spoiled-gradient-echo or SSFP

sequence (Fig E1h) ( 16 ). This minimizes

polarity artifacts over a wider range of

inversion times than the traditional mag-

nitude sequence, thus resulting in more

consistent image quality. However, in our

experience it is not as sensitive for de-

tection of small areas of enhancement.

The myocardium is evaluated for the

presence, location, and extent of en-

hancement. A useful method of refer-

ence for localization uses the American

Heart Association 17-segment model,

in which the ventricle is divided into

three levels: six basal segments, six

middle segments, and four apical seg-

ments, with the apex being the last seg-

ment ( 17 ). A useful method of reference

for extent of myocardial enhancement

uses a ﬁ ve-point scale for each segment:

score of 0 for no late enhancement, 1 =

1%–25% of the affected segment en-

hanced, 2 for 26%–50% of the affected

segment enhanced, 3 for 51%–75% of

the affected segment enhanced, and 4

for 76%–100% of the affected segment

enhanced ( 18 ). Global late-enhancement

extent as a percentage of LV myocar-

dium may be calculated by summing the

segments with late enhancement (each

weighted by the midpoint of enhance-

ment for the segmental score). The

contrast-to-noise ratio is also often pro-

vided for the late-enhancement sequence.

This can be calculated (parallel imaging

should be not be used for this calcu-

lation) as (SI

LE 2 SI

RM ]/SD

N , where

LE is mean signal intensity of late-

enhancing segments, SI

RM is mean sig-

nal intensity of remote myocardium,

and SD

N is the standard deviation of

noise . Finally, it is important to em-

phasize the overall distribution of the

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

406 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Table 2

Primary Cardiomyopathies Predominantly Involving the Heart

Genetic

* Mixed Acquired

HCM Dilated cardiomyopathy Inﬂ ammatory (myocarditis)

Arrhythmogenic right ventricular dysplasia Restrictive cardiomyopathy Stress provoked (takotsubo)

Left ventricular noncompaction Peripartum

Glycogen storage diseases (PRKAG 2, Danon) Tachycardia induced

Conduction defects Infants of diabetic mothers

Mitochondrial myopathies

Ion channel disorders (LQTS, Brugada, SQTS,

CVPT, Asian SUNDS)

* CVPT = catecholaminergic ventricular polymorphic tachycardia, IDDM = insulin-dependent diabetes mellitus, LQTS = long Q-T

syndrome, SQTS = short Q-T syndrome, SUNDS = sudden unexplained death syndrome.

Table 1

Known Prevalence Estimates of Cardiomyopathy in the United States

Disease

* Prevalence Estimate

†

All cardiomyopathies 1 in 5439 ( 8 )

HCM 1 in 500 ( 8 )

Arrthymogenic RV dysplasia 1 in 5000 ( 8 )

LV noncompaction Unknown

Dilated cardiomyopathy 1 in 2500 ( 8 )

Restrictive cardiomyopathy Unknown

Myocarditis Rare, , 1 in 200 000

Peripartum Unknown

Takotsubo 0.7% ( 103 )

Amyloidosis Unknown

Cardiac sarcoidosis Unknown

Siderotic Unknown

Scleroderma Unknown

* HCM= hypertrophic cardiomyopathy, LV = left ventricle, RV = right ventricle.

† Numbers in parentheses are reference numbers .

enhancement and whether it is in a

predominantly subepicardial, midwall,

or subendocardial distribution.

The use of contrast agents in car-

diac MR imaging is an important aspect

of the utility of cardiac MR for charac-

terizing nonischemic cardiomyopathies.

We use a commercially available gado-

linium-based contrast agent (gadopen-

tetate dimeglumine) in our protocol.

The contrast agent is injected as a bolus

(0.2 mmol per kilogram of body weight)

via an arm vein, preferably in the ante-

cubital fossa. It may be administered

by means of hand injection or infusion

pump. This should be followed by a 20–

40-mL saline bolus chaser. Gadolinium-

based contrast agents are not recom-

mended in patients with renal failure

and a glomerular ﬁ ltration rate of less

than 30 mL/kg/min because of the risk

of nephrogenic systemic ﬁ brosis .

Primary Cardiomyopathy

Genetic Cardiomyopathies

HCM.— HCM is classiﬁ ed as a pri-

mary genetic cardiomyopathy that phe-

notypically is characterized by LV and

sometimes RV (approximately 17%)

muscular hypertrophy and impaired di-

astolic function associated with a non-

dilated cavity in the absence of another

cardiac or systemic disease that could

produce the magnitude of hypertrophy

( Fig 1 ) ( 19 , 20 ). It may be associated

with LV outﬂ ow tract obstruction and

increased risk of sudden death. HCM is

genetically transmitted in an autosomal

dominant pattern with variable pene-

trance and expression ( 9 ). It is typically

associated with hypertrophy of the

muscle t o 15 mm or thicker. HCM has

an estimated incidence of one in 500 of

the general population ( 21 ) and has a

bimodal pattern of incidence. Symptoms

range from asymptomatic to progressive

dyspnea, orthopnea, paroxysmal noc-

turnal dyspnea, and sudden death, which

is particularly common in adolescents,

usually secondary to ventricular ﬁ brilla-

tion. The risk of sudden death in children

may be as high as 4%–6% per year.

At histologic examination, areas of

hypertrophy show a pattern of myoﬁ -

brillar disarray with focal areas of ne-

crosis, predominantly in the midmyo-

cardium, with subsequent myocardial

ﬁ brosis ( 22 ). Characteristic imaging

ﬁ ndings include LV wall hypertrophy

resulting in a small LV cavity that may

be obliterated in severe cases, diastolic

dysfunction with reduced LV compli-

ance, and relatively preserved systolic

function (LV ejection fraction is usually

normal at the time of diagnosis). Biopsy

has a low diagnostic yield and is not

generally performed in routine prac-

tice ( 23 ). Concomitant mitral regurgi-

tation due to a morphologically abnor-

mal mitral valve apparatus is commonly

identiﬁ ed on cardiac MR images. Clas-

sic ﬁ ndings in HCM with obstructive

features include asymmetric septal hy-

pertrophy and systolic anterior motion

of the anterior mitral leaﬂ et.

Important MR sequences in HCM.—

Cardiac MR imaging has played an

increasing role in assessing patients with

HCM and is now considered the nonin-

vasive imaging test of choice for depict-

ing the morphology of the ventricle ( Fig 1 ,

Movie 2 [online]) ( 24 ). The morphologic

and functional characteristics are clearly

demonstrated and quantiﬁ ed by using

cine SSFP sequences in the short axis.

Cardiac MR imaging can help accurately

assess the degree of systolic obstruc-

tion by systolic anterior motion on a

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 407

Table 3

Secondary Cardiomyopathies Characterized by Multiorgan Involvement

Type of Cardiomyopathy Diseases

Inﬁ ltrative Amyloidosis, Gaucher disease, Hurler disease, Hunter disease

Storage Hemochromatosis, Fabry disease, glycogen-storage disease (type II, Pompe),

Niemann-Pick disease

Toxicity Drugs, heavy metals, chemical agents

Endomyocardial Endomyocardial ﬁ brosis, hypereosinophilic syndrome (Loefﬂ er endocarditis)

Inﬂ ammatory Sarcoidosis

Endocrine Diabetes mellitus, hyperthyroidism, hypothyroidism, hyperparathyroidism,

pheochromocytoma, acromegaly

Neuromuscular

or neurologic

Friedreich ataxia, Duchenne-Becker muscular dystrophy, Emery-Dreifuss

muscular dystrophy, myotonic dystrophy, neuroﬁ bromatosis,

tuberous sclerosis

Nutritional Beriberi, pellagra, scurvy, selenium, carnitine

Autoimmune Systemic lupus erythematosus, dermatomyositis, rheumatoid arthritis,

scleroderma, polyarteritis nodosa

Electrolyte imbalance Potassium, phosphate, magnesium deﬁ ciencies; anorexia nervosa;

laxative abuse

Cancer therapy Anthracylines (doxorubicin), cyclophosphamide, radiation

three-chamber view (Movie 3[online]),

which depicts the left atrium and LV,

the mitral and aortic valves, and the

proximal ascending aorta ( Table 4 ) by

using planimetry and phase-velocity

encoding sequences of the LV outﬂ ow

tract ( 25 ). Myocardial tagging can be

used to depict detailed myocardial ab-

normalities in HCM, such as a reduc-

tion in posterior rotation, reduced

radial displacement and reduced three-

dimensional myocardial shortening, al-

though this sequence application has

not entered into mainstream imaging

practice as yet.

HCM should be distinguished from

LV hypertrophy caused by increased af-

terload, such as in cases of hypertension,

aortic stenosis, or athlete’s heart in

which the LV wall thickness can measure

up to 15 mm. Petersen et al ( 26 ) found

that that the most useful parameter to

enable distinction between HCM and

physiologic causes such as athlete’s

heart was the ratio of end-diastolic wall

thickness to end-diastolic volume. Use

of a cutoff value of 0.15 mm/mL/m

yields positive and negative predictive

values of 95% and 94%, respectively.

When evaluating for HCM, it is im-

portant to be aware of several morpho-

logic variants from the classic form, in

which the mid or apical ventricular

levels may be predominantly affected

( Fig 1 , Movie 4 [online]). A minority

of patients (5%) demonstrate circum-

ferential symmetric hypertrophy.

Late gadolinium enhancement on

cardiac MR images in patients with

HCM is signiﬁ cantly associated with

traditional risk factors for sudden death

in HCM ( Fig 1 ) ( 27 ). More recently,

even in asymptomatic or mildly symp-

tomatic patients with HCM, the pres-

ence of late enhancement has been

associated with the development of ar-

rhythmias ( 28 ). Large HCM cohort stud-

ies ( 29 , 30 ) have now shown a strong

independent association between late

enhancement and surrogate markers

of arrhythmia, sudden cardiac death,

and implantable cardioverter deﬁ brilla-

tor discharge. Finally, with its absence

of radiation exposure, serial cardiac

MR imaging seems to be an ideal tool

for screening relatives of patients with

HCM (Fig 1) ( 31 ).

Arrhythmogenic right ventricular

dysplasia.— Arrhythmogenic right ven-

tricular dysplasia (ARVD) is a rare genetic

disorder characterized by progressive

loss of myocytes with ﬁ brofatty replace-

ment of RV and, less commonly, LV

myocardium. Patients may have ventric-

ular arrhythmias and left bundle branch

block at the time of presentation, syn-

cope, or sudden cardiac death. ARVD

may be responsible for up to 5% of

sudden deaths in young athletes ( 32 ) but

has a higher prevalence in other coun-

tries such as Italy (25% of sudden

deaths in young adults) ( 33 ). Because

of the subtlety of the phenotype, con-

sensus criteria were developed on the

basis of structural, functional, and elec-

trocardiographic manifestations. In 1994,

the task force of the Working Group on

Myocardial and Pericardial Disease of

the European Society of Cardiology and

the task force of the Scientiﬁ c Council

on Cardiomyopathy of the World Heart

Federation proposed diagnostic criteria

based on the presence of major and mi-

nor criteria that involve structural, his-

tologic, electrocardiographic, genetic,

and arrhyth mic factors ( 34 ). To fulﬁ ll the

criteria for ARVD, the patient’s condi-

tion had to meet two major criteria, one

major criterion and two minor criteria,

or four minor criteria. These criteria

have since been modiﬁ ed in 2010 by the

task force ( 35 ). In the modiﬁ ed criteria,

tis sue characterization depicted on car-

diac MR images, such as fatty inﬁ ltration,

have been removed, and more empha-

sis has been placed on wall motion, vol-

ume, and ejection fraction abnormalities

( Table 5 ).

Pat hologic de scripti ons includ e ﬁ bro-

fatty replacement of myocardial tis-

sue, focal RV wall thinning and/or aneu-

rysm, RV outﬂ ow tract enlargement, and

RV dilatation ( 36 ). RV biopsy has a low

diagnostic yield because of the patchy

distribution of ﬁ brofatty inﬁ ltration and

the predominantly epicardial dis tribution

( 23 ). Cardiac MR assessment of ARVD

is now based primarily on functional

and volume abnormalities of the RV

( 35

). Many centers still provide tissue-

characterization imaging of the RV. (a)

Morphologic abnormalities include in-

tramyocardial fat deposits ( Fig 2a, 2b ),

focal wall thinning ( , 2 mm), wall hy-

pertrophy ( . 6 mm), moderator band

hypertrophy, and trabeculation thicken-

ing and disarray. (b) Functional abnor-

malities include focal or global RV wall

hypokinesis, focal or global RV dilata-

tion, and focal aneurysms in severe

cases ( Fig 2c , Movie 5 [online]). The

site of involvement of these abnormal-

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408 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Table 4

Basic Cardiac MR Protocol for Imaging of Cardiomyopathy

Sequence Imaging Plane

Repetition Time (msec)/

Echo Time (msec) Flip Angle (degrees) No. of Sections

Section

Thickness (mm) Intersection Gap (%) Other Parameters

Localizers Coronal, axial, sagittal

Half-Fourier RARE Axial sections of thorax 1000/27 160 Typically 16–20 8–10 20

SSFP

Vertical long axis Perpendicular to mitral annulus

and apex of ventricle

3.96/1.12 60 2 6–8 20 Breath hold

Horizontal long axis Parallel to mitral annulus and

middle of interventricular

septum

Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Similar to vertical-long-axis

parameters

Short axis Perpendicular to interventricular

septum, through both ventricles

(and sometimes atria)

Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Typically 6–12 Similar to vertical-

long-axis

parameters

Similar to vertical-

long-axis

parameters

Similar to vertical-long-axis

parameters

T2 weighted Similar to short-axis plane 2 R-R intervals/100 10–12 30–50 Breath hold, black blood,

double inversion

recovery, fat saturation

Late gadolinium enhanced

T1-weighted scout

† 23.49/1/12 50 1 repeated

25–30 times

8 Acquired 10–30 minutes

after contrast agent

injection

Formal late-enhancement

2D double-inversion

gradient echo

7.0/3.37

‡ 25 Typically 6–12 6–8 0–20 Enhancing areas often

conﬁ rmed by acquiring

vertical-long-axis or

4-chamber oblique image

through any abnormal region

* RARE = rapid acquisition with relaxation enhancement , SSFP = steady-state free precession, 2D = two-dimensional.

† Allows determination of optimal inversion time.

‡ Trigger delay set to image in middiastole . .

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Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 409

Figure 1: (a, b) HCM in a 52-year-old woman. (a) Short-axis SSFP MR image shows mild asymmetric hypertrophy (16 mm thick) of interventricular septum (solid

arrows), as compared with 9-mm-thick lateral free wall (open arrow). Note RV dilatation. (b) Short-axis late-gadolinium-enhanced MR image shows enhancement in

hypertrophied segment (arrow), consistent with scar. (c, d) HCM in her 16-year-old son. (c) Short-axis SSFP image shows asymmetric hypertrophy of inferoseptal

segment (arrow). (d) Short-axis late-enhancement MR image shows enhancement (arrow) in hypertrophied segment, consistent with scar. (e, f) Apical-variant HCM in

a 45-year-old man. (e) Vertical-long-axis SSFP MR image shows asymmetric apical hypertrophy, a variant of HCM that occurs in 5%–10% of cases. (f) Vertical-long-

axis late-enhancement image shows scar in hypertrophied segments (arrow).

Figure 1

ities is characteristically found in the

inferior subtricuspid area, the RV apex,

and the RV infundibulum—the so-called

triangle of dysplasia. Depiction of mor-

phologic and functional abnormalities

together improves the speciﬁ city of

ARVD diagnosis ( 37 ). The major treat-

ment implication when ARVD is sus-

pected is the decision to insert an im-

plantable deﬁ brillator.

Important cardiac MR sequences in

ARVD.— In our experience, ARVD im-

aging remains one of the most chal-

lenging applications in cardiac MR

imaging in terms of sequence perfor-

mance, time requirements, and image

interpretation. Along with the basic im-

aging protocol outlined above, cardiac

MR focuses particular attention on the

RV and RV outﬂ ow tract (RVOT). Some

centers image patients in the prone po-

sition to minimize chest wall breath ing

artifacts. Most centers use a thinner

section thickness and intersection gap

for this protocol (5–6-mm contiguous

sections are typical). Spin-echo or fast

spin-echo T1-weighted images (spin-echo

studies can be acquired in systole with

most MR units, making RV myocardial

fat inﬁ ltration ea sier to see, but fast spin -

echo studies are less affected by arti-

fact) can be acquired in an axial oblique

and short-axis stack to evaluate for myo-

cardial fat inﬁ ltration (fast spin-echo se-

quences should use double inversion).

A double-inversion pulse sequence with

a short echo time (30 msec), short echo

train length of 28–30, and thin (5-mm)

sections is optimum ( 36 ). Fat inﬁ ltration

may be conﬁ rmed by repeating the se-

quence with fat-saturation and demon-

strating myocardial signal dropout ( Fig

2a, 2b ). Note that with the new modiﬁ ed

criteria these particular sequences will

not be required, but we mention them

for completeness ( 35 ). Saturation bands

above and below the heart also help im-

prove image quality by reducing ﬂ ow arti-

facts related to slow-inﬂ owing blood. A

small ﬁ eld of view targeted to the RV

helps improve the spatial resolution fur-

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

410 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Table 5

Modiﬁ ed Major and Minor Criteria for Arrhythmogenic RV Dysplasia

Type of Criterion and Modality Criteria

Global or Regional Dysfunction and Structural Abnormalities

Major

2D echocardiography Regional RV akinesia, dyskinesia, or aneurysm AND one of the following (end diastole)

PLAX RVOT ! 32 mm (corrected for body size: [PLAX/BSA] ! 19 mm/m

2 )

PSAX RVOT ! 36 mm (corrected for body size: [PSAX/BSA] ! 21 mm/m

2 )

Fractional area change " 33%

Cardiac MR imaging Regional RV akinesia or dyskinesia or dyssynchronous RV contraction AND one of the following:

Ratio of RV end-diastolic volume to BSA ! 110 (males) or ! 100 mL/m

2 (females)

RV ejection fraction " 40%

RV angiography Regional RV akinesia, dyskinesia, or aneurysm

Minor

2D echocardiography Regional RV akinesia or dyskinesia AND one of the following

PLAX RVOT ! 29 mm to , 32 mm (corrected for body size [PLAX/BSA] ! 16 to , 19 mm/m

2 )

PSAX RVOT ! 32 mm to , 36 mm (corrected for body size [PSAX/BSA] ! 18 to , 21 mm/m

2 )

Fractional area change . 33% to " 40%

Cardiac MR imaging Regional RV akinesia or dyskinesia or dyssynchronous RV contraction AND one of the following:

Ratio of RV end-diastolic volume to BSA ! 100 to , 110 mL/m

2 (male) or ! 90 to , 100 mL/m

2 (female)

RV ejection fraction . 40% to 45%

Myocardial Characterization

Major Residual myocytes , 60% at morphometric analysis ( , 50% if estimated), with ﬁ brous replacement of RV free wall myocardium

in one or more samples, with or without fatty replacement of tissue at endomyocardial biopsy

Minor Residual myocytes 60%–75% at morphometric analysis (50%–65% if estimated), with ﬁ brous replacement of RV free wall

myocardium in one or more samples, with or without fatty replacement of tissue at endomyocardial biopsy

Repolarization Abnormality

Major Inverted T waves in right precordial leads (V

1 R, V

2 R, V

3 R) or beyond in individuals . 14 y old (in absence of complete right

bundle-branch block QRS ! 120 msec)

Minor Inverted T waves in leads V

1 and V

2 in individuals . 14 y old (in absence of complete right bundle-branch block) or in V

4 , V

5 , or V

Inverted T waves in leads V

1 , V

2 , V

3 , and V

4 in individuals . 14 y old in presence of complete right bundle-branch block

Depolarization or Conduction Abnormality

Major Epsilon wave (reproducible low-amplitude signals between end of QRS complex to onset of T wave) in right precordial

leads (V

1 R, V

2 R, V

3 R)

Minor Late potentials on signal-averaged ECG in one or more of three parameters in absence of QRS duration ! 110 msec

on standard ECG

Filtered QRS duration ! 114 msec

Duration of terminal QRS , 40 m V (low-amplitude signal duration) ! 38 msec

Root mean square voltage of terminal 40 msec " 20 m V

Terminal activation duration of QRS ! 55 msec measured from nadir of S wave to end of QRS, including R 9 , in V

1 , V

2 , or V

3 , in

absence of complete right bundle-branch block

Arrhythmias

Major Nonsustained or sustained ventricular tachycardia of RV outﬂ ow conﬁ guration, left bundle-branch block morphology

with inferior axis (positive QRS in leads II, III, and aVF and negative in aVL), or of unknown axis

Minor . 500 ventricular extra systoles per 24 h (Holter)

Family History

Major ARVC/D conﬁ rmed in ﬁ rst-degree relative who meets current task force criteria

ARVC/D conﬁ rmed pathologically at autopsy or surgery in ﬁ rst-degree relative

Identiﬁ cation of pathogenic mutation categorized as associated or probably associated with ARVC/D in patient being evaluated

Table 5 (Continues)

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 411

Type of Criterion and Modality Criteria

Minor History of ARVC/D in ﬁ rst-degree relative in whom it is not possible or practical to determine whether the family member meets

current task force criteria

Premature sudden death ( , 35 y old) due to possible ARVC/D in ﬁ rst-degree relative

ARVC/D conﬁ rmed pathologically or per current task force criteria in second-degree relative

Note.—Adapted and reprinted, with permission, from reference 35. Diagnostic terminology: for deﬁ nite diagnosis, two major OR one major and two minor OR four minor criteria from different

categories; for borderline diagnosis, one major and one minor OR three minor criteria from different categories; for possible diagnosis, one major OR two minor criteria from different categories.

ARVC/D = arrhythmogenic RV cardiomyopathy/dysplasia, BSA = body surface area, ECG = electrocardiogram, PLAX = parasternal long-axis view, PSAX = parasternal short-axis view, RVOT = RV

outﬂ ow tract, 2D = two-dimensional.

* A pathogenic mutation is a DNA alteration associated with ARVC/D that alters or is expected to alter the encoded protein, that is not observed or is rare in a large control population without ARVC/D,

and that either alters or is predicted to alter the structure or function of the protein or has a demonstrated linkage to the disease phenotype in a conclusive pedigree.

Table 5 (Continued)

Modiﬁ ed Major and Minor Criteria for Arrhythmogenic RV Dysplasia

Figure 2

Figure 2: ARVD in a 38-year-old woman.

(a) Horizontal-long-axis T1-weighted fast spin-

echo MR image without fat saturation shows ex-

tensive fat inﬁ ltration in RV free wall (arrows). Note

similar signal intensity characteristics to epicardial

fat. (b) Horizontal-long-axis T1-weighted spin-

echo MR image with fat saturation shows signal

drop-out in RV wall, conﬁ rming intramyocardial fat

(arrows). (c) Horizontal-long-axis SSFP MR image

from a more caudal level shows focal aneurysms

(arrow) in RV free wall.

ther at the cost of decreased signal-to-

noise ratio. Careful attention should be

given to the LV, which may be involved

in the cardiomyopathy, on images from

all sequences if possible ( 38 ). Note that

the recent modiﬁ ed task force criteria

emphasize global or regional dysfunc-

tion and structural alterations (Movie 5

[online]). Relatively recently, myocar-

dial LE has been used to demonstrate

scarring of the RV wall. The extent of

late enhancement has shown an excel-

lent correlation with histopathologic

ﬁ ndings and is predictive of inducible

ventricular tachycardia ( 39 ).

Cardiac MR imaging for ARVD carries

a high sensitivity but low speciﬁ city

when compared with traditional task

force criteria ( 40 ).This high sensitivity

may in part be attributed to the rigor-

ous limitations of the task force guide-

lines, which easily demonstrate the overt

forms of the disease. Because the task

force criteria have been suggested to be

relatively insensitive to less overt forms

of the disease ( 41 ), modiﬁ ed criteria have

been proposed, such that the presence

of any minor criterion in a ﬁ rst-degree

relative of a patient with proved ARVD

is regarded as clinical disease expres-

sion ( 35 ). When cardiac MR images

are assessed by using these modiﬁ ed

criteria, they frequently show abnormal

ﬁ ndings, which suggests a role in depict-

ing initial manifestations of disease

( 35 , 41 ). In one study ( 41 ), cardiac

MR imaging had 96% sensitivity and

78% speciﬁ city in a genotyped subset

of patients, only 46% of whom had

satisﬁ ed the task force criteria. In that

study, the addition of the cardiac MR

results would have enabled diagnosis

in a further 30% of proved gene car-

riers and in 75% of patients who pro-

spectively satisﬁ ed the modiﬁ ed criteria

only. That said, such an approach would

lead to an increased number of false-

positive cases in patients who subse-

quently turn out to be genotype negative.

Risk stratiﬁ cation in asymptomatic gene

carrier relatives of an index case has

not been fully elucidated.

It is important when interpreting

cardiac MR images for ARVD to be

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

412 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Figure 3: LV noncompaction in a 46-year-old

woman. (a) Vertical-long-axis SSFP MR image

shows marked increase in the noncompacted layer

of myocardium (arrow), such that the ratio of com-

pacted to noncompacted myocardium at the apical

level was greater than 2.3, consistent with LV non-

compaction. (b) Vertical-long-axis late-gadolinium-

enhanced MR image demonstrates enhancement in

the LV trabeculae, an appearance associated with a

more advanced stage of disease.

Figure 3

aware that fatty inﬁ ltration of the RV

free wall has been found in healthy sub-

jects, elderly patients, obese individuals,

long-term steroid users, and in cases of

idiopathic RV outﬂ ow tract tachycardia

( 42 ). Furthermore, RV myocardial late

enhancement has been described in

chronic right-sided myocarditis, sarcoid-

osis, Chagas disease, and Enterobacter

infection ( 37 ). We emphasize that the

interpretation of cardiac MR imaging

abnormalities should be made with cau-

tion and should not lead to a diagnosis

of ARVD on the basis of cardiac MR ﬁ nd-

ings alone. At our center, we recom-

mend a multidisciplinary approach that

involves family history, electrocardio-

gram and Holter ﬁ ndings, imaging, and

results from histopathologic and genetic

analyses before making a diagnosis of

ARVD ( 35 , 37 ).

LV no nco mpac tion .— LV non comp ac -

tion is currently classiﬁ ed as a primary

genetic cardiomyopathy ( 9 ). It is char-

acterized by the presence of an exten-

sive noncompacted myocardial layer

lining the cavity of the LV and impaired

LV systolic function. Many patients

are asymptomatic and have normal LV

systolic function, but others develop

cardiac failure, thromboembolism, and

malignant arrhythmias ( 43 , 44 ). The

compaction ratio can be quanti tatively

analyzed by measuring the thickness (in

millimeters) of the noncompacted myo-

cardium, as compared with the com-

pacted myocardium. This can be done

on a myocardial segmental basis by

using the standard 17-segment Ameri-

can Heart Association model ( 17 ). A

noncompacted-to-compacted ratio greater

than 2.3 on cardiac MR images is con-

sidered diagnostic of LV noncompaction

( 45 ). The apical antero- and inferolat-

eral segments of the LV are the most

commonly affected. RV involvement has

also been reported ( 46 ).

Important cardiac MR sequences in

LV noncompaction.— SSFP sequences

can clearly demonstrate noncompaction

features, which appear as a loose net-

work of interwoven trabeculae associ-

ated with deep myocardial recesses

( Fig 3 , Movie 6 [online]). This noncom-

pacted trabecular layer may demon-

strate perfusion defects after gadolinium

chelate injection, suggesting micro-

circulatory abnormalities ( 47 ). Late-

gadolinium-enhancement sequences may

depict trabecular late enhancement, which

appears to improve the correlation be-

tween cardiac MR ﬁ ndings and progres-

sive clinical stages of disease ( Fig 3 )

( 48 ). An advantage of cardiac MR im-

aging is the ability to acquire images in

any obliquity. In our experience, radial

vertical-long-axis projections ensure

that each section acquired passes through

the center of the ventricle and the apex,

minimizing the potential to overesti-

mate the ratio of compacted to non-

compacted myocardium.

Mixed Cardiomyopathies

Dilated cardiomyopathy.— Dilated

car diomyopathy is characterized by di-

latation of the cardiac chambers cou-

pled with impaired contraction of the

ventricles. The ventricular chambers

exhibit increased diastolic and systolic

volume and a low ejection fraction ( 49 ).

There are many causes, but the ma-

jority (50%) are idiopathic ( 50 ). Idio-

pathic dilated cardiomyopathy is the most

common cause of heart failure in the

young, with an estimated prevalence of

at least 36.5 per 100 000 persons in the

United States. The symp toms and signs

at presentation are progressive dysp-

nea and orthopnea in the majority of

patients. Arrhythmias and sudden death

may also occur.

Histopathologic features demon-

strate interstitial ﬁ brosis and a nu-

meric decrease in myocyte units. Al-

terations in the genetic expression of

proteins that regulate cardiac muscle

contraction have been detected ( 51 ).

Such abnormalities may be patchy, re-

sulting in a low diagnostic yield from

myocardial biopsy. Echocardiography

remains the principal examination for

dilated cardiomyopathy, but cardiac

MR imaging provides an optimal as-

sessment of chamber size and systolic

dysfunction. Systolic dysfunction is

the most important independent pre-

dictor of outcome in dilated cardio-

myopathy, and evaluation of diastolic

ﬁ lling allows further identiﬁ cation of

subgroups with divergent long-term

prognoses ( 52 ).

Important cardiac MR sequences

in dilated cardiomyopathy.— Late-

enhancement sequences have value in

the examination of patients suspected

of having dilated cardiomyopathy ( Fig 4 ,

Movie 7 [online]). Late-enhancement

MR images may demonstrate areas of

ﬁ brosis within the myocardium, char-

acteristically in the mid- or subepicar-

dial myocardium, allowing differentiation

from ischemic cardiomyopathy ( 52 ). A

subgroup of patients with dilated car-

diomyopathy will have ﬁ brosis in a pre-

dominantly subendocardial distribution,

characteristic of infarction (it has been

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Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 413

Figure 4: Idiopathic dilated cardiomyopathy in

a 28-year-old woman with shortness of breath.

(a) Four-chamber SSFP MR image shows marked

dilation of LV, which measures 68 mm. Note black

jet (arrow) from mitral regurgitation, a common

ﬁ nding in dilated cardiomyopathy. (b) Short-axis

late-gadolinium-enhanced MR image shows en-

hancement indicative of scar in midmyocardium of

interventricular septum (arrows). Such a ﬁ nding

indicates a poorer prognosis in dilated

cardiomyopathy.

Figure 4

suggested that these may represent

coronary emboli–induced ischemic car-

diomyopathy cases or ruptured coronary

plaques that have subsequently recana-

lized) ( 53 ). The presence of midwall ﬁ -

brosis on cardiac MR images has been

shown to have prognostic implications

in patients with dilated cardiomyopathy

as an independent predictor of the

combined endpoint of mortality from

all causes and cardiovascular hospitali-

zation and of the development of ven-

tricular tachycardias ( 54 ).

Restrictive cardiomyopathy.— This

condition is characterized by ventric-

ular diastolic dysfunction and resultant

biatrial enlargement with relatively nor-

mal ventricular size and systolic func-

tion ( 55 ). The pathophysiologic changes

are due to reduced myocardial compli-

ance, which elevates ventricular pres-

sures with small increases in volume

causing decreased ﬁ lling in diastole, re-

sulting in diastolic heart failure with

preservation of systolic function ( 56 , 57 ).

Symptoms range from increasing dysp-

nea and exercise intolerance to palpita-

tions, syncopal attacks and conduction

disturbances.

Cases may be divided into myocar-

dial or endomyocardial ( 23 ). The myo-

cardial causes may be subdivided into

noninﬁ ltrative, inﬁ ltrative and storage

disease and the endomyocardial sub-

group are made up of a number of un-

derlying causes including endomyocar-

dial ﬁ brosis, drugs, anthracyclines and

carcinoid ( 56 ). Clinically, restrictive

cardiomyopathy is frequently difﬁ cult to

distinguish from constrictive pericardi-

tis ( 58 ). Identifying pericardial thicken-

ing and nonbreakage of myocardial tag

lines across the pericardium helps dis-

tinguish these conditions ( 59 , 60 ). En-

domyocardial biopsy may also be help-

ful in distinguishing restrictive from

constrictive cardiomyopathy, particu-

larly if entities such as amyloidosis or

hemochromatosis are suspected clini-

cally ( 23 ). However, if pericardial thick-

ening or CMR features of restrictive

cardiomyopathy are identiﬁ ed and are

compatible with the clinical and echo-

cardiographic picture of constrictive or

restrictive physiology then biopsy may

be obviated.

Important cardiac MR sequences

for restrictive cardiomyopathy.— Con-

sensus statements have clariﬁ ed several

indexes used to measure diastolic heart

failure ( 61 ). SSFP images typically show

the heart with biatrial enlargement and

normal ventricular size ( Fig 5 , Movie 8

[online]). Several parameters of ven-

tricular relaxation can be calculated from

the short-axis SSFP stack, including time

to peak ﬁ lling, ear ly diastoli c ﬁ lling time,

and rate of peak ﬁ lling ( 62 ), which are

altered in restrictive cardiomyopathy.

Studies have also shown the feasibility

of using phase-velocity–encoding se-

quences to mea sure mitral inﬂ ow (the

E/A ratio) with good correlations with

echocardiographic ﬁ ndings ( 63 ). The

E/A ratio is a transmitral ﬂ ow measure-

ment of peak ﬂ ow velocity in early

diastole (E wave) and during atrial con-

traction (A wave). In diastolic dysfunc-

tion, a greater portion of end-diastolic

volume results from late ﬁ lling rather

than early ﬁ lling because of the stiffness

of the ventricle, therefore reducing the

E/A ratio.

A further useful sequence in restric-

tive cardiomyopathy is the application

of myocardial tags. This technique pro-

vides detailed quantitative assessment

of myocardial strain or deformation

seen in diastolic dysfunction ( 64

Acquired Cardiomyopathies

Inﬂ ammatory (myocarditis).— Myo-

cardial inﬂ ammation is a nonspeciﬁ c

response to various insults such as vi-

ral or bacterial infection, cardiotoxic

agents, catecholamines, infarction, or

mechanical injury ( 9 ). In North America

and Europe, viral infection remains the

commonest cause. Several viruses have

been associated with myocarditis, based

on the detection of viral genome within

cardiac tissue. The commonest include

Coxsackie B virus, non-Coxsackie en-

teroviruses, certain strains of adeno-

virus, parvovirus B19, and Epstein-Barr

virus ( 65 ). The sequela of severe viral

myocarditis is dilated cardiomyopathy,

which is thought to occur in up to 10%

of cases ( 66 ). Myocarditis may manifest

with a variety of symptoms, including

chest pain, recent onset of heart fail-

ure, atrial or ventricular arrhythmias,

cardiogenic shock, or even sud den

death.

Endomyocardial biopsy results are

considered the reference standard in

diagnosis ( 67 ). The 1987 Dallas crite-

ria require lymphocytic inﬁ ltration as-

sociated with myocyte injury in the ab-

sence of ischemia. Such criteria are

very speciﬁ c but have a low sensitivity,

related to sampling error caused by

patchy involvement of the myocardium

( 68 ), and high interobserver variability

in interpretation ( 69 ).

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

414 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

Important cardiac MR sequences in

myocarditis.— T2-weighted fast spin-echo

MR imaging has been found to be useful

in cases of acute myocarditis, because it

depicts edema ( 70 ). The sequence is a

breath-hold black-blood T2-weighted

triple inversion-recovery sequence (rep-

etition time/echo time msec/inversion

time msec, two R-R intervals/65/140);

typically, three to four short-axis sec-

tions (8–12-mm thick, echo train length

of 32, 256 3 256 matrix) are acquired

( Fig 6 ). An edema ratio, which is a quan-

titative measure of active inﬂ ammation ,

can be calculated based on the ratio of

abnormal to normal myocardial signal

intensity, with values greater than or

equal to 2 being considered abnormal.

To calculate the edema ratio from T2-

weighted images, a region of interest

encompassing the entire LV myocar-

dium and a second region of interest

encompassing the entire visible right

erector spinae or latissimus dorsi muscle

(skeletal muscle), depending on the ho-

mogeneity of the muscle signal inten-

sity, are recorded on the same section.

The mean myocardial signal intensity

(SI

myo ) is related to the mean skeletal

muscle signal intensity (SI

skm ) by using

the equation ER = SI

myo /SI

skm , where ER

is the edema ratio. An ER greater than

or equal to 2 is used to determine the

presence of active inﬂ ammation ( 70 ). It

should be noted that several problems

exist with T2-weighted sequences for

cardiac MR imaging: (a) Phased-array

coils cause regional myocardial signal

variation; (b) slow-ﬂ owing blood in

the trabeculae results in high signal

intensity adjacent to the endocardium,

which can make subendocardial abnor-

malities difﬁ c ult to interpret ; (c) through-

plane motion reduces cardiac signal

intensity; and (d) qualitative analysis is

subjective. Thus, when calculating the

edema ratio care must be taken that an

efﬁ cient coil intensity-correction algo-

rithm is implemented, and quantitative

analysis is recommended ( 71 ).

A useful second sequence for myo-

carditis makes use of T1-weighted spin-

echo or fast spin-echo sequences (R-R

interval/21, echo train length of four,

350–400-mm ﬁ eld of view, 512 3 512

matrix) performed with a body coil be-

fore and about 15 seconds after intrave-

nous injection of contrast material to

obtain ﬁ ve identical axial sections en-

compassing the myocardium ( Fig 6 )

( 72 ). A saturation band may be posi-

tioned across the atria to reduce the

signal from slow-ﬂ owing atrial blood. A

global enhancement ratio of myocardial

enhancement to skeletal muscle en-

hancement can be calculated, with

values greater than or equal to 4 being

considered abnormal. To calculate the

global enhancement ratio, regions of in-

terest that include both the entire LV

Figure 5: Diastolic heart failure in a 52-year-old

man. Horizontal-long-axis SSFP MR image shows

biatrial dilatation characteristic of diastolic heart

failure, with a regurgitant jet (arrow) consistent with

tricuspid regurgitation.

Figure 5

Figure 6: Acute myocarditis in a 28-year-old man. (a) Short-axis T2-weighted MR image shows increased

signal intensity in inferolateral segment (arrows) (edema ratio, 2.7). (b, c) Axial T1-weighted MR images

obtained (b) before and (c) immediately after gadolinium enhancement show increased myocardial global

enhancement relative to skeletal muscle. Global enhancement ratio, a quantitative measure of the difference

in signal intensity before and after contrast administration, was 4.2 (score . 4 is considered abnormal).

Note saturation band across the atria to null signal from slow-ﬂ owing blood. (d) Horizontal-long-axis late-

gadolinium-enhancement MR image shows enhancement that indicates myocardial edema and necrosis in

inferolateral segment, characteristic of acute myocarditis (straight arrow). Note two smaller foci of enhance-

ment in anterior segment (curved arrow).

Figure 6

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 415

Figure 7: Peripartum cardiomyopathy 6 days after

delivery in a 27-year-old woman. Her pregnancy

was complicated by preeclampsia at 33 weeks for

which she underwent emergency caesarian section.

She developed progressive dyspnea postpartum.

Computed tomographic pulmonary angiogram

(not shown) was negative for pulmonary embolism.

Short-axis SSFP MR images at (a) diastole and

(b) systole show akinesis of lateral segments (arrow)

during systole. LV ejection fraction was 42%.

Figure 7

ure, and (c) absence of demonstrable

heart disease before the last month of

pregnancy. Cardiac MR imaging is a

useful tool to conﬁ rm the presence of

cardiomyopathy ( 76 ). Risk factors for

peripartum cardiomyopathy include ad-

vanced maternal age, multiparity, Afri-

can race, twins, gestational hypertension,

and tocolysis ( 77 ). Cardiac MR is also a

useful tool for follow-up, with only half

of patients recovering full function

( 78 ).

Important cardiac MR sequences

for peripartum cardiomyopathy.— Spe-

ciﬁ c cardiac MR imaging appearances

have been relatively recently described

in patients with peripartum cardiomy-

opathy ( Fig 7 , Movie 9 [online]) ( 78 , 79 ).

Cine SSFP sequences allow an excellent

assessment of LV function ( 79 ). Late

gadolinium enhancement may demon-

strate late enhancement predominately

in the midmyocardium, involving the

anterior and anterolateral segments

( 80 ). These abnormalities may regress

over time, corresponding to an improve-

ment in LV function.

Gadolinium-based contrast agents

should be used with caution during

pregnancy. Although there are no docu-

mented teratogenic effects, evidence in

the literature is scarce and predomi-

nantly relies on animal data and limited

case series. Gadopentetate dimeglumine

inadvertently administered in three cases

at 1, 3, and 5 months gestation and in

another 11 women at 16–37 weeks ges-

tation were not associated with subse-

quent fetal abnormality ( 81 , 82 ), Gado-

pentetate dimeglumine (0.1 mmol/kg)

used to image the placenta was not as-

sociated with adverse effects on the fe-

tus. Similarly, no negative outcomes were

experienced in a study of 27 women

who were administered gadolinium in

the ﬁ rst trimester of pregnancy ( 83 ).

Takotsubo cardiomyopathy.— Takot-

subo cardiomyopathy is a relative recently

described cardiomyopathy provoked by

emotional stress and is most commonly

seen in postmenopausal women ( 84 ).

The clinical manifestation is similar to

that of acute myocardial infarction with

acute onset of congestive heart failure

and typical electrocardiogram changes

in the anterior leads, with absence of

signiﬁ cant coronary artery disease at

coronary angiogrpahy. Takotsubo car-

diomyopathy is characterized by an acute

temporary stunning of the apical myo-

cardium resulting in a transient apical

ballooning with bulging out of the LV

apex and a hypercontractile LV base. It

is likely that there are multiple causa-

tive factors, including vasospasm and

an abnormal response to catechol-

amines. Histopathologic analysis has

revealed that in the acute phase, vacu-

oles of different sizes induce cellular

hypertrophy along with increased in-

tracellular glycogen ( 85 ). Abnormal-

myocardium and the skeletal muscle at

the same level are mapped on unen-

hanced T1-weighted images and are

copied to the contrast-enhanced im-

ages. The average signal intensities of

the myocardium and skeletal muscles be-

fore and after contrast enhancement are

measured according to the following:

(SI

postmyo 2 SI

premyo )/SI

premyo and (SI

postskm

2 SI

preskm )/SI

preskm , where SI

postmyo and

premyo are myocardial signal intensity

after and before contrast enhancement,

respectively, and SI

postskm and SI

preskm

are skeletal muscle signal intensity after

and before enhancement, respectively.

Finally, late-enhancement sequences

may demonstrate late enhancement in

patients with acute myocarditis, which

is most characteristically depicted in

the inferolateral segment of the LV

( Fig 6 ), although changes in the septal

segments are also well described. Gen-

erally, when evaluating for myocarditis,

a combination of sequences is used,

with the highest diagnostic accuracy

being obtained when two of three are

positive (sensitivity, 76%; speciﬁ city,

95.5%; overall diagnostic accuracy,

85%) ( 70 , 73 ). Cardiac MR has particu-

lar clinical utility in the acute setting

because patients with acute myocarditis

often have an acute coronary syndrome

at presentation, and it may be difﬁ cult

to differentiate myocarditis from myo-

cardial infarction on the basis of clinical

ﬁ ndings.

Peripartum cardiomyopathy.— Peri-

partum cardiomyopathy is a rare car-

diomyopathy that occurs in previously

healthy women who develop symptoms

of heart failure during the peripartum

period ( 74 ). The cause of peripartum

cardiomyopathy is unknown, although it

is thought to originate from two broad

categories of disease, inﬂ ammatory and

noninﬂ ammatory. Of the inﬂ ammatory

causes, viral myocarditis is the most

common, whereas for noninﬂ ammatory

causes several factors are hypothesized

to play a role, including malnutrition, ge-

netics, hormone function, and increased

adrenergic tone ( 75 ). The diagnostic

criteria are (a) onset of heart failure in

the last month of pregnancy or in the

ﬁ rst 5 postpartum months, (b) absence

of a determinable cause of cardiac fail-

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

416 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

ities in contractile and cytoskeletal

proteins have been noted. Oncotic and

apoptotic cell death are not characteris-

tic. After functional recovery, these his-

topathologic abnormalities show complete

reversibility. The clinical corollary is that

if the patient survives the initial mani-

festation, the disease tends to resolve

with improved LV function within a few

days (a small subgroup may have a longer

recovery period) ( 86 ).

Important cardiac MR sequences in

takotsubo cardiomyopathy.— Cine SSFP

sequences in vertical-long-axis and

short-axis views allow a detailed assess-

ment of LV function, size, and regional

wall motion abnormalities in this car-

diomyopathy ( Fig 8 , Movie 10 [online]).

Two useful sequences for imaging takot-

subo cardiomyopathy are T2-weighted

and late-enhancement sequences. Find-

ings from both are characteristically

negative in this disease ( Fig 8 ). Thus,

cardiac MR is likely to play an increasing

role in helping to distinguish between

acute myocardial infarction and takot-

subo cardiomyopathy ( 87 ).

Secondary Cardiomyopathy

Cardiac Amyloidosis

Amyloidosis is classiﬁ ed into many dif-

ferent forms on the basis of the amy-

loid precursor protein: immunoglobu-

lin light chain (AL) derived–amyloidosis,

reactive (secondary) AA amyloidosis,

transthyretin (ATTR)-related heredi-

tary amyloidosis, and b 2-microglobulin

(Abeta2M)-derived dialysis-related am-

yloidosis ( 88 ). Primary systemic amy-

loidosis (immunoglobulin light chain)

and hereditary transthyretin (TTR) types

cause cardiomyopathies that result in

restrictive ventricular ﬁ lling and, usu-

ally, a poor prognosis ( 89 ). Cardiac ac-

cumulation of these various proteins in

the insoluble ﬁ brillar amyloid confor-

mation occurs principally in the myo-

cardial interstitium. There often is as-

sociated endomyocardial ﬁ brosis leading

to diastolic dysfunction.

Important cardiac MR sequences in

amyloidosis.— A diffuse decrease in sig-

nal intensity on T1-weighted fast spin-

echo images may be found in cardiac

amyloid, although this requires formal

measurement in a region of interest

( 90 , 91 ). Ventricular myocardial thick-

ening affects the right and left ventri-

cles, and a useful distinguishing feature

from HCM is that it generally results in

a diffuse rather than a focal pattern of

hypertrophy ( Fig 9 , Movie 11 [online]).

Thickening ( . 6 mm) of the interatrial

septum and posterior right atrial wall is

also suggestive of cardiac amyloidosis

( 63 ). Marked thickening of the LV wall

is associated with a survival time of less

than 6 months ( 64 ). Aside from the fast

spin-echo sequences, late-enhancement

sequences have been found to demon-

strate diffuse or subendocardial late en-

hancement ( Fig 9 ) ( 89 , 92 ). It can be

challenging to depict these areas on

cardiac MR images, because the total

amyloid protein load results in rapid

washout of gadolinium-based contrast

material from the myocardium. Thus, it

Figure 8

Figure 8: Takotsubo cardiomyopathy in a

73-year-old woman admitted with collapse and

chest pain after a road trafﬁ c accident. (a) Four-

chamber SSFP MR image shows apical ballooning

(arrows). (b) SSFP image during systole shows

severe apical hypokinesis (arrows) . (c) Late-

gadolinium-enhanced four-chamber MR image

shows absence of scar, which effectively excludes

diagnosis of myocardial infarction.

can be difﬁ cult to determine the opti-

mal inversion time to null normal myo-

cardium, because it may be unclear

which myocardial areas are normal

( 93 ). Typically, a short-axis image will

be acquired by using multiple inversion

times. The time at which remote myo-

cardium passes through the null point

should be used to render normal myo-

cardium as black regions and areas of

amyloid disposition as bright regions.

The time to commence imaging for late

enhancement is shorter than that for

other cardiomyopathies and should be-

gin early (5 minutes after contrast agent

injection). Focal enhancement corre-

lates signiﬁ cantly with areas of regional

hypokinesis or akinesis ( 92 ). When

compared with the diagnostic test of

choice of endomyocardial biopsy, car-

diac MR imaging provides good sensi-

tivity (80%) and high speciﬁ city (94%)

( 93 ). The authors of one study ( 94 )

showed that the abnormal gadolinium

kinetics, speciﬁ cally the 2-minute post-

gadolinium intramyocardial inversion

time difference between subepicardium

and subendocardium at a threshold

value of 23 msec, predicted mortality

with 85% accuracy.

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 417

Figure 9: Biopsy-proved amyloidosis in a

78-year-old man admitted with progressive dysp-

nea. (a) Horizontal-long-axis SSFP MR image shows

marked hypertrophy of LV, particularly the septum

(∗). Also note diffuse hypertrophy of RV free wall

(straight arrows) and thickening of interatrial septum

(curved arrow). (b) Late-gadolinium-enhanced

short-axis MR image shows extensive enhancement

in predominantly subendocardial distribution (ar-

rows), although it is almost transmural in certain

segments, such is the extent of the inﬁ ltration. Note

absence of conformation to any vascular territory.

Figure 9

Figure 10: Short-axis MR images in a 43-year-old

woman with palpitations and sudden loss of con-

sciousness and known history of mediastinal sar-

coidosis. (a) T2-weighted image shows

high-signal-intensity foci in midmyocardium, in the

interventricular septum and lateral wall (arrows),

indicative of acute myocardial edema. (b) Late-

gadolinium-enhanced image shows extensive inﬁ l-

tration of LV myocardium in a nonvascular territory

distribution.

Figure 10

Cardiac Sarcoidosis

Cardiac abnormalities are caused by

inﬁ ltration of sarcoid granulomas ( 95 ).

The classic clinical manifestation is

heart block; however, other clinical fea-

tures of sarcoid heart disease include

congestive heart failure, cor pulmonale,

supraventricular and ventricular ar-

rhythmias, conduction disturbances, ven-

tricular aneurysms, pericardial effu-

sion, and sudden death. About 7% of

patients with sarcoidosis develop car-

diac symptoms, but postmortem stud-

ies have revealed cardiac involvement

in 20%–50% of patients ( 96 ). Accurate

diagnosis relies on endomyocardial bi-

opsy ﬁ ndings for noncaseating granu-

lomas or on a positive biopsy result

from noncardiac tissue and cardiac ab-

normalities for which other possible

causes have been excluded ( 95 ).

Important cardiac MR sequences in

sarcoidosis.— T2-weighted fast spin-

echo images (usually short axis) may

depict sarcoid lesions as patchy hyper-

intense areas in myocardium, which

may be transmural and characteristi-

cally causes LV wall thinning ( Fig 10 ,

Movie 12 [online]) ( 97 ). Late gadolin-

ium enhancement may also demon-

strate sarcoid lesions ( 96 ). Such lesions

are characteristically patchy and usually

appear in the midwall and subepicar-

dium but may involve any layer of ven-

tricular myocardium and can mimic

coronary artery disease ( 98 ). Careful

attention should be paid to the RV myo-

cardium and RVOT, which can also

show enhancement. If a tissue diagno-

sis is required, cardiac MR imaging may

provide optimal guidance for endomyo-

cardial biopsy ( 99 ). Improvement in

cardiac symptoms and MR ﬁ ndings has

been observed after steroid therapy

( 97 , 100 ).

Siderotic Cardiomyopathy

Cardiac iron overload is a common

problem worldwide affecting patients

with b -thalassemia major ( 101 ). A mu-

tation in the b -globin gene causes de-

fective erythropoiesis that, in its homo-

zygous form, results in severe anemia.

Over 70% of these patients die of heart

failure. Measurement of myocardial iron

by using biopsy specimens is invasive

is likely to prevent the mortality seen

in patients with established ventricu-

lar dysfunction. The conventional treat-

ment for severe myocardial disease with

heart failure is long-term, continuous,

high-dose intravenous deferoxamine. Pre-

liminary evidence suggests that this ap-

proach is effective and may result in a

reversal of diastolic heart failure.

Important cardiac MR sequences in

iron overload.— Short-axis SSFP images

provide an accurate assessment of global

ventricular function that may be af-

fected in iron overload cardiomyopathy

and difﬁ cult to use for monitoring. Myo-

cardial iron content cannot be predicted

on the basis of serum ferritin or liver

iron levels, and conventional assess-

ments of cardiac function can only dem-

onstrate those with advanced disease.

Severe LV systolic dysfunction and heart

failure are signs of advanced cardiac si-

derosis, which usually does not respond

as well to chelation therapy as do ear-

lier disease stages. Early diagnosis and

treatment of myocardial iron overload

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

418 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

(Movie 13 [online]). Furthermore, sev-

eral studies have now proved the utility

of speciﬁ c T2*-weighted sequences for

noninvasive quantiﬁ cation of ventric-

ular iron deposition ( 102 ). The sequence

can be performed by acquiring a single

10-mm-thick short-axis midventricu-

lar section of the LV at 8–10 echo times

(2.6–16.7 msec at 2.02-msec increments)

with standard shimming in a single breath

hold and a ﬂ ip angle of 20°, a matrix of

128 3 128 pixels, a ﬁ eld-of-view of ap-

proximately 40 cm, and a sampling

bandwidth of 1950 Hz/pixel ( Fig 11a ).

A delay of 0 msec after the R-wave trigger

is usually chosen to obtain a high-quality

image when blood ﬂ ow and myocar-

dial wall motion artifacts are minimized.

A homogeneous full-thickness region of

interest is placed in the LV septum en-

compassing both epicardial and endo-

cardial regions. The signal intensity of

this region is measured for each image

and plotted against echo time to form

an exponential decay curve ( Fig 11b ).

To derive mean T2*, an exponential

trend line is ﬁ tted with an equation in

the form y = K

e 2 TE/T2*, where K

e is

a constant, TE is echo time, and y is the

signal intensity on the image ( Fig 11c ).

Several studies have shown a clear rela-

tionship between reduced myocardial

T2* ( , 20 msec), indicating iron over-

load, and LV dysfunction. Cardiac MR

imaging has been used to evaluate dif-

ferent chelation regimes speciﬁ cally for

their action on the myocardium, and

myocardial T2* increase correlates well

with the recovery of LV function after

intravenous iron chelation ( 101 , 102 ). The

sequence is also useful for other causes

of myocardial iron overload such as he-

mochromatosis, sickle cell disease, and

in cases where multiple blood transfu-

sions are required (eg, certain myelo-

dysplasias).

Scleroderma

Scleroderma is a connective tissue

disorder characterized by vascular

and ﬁ brotic lesions. It predominates

in the skin but can also be found in

the lungs, kidneys, and heart ( 103 ).

Myocardial ﬁ brosis has been docu-

mented to be a common ﬁ nding in pa-

thology and autopsy studies ( 104 ).

Figure 11

Figure 11: Iron overload cardiomyopathy

in an 83-year-old woman with a history of

multiple blood transfusions for myelodys-

plasia. (a) Short-axis T2*-weighted MR

image with region of interest (red) placed

in the septum. Note low signal intensity of

the liver, representing elevated hepatic iron

content. (b) Graph shows exponential

decay in T2* values echo time increases.

tial trend line ﬁ tted to the graph in b . In

this case, the mean T2* measured 12.7,

indicating severe myocardial iron overload.

The exact prevalence is difﬁ cult to as-

sess because patients often have oc-

cult disease, and autopsy studies are

biased toward those with the most se-

vere disease. The development of clin-

ical symptoms and signs is generally a

poor prognostic indicator, with a

5-year survival rate of 30%. The rea-

sons for this poorer prognosis include

the development of progressive RV

and LV failure, ventricular arrhyth-

mias, coronary artery disease, and

pericardial disease ( 105 ).

Important cardiac MR sequences in

scleroderma.— Speciﬁ c cardiac MR ap-

pearances have been relatively recently

described for late-enhancement se-

quences ( Fig 12 , Movie 14 [online])

( 106 ). Late-enhancing myocardial seg-

ments may be identiﬁ ed in up to 66% of

patients, with a characteristic linear

midmyocardial distribution predomi-

nantly affecting the basal and midven-

tricular levels. Its presence is associated

with development of arrhythmias.

Unknown Cardiomyopathy: A Practical

Approach

When a patient is referred for cardiac

MR imaging, an important initial task is

to ﬁ rst gain as much clinical informa-

tion about the suspected diagnosis Ob-

taining electrocardiographic and echo-

cardiographic ﬁ ndings is very valuable,

as well. Do not acquire or report on

cardiac MR images in a clinical vacu-

um—discuss the case with the refer-

ring doctor if possible. A practical im-

aging approach is to perform (after

acquisition of the appropriate localiz-

ers) a set of axial sequences through

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 419

the thorax to obtain a general view of

the anatomy of the heart and mediasti-

num followed by SSFP images in the

vertical-long-axis, horizontal-long-axis,

and short-axis image planes to assess

myocardial morphology and function.

If myocardial hypertrophy is identi-

ﬁ ed, speciﬁ c sequences for hypertro-

phic, amyloid, or other inﬁ ltrative car-

diomyopathy should be acquired. If

myocardial thinning is identiﬁ ed, spe-

ciﬁ c sequences for dilated cardiomy-

opathy or chronic myocardial infarction

should be performed. If biatrial enlarge-

ment is identiﬁ ed, speciﬁ c sequences

for a restrictive cardiomyopathy should

be performed. If there is a clinical sus-

picion of an acute myocardial process

such as acute myocardial infarction or

myocarditis, a stack of T2-weighted

images is also important. Further spe-

ciﬁ c sequences can be added, depend-

ing on the clinical question being asked.

Conclusion

Cardiac MR is an excellent imaging tech-

nique for assessing the morphologic

and functional characteristics of cardio-

myopathy. The use of late-enhancement

sequences has had an important effect

on the ability to characterize the myo-

cardium and also aids in improving clin-

ical risk stratiﬁ cation. Finally, the lack

of ionizing radiation makes cardiac MR

Figure 12: Short-axis late-gadolinium-enhanced

MR image in a 48-year-old man with a long history

of scleroderma and progressive dyspnea shows

extensive enhancement in anterior and inferior RV

insertion points (arrows).

Figure 12 imaging an important tool in screening

and serial assessment of progressive

myocardial diseases.

Disclosures of Potential Conﬂ icts of Interest:

D.H.O. No potential conﬂ icts of interest to dis-

close. S.A. Financial activities related to the pre-

sent article: none to disclose. Financial activities

not related to the present article: is a consultant

for Perceptive Informatics; has received a grant

or has a grant pending from Bracco. Other rela-

tionships: none to disclose. V.C. No potential

conﬂ icts of interest to disclose. K.Y. No potential

conﬂ icts of interest to disclose. R.P.K. No poten-

tial conﬂ icts of interest to disclose. R.M. No po-

tential conﬂ icts of interest to disclose. D.K. No

potential conﬂ icts of interest to disclose. R.C.C.

Financial activities related to the present article:

none to disclose. Financial activities not related

to the present article: is a consultant for Astellas

and GE Healthcare; has received grants or has

grants pending from Astellas and GE Healthcare.

Other relationships: none to disclose. J.D.D. No

potential conﬂ icts of interest to disclose.

References

1 . Bluemke DA , Ach enba ch S , Budoff M , et al .

Noninvasive coronary artery imaging: mag-

netic resonance angiography and multide-

tector computed tomography angiography:

a scientiﬁ c statement from the American

Heart Association Committee on Cardio-

vascular Imaging and Intervention of the

Council on Cardiovascular Radiology and

Intervention, and the Councils on Clinical

Cardiology and Cardiovascular Disease in

the Young . Circulation 2008 ; 11 8 ( 5 ): 586 – 606 .

2 . Finn JP , Nael K , Deshpande V , Ratib O ,

Laub G . Cardiac MR imaging: state of the

technology . Radiology 2006 ; 241 ( 2 ): 338 – 354 .

3 . Pennell DJ , Sechtem UP , Higgins CB , et al .

Clinical indications for cardiovascular mag-

netic resonance (CMR): Consensus Panel

report . Eur Heart J

2004 ; 25 ( 21 ): 1940 – 1965 .

4 . Hundley WG , Bluemke DA , Finn JP , et al .

ACCF/ACR/AHA/NASCI/SCMR 2010 expert

consensus document on cardiovascular

magnetic resonance: a report of the Amer-

ican College of Cardiology Foundation Task

Force on Expert Consensus Documents .

Circulation 2010 ; 121 ( 22 ): 2462 – 2508 .

5 . Roger VL , Go AS , Lloyd-Jones DM , et al .

Heart disease and stroke statistics—2011 up-

date: a report from the American Heart As-

sociation . Circulation 2011 ; 123 ( 4 ): e18 – e209 .

6 . Quiñones MA , Douglas PS , Foster E , et al .

American College of Cardiology/American

Heart Association clinical competence

statement on echocardiography: a report

of the American College of Cardiology/

American Heart Association/American

College of Physicians—American Society

of Internal Medicine Task Force on Clini-

cal Competence . Circulation 2003 ; 107 ( 7 ):

1068 – 1089 .

7 . Daniel WG , Erbel R , Kasper W , et al .

Safety of transesophageal echocardiogra-

phy. A multicenter survey of 10,419 exami-

nations . Circulation 1991 ; 83 ( 3 ): 817 – 821 .

8 . Fuster V , Kim RJ . Frontiers in cardiovascu-

lar magnetic resonance . Circulation 2005 ;

112 ( 1 ): 135 – 144 .

9 . Maron BJ , Towbin JA , Thiene G , et al .

Contemporary deﬁ

nitions and classiﬁ cation

of the cardiomyopathies: an American Heart

Association scientiﬁ c statement from the

council on clinical cardiology, heart failure

and transplantation committee; quality of

care and outcomes research and functional

genomics and translational biology inter-

disciplinary working groups; and Council on

Epidemiology and Prevention . Circulation

2006 ; 113 ( 14 ): 1807 – 1816 .

10 . Elliott P , Andersson B , Arbustini E , et al .

Classiﬁ cation of the cardiomyopathies: a

position statement from the European So-

ciety Of Cardiology Working Group on

Myocardial and Pericardial Diseases . Eur

Heart J 2008 ; 29 ( 2 ): 270 – 276 .

11 . Kramer CM , Barkhausen J , Flamm SD ,

Kim RJ , Nagel E ; Society for Cardiovascu-

lar Magnetic Resonance Board of Trustees

Task Force on Standardized Protocols .

Standardized cardiovascular magnetic res-

onance imaging (CMR) protocols, society

for cardiovascular magnetic resonance:

board of trustees task force on standard-

ized protocols . J Cardiovasc Magn Reson

2008 ; 10 : 35 .

12 . Evangelista A , Flachskampf F , Lancellotti

P , et al . European Association of Echocar-

diography recommendations for standardi-

zation of performance, digital storage and

reporting of echocardiographic studies .

Eur J Echocardiogr 2008 ; 9 ( 4 ): 438 – 448 .

13 . Salton CJ , Chuang ML , O’Donnell CJ ,

et al . Gender differences and normal left

ventricular anatomy in an adult population

free of hypertension. A cardiovascular mag-

netic resonance study of the Framingham

Heart Study Offspring cohort . J Am Coll

Cardiol 2002 ; 39 ( 6 ): 1055 – 1060 .

14 . Heidemann RM , Ozsarlak O , Parizel PM ,

et al . A brief review of parallel magnetic

resonance imaging . Eur Radiol 2003 ; 13 ( 10 ):

2323 – 2337 .

15 . Gupta A , Lee VS , Chung YC , Babb JS ,

Simonetti OP . Myocardial infarction: opti-

mization of inversion times at delayed

contrast-enhanced MR imaging . Radiology

2004 ; 233 ( 3 ): 921 – 926 .

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

420 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

16 . Huber AM , Schoenberg SO , Hayes C , et al .

Phase-sensitive inversion-recovery MR im-

aging in the detection of myocardial infarc-

tion . Radiology 2005 ; 237 ( 3 ): 854 – 860 .

17 . Cerqueira MD , Weissman NJ , Dilsizian V ,

et al . Standardized myocardial segmenta-

tion and nomenclature for tomographic im-

aging of the heart: a statement for health-

care professionals from the Cardiac Imaging

Committee of the Council on Clinical Car-

diology of the American Heart Association .

Circulation 2002 ; 105 ( 4 ): 539 – 542 .

18 . Cury RC , Cattani CAM , Gabure LAG , et al .

Diagnostic performance of stress perfu-

sion and delayed-enhancement MR imag-

ing in patients with coronary artery disease .

Radiology 2006 ; 240 ( 1 ):

39 – 45 .

19 . Hansen MW , Merchant N . MRI of hypertro-

phic cardiomyopathy: part I, MRI appear-

ances . AJR Am J Roentgenol 2007 ; 189 ( 6 ):

1335 – 1343 .

20 . Hansen MW , Merchant N . MRI of hyper-

trophic cardiomyopathy: part 2, Differen-

tial diagnosis, risk stratiﬁ cation, and post-

treatment MRI appearances . AJR Am J

Roentgenol 2007 ; 189 ( 6 ): 1344 – 1352 .

21 . Maron BJ , McKenna WJ , Danielson GK ,

et al . American College of Cardiology/

European Society of Cardiology clinical

expert consensus document on hyper-

trophic cardiomyopathy. A report of the

American College of Cardiology Founda-

tion Task Force on Clinical Expert Con-

sensus Documents and the European So-

ciety of Cardiology Committee for Practice

Guidelines . J Am Coll Cardiol 2003 ; 42 ( 9 ):

1687 – 1713 .

22 . Moon JC , Reed E , Sheppard MN ,

et al . The histologic basis of late gado-

linium enhancement cardiovascular mag-

netic resonance in hypertrophic cardiomy-

opathy . J Am Coll Cardiol 2004 ; 43 ( 12 ):

2260 – 2264 .

23 . Cooper LT , Baughman KL , Feldman AM ,

et al . The role of endomyocardial biopsy

in the management of cardiovascular disease:

a scientiﬁ c statement from the American

Heart Association, the American College of

Cardiology, and the European Society of

Cardiology . Circulation 2007 ; 11 6 ( 19 ):

2216 – 2233 .

24 . Rickers C , Wilke NM , Jerosch-Herold M ,

et al . Utility of cardiac magnetic resonance

imaging in the diagnosis of hypertrophic

cardiomyopathy . Circulation 2005 ; 112 ( 6 ):

855 – 861 .

25 . Schulz-Menger J , Strohm O , Waigand J ,

Uhlich F , Dietz R , Friedrich MG . The value

of magnetic resonance imaging of the left

ventricular outﬂ ow tract in patients with

hypertrophic obstructive cardiomyopathy

after septal artery embolization . Circula-

tion 2000 ; 101 ( 15 ): 1764 – 1766 .

26 . Pet ers en SE , Selvanayagam JB , Francis JM ,

et al . Differentiation of athlete’s heart from

pathological forms of cardiac hypertrophy

by means of geometric indices derived from

cardiovascular magnetic resonance . J Car-

diovasc Magn Reson 2005 ; 7 ( 3 ): 551 – 558 .

27 . Moon JC , McKenna WJ , McCrohon JA ,

Elliott PM , Smith GC , Pennell DJ . Toward

clinical risk assessment in hypertrophic

cardiomyopathy with gadolinium cardio-

vascular magnetic resonance . J Am Coll

Cardiol 2003 ; 41 ( 9 ): 1561 – 1567 .

28 . Adabag AS , Maron BJ , Appelbaum E , et al .

Occurrence and frequency of arrhythmias

in hypertrophic cardiomyopathy in relation

to delayed enhancement on cardiovascu-

lar magnetic resonance . J Am Coll Cardiol

2008 ; 51 ( 14 ): 1369 – 1374 .

29 . Rubinshtein R , Glockner JF , Ommen SR ,

et al . Characteristics and clinical signiﬁ

cance of late gadolinium enhancement by

contrast-enhanced magnetic resonance im-

aging in patients with hypertrophic cardio-

myopathy . Circ Heart Fail 2010 ; 3 ( 1 ): 51 – 58 .

30 . O’Hanlon R , Grasso A , Roughton M , et al .

Prognostic signiﬁ cance of myocardial ﬁ bro-

sis in hypertrophic cardiomyopathy . J Am

Coll Cardiol 2010 ; 56 ( 11 ): 867 – 874 .

31 . Sipola P , Lauerma K , Jääskeläinen P , et al .

Cine MR imaging of myocardial contractile

impairment in patients with hypertrophic

cardiomyopathy attributable to Asp175Asn

mutation in the alpha-tropomyosin gene .

Radiology 2005 ; 236 ( 3 ): 815 – 824 .

32 . Liberthson RR . Sudden death from cardiac

causes in children and young adults .

N Engl

J Med 1996 ; 334 ( 16 ): 1039 – 1044 .

33 . Thiene G , Nava A , Corrado D , Rossi L ,

Pennelli N . Right ventricular cardiomyop-

athy and sudden death in young people .

N Engl J Med 1988 ; 318 ( 3 ): 129 – 133 .

34 . McKenna WJ , Thiene G , Nava A , et al . Di-

agnosis of arrhythmogenic right ventricular

dysplasia/cardiomyopathy. Task Force of

the Working Group Myocardial and Peri-

cardial Disease of the European Society of

Cardiology and of the Scientiﬁ c Council on

Cardiomyopathies of the International So-

ciety and Federation of Cardiology . Br

Heart J 1994 ; 71 ( 3 ): 215 – 218 .

35 . Marcus FI

, McKenna WJ , Sherrill D , et al .

Diagnosis of arrhythmogenic right ventric-

ular cardiomyopathy/dysplasia: proposed

modiﬁ cation of the task force criteria . Cir-

culation 2010 ; 121 ( 13 ): 1533 – 1541 .

36 . Castillo E , Tandri H , Rodriguez ER , et al .

Arrhythmogenic right ventricular dyspla-

sia: ex vivo and in vivo fat detection with

black-blood MR imaging . Radiology 2004 ;

232 ( 1 ): 38 – 48 .

37 . Jain A , Tandri H , Calkins H , Bluemke DA .

Role of cardiovascular magnetic resonance

imaging in arrhythmogenic right ventricu-

lar dysplasia . J Cardiovasc Magn Reson

2008 ; 10 : 32 .

38 . Sen-Chowdhry S , Syrris P , Prasad SK , et al .

Left-dominant arrhythmogenic cardiomy-

opathy: an under-recognized clinical entity .

J Am Coll Cardiol 2008 ; 52 ( 25 ): 2175 – 2187 .

39 . Tandri H , Saranathan M , Rodriguez ER ,

et al . Noninvasive detection of myocardial

ﬁ brosis in arrhythmogenic right ventricular

cardiomyopathy using delayed-enhancement

magnetic resonance imaging . J Am Coll

Cardiol 2005 ; 45 ( 1 ): 98 – 103 .

40 . Sen-Chowdhry S , Prasad SK , Syrris P , et al .

Cardiovascular magnetic resonance in ar-

rhythmogenic right ventricular cardiomyopa-

thy revisited: comparison with task force

criteria and genotype . J Am Coll Cardiol

2006 ; 48

( 10 ): 2132 – 2140 .

41 . Sen-Chowdhry S , Syrris P , War d D ,

Asimaki A , Sevdalis E , McKenna WJ . Clin-

ical and genetic characterization of families

with arrhythmogenic right ventricular dys-

plasia/cardiomyopathy provides novel in-

sights into patterns of disease expression .

Circulation 2007 ; 115 ( 13 ): 1710 – 1720 .

42 . Tandri H , Macedo R , Calkins H , et al . Role

of magnetic resonance imaging in ar-

rhythmogenic right ventricular dysplasia:

insights from the North American ar-

rhythmogenic right ventricular dysplasia

(ARVD/C) study . Am Heart J 2008 ; 155 ( 1 ):

147 – 153 .

43 . Murphy RT , Thaman R

, Blanes JG , et al .

Natural history and familial characteristics

of isolated left ventricular non-compaction .

Eur Heart J 2005 ; 26 ( 2 ): 187 – 192 .

44 . Oechslin EN , Attenhofer Jost CH , Rojas

JR , Kaufmann PA , Jenni R . Long-term fol-

low-up of 34 adults with isolated left ven-

tricular noncompaction: a distinct cardio-

myopathy with poor prognosis . J Am Coll

Cardiol 2000 ; 36 ( 2 ): 493 – 500 .

45 . Petersen SE , Selvanayagam JB , Wiesmann

F , et al . Left ventricular non-compaction:

insights from cardiovascular magnetic res-

onance imaging . J Am Coll Cardiol 2005 ;

46 ( 1 ): 101 – 105 .

46 . Burke A , Mont E , Kutys R , Virmani R . Left

ventricular noncompaction: a pathological

study of 14 cases . Hum Pathol 2005 ; 36 ( 4 ):

403 – 411 .

47 . Weiford BC , Subbarao VD , Mulhern KM .

Noncompaction of the ventricular myocar-

dium . Circulation 2004 ; 109 ( 24 ): 2965 – 2971 .

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

Radiology: Volume 262: Number 2—February 2012 n radiology.rsna.org 421

48 . Dodd JD , Holmvang G , Hoffmann U , et al .

Quantiﬁ cation of left ventricular noncom-

paction and trabecular delayed hyperen-

hancement with cardiac MRI: correlation

with clinical severity . AJR Am J Roentgenol

2007 ; 189 ( 4 ): 974 – 980 .

49 . Richardson P , McKenna W , Bristow M ,

et al . Report of the 1995 World Health Or-

ganization/International Society and Fed-

eration of Cardiology Task Force on the

Deﬁ nition and Classiﬁ cation of cardiomy-

opathies . Circulation 1996 ; 93 ( 5 ): 841 – 842 .

50 . Felker GM , Hu W , Hare JM , Hruban RH ,

Baughman KL ,

Kasper EK . The spectrum

of dilated cardiomyopathy. The Johns Hop-

kins experience with 1,278 patients . Medi-

cine (Baltimore) 1999 ; 78 ( 4 ): 270 – 283 .

51 . Carlquist JF , Menlove RL , Murray MB ,

O’Connell JB , Anderson JL . HLA class II

(DR and DQ) antigen associations in idio-

pathic dilated cardiomyopathy. Validation

study and meta-analysis of published HLA

association studies . Circulation 1991 ; 83 ( 2 ):

515 – 522 .

52 . Rihal CS , Nishimura RA , Hatle LK , Bailey

KR , Ta ji k AJ . Systolic and diastolic dysfunc-

tion in patients with clinical diagnosis of

dilated cardiomyopathy. Relation to symp-

toms and prognosis . Circulation 1994 ;

90 ( 6 ): 2772

– 2779 .

53 . McCrohon JA , Moon JC , Prasad SK , et al .

Differentiation of heart failure related to

dilated cardiomyopathy and coronary artery

disease using gadolinium-enhanced cardio-

vascular magnetic resonance . Circulation

2003 ; 108 ( 1 ): 54 – 59 .

54 . Assomull RG , Prasad SK , Lyne J , et al .

Cardiovascular magnetic resonance, ﬁ brosis,

and prognosis in dilated cardiomyopathy .

J Am Coll Cardiol 2006 ; 48 ( 10 ): 1977 – 1985 .

55 . Kushwaha SS , Fallon JT , Fuster V . Restric-

tive cardiomyopathy . N Engl J Med 1997 ;

336 ( 4 ): 267 – 276 .

56 . Zile MR , Lewinter MM . Left ventricular end-

diastolic volume is normal in patients with

heart failure and a normal ejection fraction:

a renewed consensus in diastolic heart fail-

ure . J Am Coll Cardiol 2007 ; 49 ( 9 ): 982 – 985 .

57 . Zile MR , Gaasch WH , Carroll JD , et al .

Heart failure with a normal ejection frac-

tion: is measurement of diastolic function

necessary to make the diagnosis of dia-

stolic heart failure? Circulation 2001 ;

104 ( 7 ): 779 – 782 .

58 . Wilmshurst PT , Katritsis D . Restrictive

cardiomyopathy . Br Heart J 1990 ; 63 ( 6 ):

323 – 324 .

59 . Kojima S , Yamada N , Goto Y . Diagnosis of

constrictive pericarditis by tagged cine

magnetic resonance imaging . N Engl J Med

1999 ; 341 ( 5 ): 373 – 374 .

60 . Masui T , Finck S , Higgins CB . Constrictive

pericarditis and restrictive cardiomyopa-

thy: evaluation with MR imaging . Radiol-

ogy 1992 ; 182 ( 2 ): 369 – 373 .

61 . Paulus WJ , Ts chöp e C , Sanderson JE , et al .

How to diagnose diastolic heart failure:

a consensus statement on the diagnosis

of heart failure with normal left ventricular

ejection fraction by the Heart Failure and

Echocardiography Associations of the Eu-

ropean Society of Cardiology . Eur Heart J

2007 ; 28 ( 20 ): 2539 –

2550 .

62 . Kudelka AM , Turner DA , Liebson PR ,

Macioch JE , Wang JZ , Barron JT . Compar-

ison of cine magnetic resonance imaging

and Doppler echocardiography for evalua-

tion of left ventricular diastolic function .

Am J Cardiol 1997 ; 80 ( 3 ): 384 – 386 .

63 . Rathi VK , Doyle M , Yamrozik J , et al . Rou-

tine evaluation of left ventricular diastolic

function by cardiovascular magnetic reso-

nance: a practical approach . J Cardiovasc

Magn Reson 2008 ; 10 : 36 .

64 . Yeon SB , Reichek N , Tallant BA , et al . Val-

idation of in vivo myocardial strain mea-

surement by magnetic resonance tagging

with sonomicrometry .

J Am Coll Cardiol

2001 ; 38 ( 2 ): 555 – 561 .

65 . Mahrholdt H , Wagner A , Deluigi CC ,

et al . Presentation, patterns of myocar-

dial damage, and clinical course of viral

myocarditis . Circulation 2006 ; 114 ( 15 ):

1581 – 1590 .

66 . Gutberlet M , Spors B , Thoma T , et al . Sus-

pected chronic myocarditis at cardiac MR:

diagnostic accuracy and association with

immunohistologically detected inﬂ amma-

tion and viral persistence . Radiology 2008 ;

246 ( 2 ): 401 – 409 .

67 . Karatolios K , Pankuweit S , Maisch

B . Di-

agnosis and treatment of myocarditis: the

role of endomyocardial biopsy . Curr Treat

Options Cardiovasc Med 2007 ; 9 ( 6 ):

473 – 481 .

68 . Feldman AM , McNamara D . Myocarditis .

N Engl J Med 2000 ; 343 ( 19 ): 1388 – 1398 .

69 . Chow LH , Radio SJ , Sears TD , McManus

BM . Insensitivity of right ventricular en-

domyocardial biopsy in the diagnosis of

myocarditis . J Am Coll Cardiol 1989 ; 14 ( 4 ):

915 – 920 .

70 . Abdel-Aty H , Boyé P , Zagrosek

A , et al .

Diagnostic performance of cardiovascular

magnetic resonance in patients with sus-

pected acute myocarditis: comparison of

different approaches . J Am Coll Cardiol

2005 ; 45 ( 11 ): 1815 – 1822 .

71 . Giri S , Chung YC , Merchant A , et al . T2

quantiﬁ cation for improved detection of

myocardial edema . J Cardiovasc Magn

Reson 2009 ; 11 : 56 .

72 . Friedrich MG , Strohm O , Schulz-Menger

J , Marciniak H , Luft FC , Dietz R . Contrast

media-enhanced magnetic resonance imag-

ing visualizes myocardial changes in the

course of viral myocarditis . Circulation

1998 ; 97 ( 18 ): 1802 – 1809 .

73 . Friedrich MG , Sechtem U , Schulz-Menger

J , et al . Cardiovascular magnetic resonance

in myocarditis: A JACC White Paper . J Am

Coll Cardiol 2009 ; 53 ( 17 ): 1475 – 1487 .

74 . Abboud J , Murad Y , Chen-Scarabelli C ,

Saravolatz L , Scarabelli TM . Peripartum

cardiomyopathy: a comprehensive review .

Int J Cardiol 2007 ; 118 ( 3 ): 295 – 303 .

75 . Baruteau AE , Leurent G , Martins RP , et al .

Peripartum cardiomyopathy in the era of

cardiac magnetic resonance imaging: ﬁ rst

results and perspectives . Int J Cardiol

2010 ; 144 ( 1 ): 143 – 145 .

76 . Baruteau AE , Leurent G , Schleich JM ,

Gervais R , Daubert JC , Mabo P . Can peri-

partum cardiomyopathy be familial? Int J

Cardiol 2009 ; 137 ( 2 ): 183 – 185 .

77 . Sliwa K , Förster O , Libhaber E , et al .

Peripartum cardiomyopathy: inﬂ ammatory

markers as predictors of outcome in 100

prospectively studied patients . Eur Heart J

2006 ; 27 ( 4 ): 441 – 446 .

78 . Mouquet F , Lions C , de Groote P , et al .

Characterisation of peripartum cardiomy-

opathy by cardiac magnetic resonance im-

aging . Eur Radiol 2008 ; 18 ( 12 ): 2765 – 2769 .

79 . Kawano H , Tsuneto A , Koide Y , et al . Mag-

netic resonance imaging in a patient with

peripartum cardiomyopathy . Intern Med

2008 ; 47 ( 2 ): 97 – 102 .

80 . Marmursztejn J , Vignaux O , Gofﬁ net F ,

Cabanes L , Duboc D . Delayed-enhanced

cardiac magnetic resonance imaging fea-

tures in peripartum cardiomyopathy . Int J

Cardiol

2009 ; 137 ( 3 ): e63 – e64 .

81 . Shoenut JP , Semelka RC , Silverman R ,

Ya ff e CS , Micﬂ ikie r AB . MRI in the diagnosis

of Crohn’s disease in two pregnant women .

J Clin Gastroenterol 1993 ; 17 ( 3 ): 244 – 247 .

82 . Barkhof F , Heijboer RJ , Algra PR . Inadver-

tent i.v. administration of gadopentetate

dimeglumine during early pregnancy . AJR

Am J Roentgenol 1992 ; 158 ( 5 ): 1171 .

83 . De Santis M , Straface G , Cavaliere AF

Carducci B , Caruso A . Gadolinium pericon-

ceptional exposure: pregnancy and neona-

tal outcome . Acta Obstet Gynecol Scand

2007 ; 86 ( 1 ): 99 – 101 .

REVIEW: Cardiac MR Imaging of Nonischemic Cardiomyopathies O’Donnell et al

422 radiology.rsna.org n Radiology: Volume 262: Number 2—February 2012

84 . Sharkey SW , Windenburg DC , Lesser JR ,

et al . Natural history and expansive clinical

proﬁ le of stress (tako-tsubo) cardiomyopa-

thy . J Am Coll Cardiol 2010 ; 55 ( 4 ): 333 – 341 .

85 . Nef HM , Möllmann H , Kostin S , et al .

Tako-Tsubo cardiomyopathy: intraindividu-

al structural analysis in the acute phase

and after functional recovery . Eur Heart J

2007 ; 28 ( 20 ): 2456 – 2464 .

86 . Gianni M , Dentali F , Grandi AM , Sumner G ,

Hiralal R , Lonn E

. Apical ballooning syndrome

or takotsubo cardiomyopathy: a systematic

review . Eur Heart J 2006 ; 27 ( 13 ): 1523 – 1529 .

87 . Eitel I , Behrendt F , Schindler K , et al . Dif-

ferential diagnosis of suspected apical bal-

looning syndrome using contrast-enhanced

magnetic resonance imaging . Eur Heart J

2008 ; 29 ( 21 ): 2651 – 2659 .

88 . Kwong RY , Falk RH . Cardiovascular mag-

netic resonance in cardiac amyloidosis .

Circulation 2005 ; 111 ( 2 ): 122 – 124 .

89 . Maceira AM , Joshi J , Prasad SK , et al .

Cardiovascular magnetic resonance in car-

diac amyloidosis . Circulation 2005 ; 111 ( 2 ):

186 – 193 .

90 . vanden Driesen RI , Slaughter RE , Strugnell

WE . MR ﬁ ndings in cardiac amyloidosis .

AJR Am J Roentgenol 2006 ; 186 ( 6 ):

1682 – 1685 .

91 . Krombach GA , Hahn C , Tomars M , et al .

Cardiac amyloidosis: MR imaging ﬁ ndings

and T1 quantiﬁ ca tio n, compari son wi th con -

trol subjects . J Magn Reson Imaging 2007 ;

25 ( 6 ): 1283 – 1287 .

92 . Perugini E , Rapezzi C

, Piva T , et al . Non-

invasive evaluation of the myocardial sub-

strate of cardiac amyloidosis by gadolinium

cardiac magnetic resonance . Heart 2006 ;

92 ( 3 ): 343 – 349 .

93 . Vogelsberg H , Mahrholdt H , Deluigi CC ,

et al . Cardiovascular magnetic resonance

in clinically suspected cardiac amyloid-

osis: noninvasive imaging compared to

endomyocardial biopsy . J Am Coll Cardiol

2008 ; 51 ( 10 ): 1022 – 1030 .

94 . Maceira AM , Prasad SK , Hawkins PN ,

Roughton M , Pennell DJ . Cardiovascular

magnetic resonance and prognosis in car-

diac amyloidosis . J Cardiovasc Magn Reson

2008 ; 10 : 54 .

95 . Smedema JP , Snoep G , van Kroonenburgh

MPG , et al . The additional value of

gadolinium-enhanced MRI to standard as-

sessment for cardiac involvement in pa-

tients with pulmonary sarcoidosis . Chest

2005 ; 128 ( 3 ): 1629 – 1637 .

96 . Smedema JP , Snoep G , van Kroonenburgh

MP , et al . Evaluation of the accuracy of

gadolinium-enhanced cardiovascular mag-

netic resonance in the diagnosis of car-

diac sarcoidosis . J Am Coll Cardiol 2005 ;

45 ( 10 ): 1683 – 1690 .

97 . Vignaux O , Dhote R , Duboc D , et al . Clin-

ical signiﬁ cance of myocardial magnetic

resonance abnormalities in patients with

sarcoidosis: a 1-year follow-up study . Chest

2002 ;

122 ( 6 ): 1895 – 1901 .

98 . Mahrholdt H , Wagner A , Judd RM ,

Sechtem U , Kim RJ . Delayed enhancement

cardiovascular magnetic resonance assess-

ment of non-ischaemic cardiomyopathies .

Eur Heart J 2005 ; 26 ( 15 ): 1461 – 1474 .

99 . Uemura A , Morimoto S , Hiramitsu S , Kato

Y , Ito T , Hishida H . Histologic diagnostic

rate of cardiac sarcoidosis: evaluation of

endomyocardial biopsies . Am Heart J

1999 ; 138 ( 2 Pt 1 ): 299 – 302 .

100 . Ichinose A , Otani H , Oikawa M , et al . MRI

of cardiac sarcoidosis: basal and subepi-

cardial localization of myocardial lesions

and their effect on left ventricular func-

tion . AJR Am J Roentgenol 2008 ; 191 ( 3 ):

862 – 869 .

101 . Anderson LJ , Westwood MA , Holden S ,

et al . Myocardial iron clearance during re-

versal of siderotic cardiomyopathy with in-

travenous desferrioxamine: a prospective

study using T2* cardiovascular magnetic

resonance . Br J Haematol 2004 ; 127 ( 3 ):

348 – 355 .

102 . Tanner MA , Galanello R , Dessi C , et al . A

randomized, placebo-controlled, double-

blind trial of the effect of combined ther-

apy with deferoxamine and deferiprone

on myocardial iron in thalassemia major

using cardiovascular magnetic resonance .

Circulation 2007 ; 115 ( 14 ):

1876 – 1884 .

10 3 . Va rg a JA , Troj an owsk a M . Fibrosis in sys-

temic sclerosis . Rheum Dis Clin North Am

2008 ; 34 ( 1 ): 115 – 143, vii .

104 . Kahan A , Allanore Y . Primary myocardial

involvement in systemic sclerosis . Rheuma-

tology (Oxford) 2006 ; 45 ( Suppl 4 ): iv14 – iv17 .

105 . Champion HC . The heart in scleroderma .

Rheum Dis Clin North Am 2008 ; 34 ( 1 ): 181 –

190, viii .

106 . Tzelepis GE , Kelekis NL , Plastiras

SC ,

et al . Pattern and distribution of myocar-

dial ﬁ brosis in systemic sclerosis: a de-

layed enhanced magnetic resonance imag-

ing study . Arthritis Rheum 2007 ; 56 ( 11 ):

3827 – 3836 .

3-Tesla cardiac magnetic resonance imaging in primary dilated cardiomyopathy

Article

Full-text available

Apr 2024

Background Cardiac magnetic resonance imaging (CMR) is an excellent non-invasive imaging tool in the assessment of patients with dilated cardiomyopathy (DCM). Few studies have analysed the findings in primary (idiopathic) DCM. Objectives To study the CMR features in primary DCM. Methods We conducted a descriptive observational study on 20 adult patients with suspected or confirmed primary DCM. Each patient underwent a dedicated 3-Tesla CMR scan, and the findings were evaluated. Results Seventeen patients had systolic dysfunction with a reduced ejection fraction and elevated end-diastolic volume, 19 patients had contractile dysfunction in the form of global left ventricular hypokinesia, 13 patients showed no abnormal delayed contrast enhancement with gadolinium administration, and 7 patients showed abnormal late gadolinium enhancement patterns. Conclusion In patients with primary DCM, CMR is a powerful diagnostic tool that can definitively establish the diagnosis, assess the severity of the disease, predict the risk of future adverse cardiovascular outcomes, check for complications, and assist in future follow-ups. Study synopsis What the study adds. Cardiac magnetic resonance imaging (CMR) is an excellent non-invasive imaging tool in the assessment of patients with primary dilated cardiomyopathy (DCM). Findings include global ventricular enlargement, systolic dysfunction (ejection fraction <40%), and elevated end-diastolic (≥140 mL) and end-systolic volumes. Global abnormal wall contractility is often seen. In DCM there is either no abnormal gadolinium enhancement or curvilinear mid-myocardial or subepicardial late gadolinium enhancement, unrelated to a coronary artery distribution. Implications of the findings. In patients with primary DCM, CMR provides powerful diagnostic and prognostic information. Enhanced awareness and understanding of this relatively uncommon condition among clinicians and radiologists would be of benefit in patient management and treatment.

Role of cardiac magnetic resonance imaging in the diagnosis of nonischemic cardiomyopathies

Article

Full-text available

Dec 2022

In recent decades, cardiac magnetic resonance imaging (CMR) has been established as a valuable tool in the diagnosis of patients with or at risk of heart failure. With its ability to characterize tissue changes in the myocardium, CMR can provide detailed and clinically useful information about the type and severity of cardiac damage. The method is not only important for differentiation of ischemic from non-ischemic cardiomyopathy, but also contributes to the correct diagnosis of non-ischemic cardiomyopathy subtypes. It is also widely accepted as a reference standard for the quantification of myocardial mass, volumes and ejection fraction. The purpose of this article is to review the role of CMR in the diagnosis and treatment of non-ischemic cardiomyopathies.

Myocardial Tissue Characterization by Cardiac Magnetic Resonance: A Primer for the Clinician

Article

Full-text available

Jan 2023

Imaging plays an important role in the diagnosis, management, and prognosis of cardiac conditions. Over the last three decades, cardiac magnetic resonance (CMR) has established itself as a promising imaging tool in the assessment of patients with various cardiac ailments. CMR is now being considered as a one-stop-shop diagnostic test because of its ability to comprehensively assess the heart. The greatest strength of CMR is its ability to characterize the myocardium noninvasively aiding in the diagnosis of patients. Despite its growing use worldwide, the adoption of the modality in India has been relatively slow. One major reason for this is the lack of expertise and resources to perform CMR. Another reason for the slow adoption of CMR in India is the limited understanding of practicing clinicians on how the modality works and what the various clinical applications of CMR are. In this review, we aimed to address these shortcomings by outlining some basics of CMR and its utility in day-to-day practice with a special focus on myocardial tissue characterization.

Diagnostic value of cardiothoracic ratio in patients with non-ischaemic cardiomyopathy: comparison to cardiovascular magnetic resonance

Article

Jan 2024
Curr Probl Diagn Radiol

Purpose To determine the reliability of the cardiothoracic ratio (CTR) as a simple method to assess the cardiac size and function in patients with non-ischemic cardiomyopathy (NICM). Methods In a sample of 91 patients (66 patients with diagnosed non-ischemic cardiomyopathy and 25 controls) we calculated the CTR on a posteroanterior chest radiograph and ventricular and atrial size based on accepted CMR values. Left and right ventricular ejection fraction was also calculated. The CTR and cardiac chamber size were compared between patients with NICM and healthy individuals. The distinction between normal and increased cardiac chamber size was made using published normal CMR reference values stratified by age and gender. Results CTR values were higher in the NICM group (50.7±5.5% Vs. 45.3±4.7%, p<0.001). Likewise, LVEDVi, LV indexed mass, LA indexed volume, LA indexed area, and RA indexed area were higher, and LVEF and RVEF were lower in patients with non-ischemic cardiomyopathy (p<0.05). In patients with non-ischemic cardiomyopathy, the greatest correlation between CTR and CMR values was with LVEDVi (ρ=0.4, p<0.001), LA indexed volume (ρ=0.5, p<0.001), LA indexed area (ρ=0.5, p<0.001) and RA indexed area (ρ=0.4, p<0.001). However, the correlation strength was only moderate. Conclusion Despite patients with NICM had higher CTR values than the control group, a substantial proportion of these patients showed normal CTRs (<50%). This fact limits the usefulness of CTR to reliably predict NICM. Correlation between CTR and heart chamber dilation on CMR was only weak to moderate.

Cardiac MRI: State of the Art

Article

Apr 2023
RADIOLOGY

Cardiac MRI plays an important role in the evaluation of cardiovascular diseases (CVDs), including ischemic heart disease, cardiomyopathy, valvular disease, congenital disease, pericardial disease, and masses. Large multicenter trials have shown the positive impact of MRI-based management on outcomes in several CVDs. These results have made MRI an indispensable technique in the evaluation of these diseases, and cardiac MRI has an important role in multisociety guidelines. MRI is the reference standard for quantification of ventricular volumes and function. Flow imaging enables accurate quantification of flow and velocities through valves, shunts, and surgical conduits or baffles. Late gadolinium enhancement and parametric mapping techniques enable tissue characterization and yield prognostic information. In the past decade, cardiac MRI technology has seen rapid advances in both hardware and sequences. Multiple novel sequences, such as parametric mapping and four-dimensional flow, are increasingly being incorporated into routine clinical practice. Acceleration strategies have matured, allowing faster acquisition of cardiac MRI sequences in patients with arrhythmia and poor breath holding. Challenges of cardiac MRI at high-field-strength magnets and in patients with indwelling cardiac devices or severe renal dysfunction have been mitigated. Artificial intelligence techniques are decreasing the complexity of MRI acquisition and postprocessing. This article reviews the current state of the art and emerging techniques in cardiac MRI.

Cardiomyopathy

Chapter

Nov 2014

PATTERN OF MYOCARDIAL FIBROSIS IN ISCHEMIC AND NON ISCHEMIC CARDIOMYOPATHY

Article

Full-text available

Jul 2021

Background: The cardiomyopathies are defined as “heterogenous group of diseases of the myocardium associated with mechanical or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to variety of causes that frequently are genetic. Methods: The cross sectional hospital based study was conducted in the Department of Radiodiagnosis in patients with heart failure with LVEF (Left Ventricular Ejection Fraction) of <45% without RWM (Regional Wall Motion) abnormality on echocardiography evaluated in department of cardiology at IGMC, Shimla over a period of one year. Demographics: Demographics Overall Group Male Female Mean Age (year ) ± SD 53.86 ± 16.34 54.45 ± 19.41 53.94 ± 16.36 Gender (n) 38 17 (44.73%) 21(55.26%) Results: Myocardial fibrosis was seen in 4 (100%) patients of ischemic cardiomyopathy and 3 (18.75%) patients of non ischemic cardiomyopathy with significant P value of 0.01 and odd ratio of 1.67. The subendocardial myocardial fibrosis was seen in 3 (75%) patients of ischemic cardiomyopathy while it was not seen in non ischemic cardiomyopathy with significant P value of < 0.01 and odd ratio of 3.59. Conclusion: Cardiac MRI has important role in dilated cardiomyopathies for distinguishing ischemic and non ischemic types as well as in evaluation of various etiological types. Keywords: MRI, Myocardial, Cardiomyopathies

Radiologic Manifestations of Pulmonary and Cardiovascular Complications of COVID-19: A Narrative Review

Article

Jun 2021

Elliott et al. classification of cardiomyopathies

Chapter

Nov 2014

Idiopathic dilated cardiomyopathy

Chapter

Nov 2014

Natural history and familial characteristics of isolated left ventricular non-compaction

Article

Full-text available

Jul 2008

The Additional Value of Gadolinium-Enhanced MRI to Standard Assessment for Cardiac Involvement in Patients With Pulmonary Sarcoidosis

Article

Full-text available

Jan 2005
CHEST

Jan P Smedema

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D): proposed modification of the task force criteria

Article

Jan 2010

Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium-enhanced cardiovascular magnetic resonance

Article

Nov 2003

Left Ventricular Non-Compaction

Article

Jul 2005

We aimed to test the diagnostic accuracy of cardiovascular magnetic resonance (CMR) imaging in distinguishing pathological left ventricular non-compaction (LVNC) from lesser degrees of trabecular layering seen in healthy volunteers and, in those with cardiomyopathies and concentric left ventricular hypertrophy, potential differential diagnoses. We hypothesized that pathological trabeculation could be distinguished by determining the ratio of non-compacted to compacted myocardium (NC/C ratio). Left ventricular non-compaction is characterized by a non-compacted myocardial layer in the left ventricle. Cardiovascular magnetic resonance images this layer with unprecedented quality, particularly in the ventricular apex, where echocardiography has inherent difficulties. We analyzed magnetic resonance cine images, using the 17-segment model in 45 healthy volunteers, 25 athletes, 39 patients with hypertrophic cardiomyopathy and 14 with dilated cardiomyopathy, 17 with hypertensive heart disease, and 30 with aortic stenosis, as well as images from 7 patients previously diagnosed with LVNC whose diagnoses were supported by additional features. Areas of non-compaction were common and occurred more frequently in all groups studied in apical and lateral, rather than in basal or septal, segments. A NC/C ratio of >2.3 in diastole distinguished pathological non-compaction, with values for sensitivity, specificity, and positive and negative predictions of 86%, 99%, 75%, and 99%, respectively. Left ventricular non-compaction is diagnosed accurately with CMR using the NC/C ratio in diastole.

Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Article

Jan 2005
CIRCULATION

Alicia Maceira

Background: Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium. Methods and results: Late gadolinium enhancement CMR was performed in 30 patients with cardiac amyloidosis. In 22 of these, myocardial gadolinium kinetics with T1 mapping was compared with that in 16 hypertensive controls. One patient had CMR and autopsy only. Subendocardial T1 in amyloid patients was shorter than in controls (at 4 minutes: 427+/-73 versus 579+/-75 ms; P<0.01), was shorter than subepicardium T1 for the first 8 minutes (P< or =0.01), and was correlated with markers of increased myocardial amyloid load, as follows: left ventricular (LV) mass (r=-0.51, P=0.013); wall thickness (r=-0.54 to -0.63, P<0.04); interatrial septal thickness (r=-0.52, P=0.001); and diastolic function (r=-0.42, P=0.025). Global subendocardial late gadolinium enhancement was found in 20 amyloid patients (69%); these patients had greater LV mass (126+/-30 versus 93+/-25 g/m2; P=0.009) than unenhanced patients. Histological quantification showed substantial interstitial expansion with amyloid (30.5%) but only minor fibrosis (1.3%). Amyloid was dominantly subendocardial (42%) compared with midwall (29%) and subepicardium (18%). There was 97% concordance in diagnosis of cardiac amyloid by combining the presence of late gadolinium enhancement and an optimized T1 threshold (191 ms at 4 minutes) between myocardium and blood. Conclusions: In cardiac amyloidosis, CMR shows a characteristic pattern of global subendocardial late enhancement coupled with abnormal myocardial and blood-pool gadolinium kinetics. The findings agree with the transmural histological distribution of amyloid protein and the cardiac amyloid load and may prove to have value in diagnosis and treatment follow-up.

Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy

Article

Aug 2005
CIRCULATION

Carsten Rickers

Background— Two-dimensional echocardiography is currently the standard test for the clinical diagnosis of hypertrophic cardiomyopathy (HCM). The present study was undertaken to determine whether cardiac MRI (CMR) affords greater accuracy than echocardiography in establishing the diagnosis and assessing the magnitude of left ventricular (LV) hypertrophy in HCM. Methods and Results— Forty-eight patients (age 34±16 years) suspected of having HCM (or with a confirmed diagnosis) were imaged by both echocardiography and CMR to assess LV wall thickness in 8 anatomic segments (total n=384 segments) and compared in a blinded fashion. Maximum LV thickness was similar by echocardiography (21.7±9.1 mm) and CMR (22.5±9.6 mm; P=0.21). However, in 3 (6%) of the 48 patients, echocardiography did not demonstrate LV hypertrophy, and CMR identified otherwise undetected areas of wall thickening in the anterolateral LV free wall (17 to 20 mm), which resulted in a new diagnosis of HCM. In the overall study group, compared with CMR, echocardiography also underestimated the magnitude of hypertrophy in the basal anterolateral free wall (by 20±6%; P=0.001), as well as the presence of extreme LV wall thickness (≥30 mm) in 10% of patients (P<0.05). Conclusions— CMR is capable of identifying regions of LV hypertrophy not readily recognized by echocardiography and was solely responsible for diagnosis of the HCM phenotype in an important minority of patients. CMR enhances the assessment of LV hypertrophy, particularly in the anterolateral LV free wall, and represents a powerful supplemental imaging test with distinct diagnostic advantages for selected HCM patients.

Standardized Myocardial Segmentation and Nomenclature for Tomographic Imaging of the Heart: A Statement for Healthcare Professionals From the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association

Article

Mar 2002
CIRCULATION

Manuel D. Cerqueira

Nuclear cardiology, echocardiography, cardiovascular magnetic resonance (CMR), cardiac computed tomography (CT), positron emission computed tomography (PET), and coronary angiography are imaging modalities that have been used to measure myocardial perfusion, left ventricular function, and coronary anatomy for clinical management and research. Although there are technical differences between these modalities, all of them image the myocardium and the adjacent cavity. However, the orientation of the heart, angle selection for cardiac planes, number of segments, slice display and thickness, nomenclature for segments, and assignment of segments to coronary arterial territories have evolved independently within each field. This evolution has been based on the inherent strengths and weaknesses of the technique and the practical clinical application of these modalities as they are used for patient management. This independent evolution has resulted in a lack of standardization and has made accurate intra- and cross-modality comparisons for clinical patient management and research very difficult, if not, at times, impossible. Attempts to standardize these options for all cardiac imaging modalities should be based on the sound principles that have evolved from cardiac anatomy and clinical needs.1–3⇓⇓ Selection of standardized methods must be based on the following criteria: An earlier special report from the American Heart Association, American College of Cardiology, and Society of Nuclear Medicine4 defined standards for plane selection and display orientation for serial …

American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy

Article

Nov 2003
J AM COLL CARDIOL

Preamble......1688 Introduction......1688 General Considerations and Perspectives......1688 Nomenclature, Definitions, and Clinical Diagnosis......1689 Obstruction to LV Outflow......1689 Genetics and Molecular Diagnosis......1690 General Considerations for Natural History and Clinical Course

Evaluation of the Accuracy of Gadolinium-enhanced Cardiovascular Magnetic Resonance in the Diagnosis of Cardiac Sarcoidosis

Article

Sep 2005

OBJECTIVES: This study analyzed the accuracy of gadolinium-enhanced cardiovascular magnetic resonance (CMR) for the diagnosis of cardiac sarcoidosis (CS). BACKGROUND: The diagnosis of CS was made according to the guidelines of the Japanese Ministry of Health and Welfare (1993); CMR has not been incorporated into the guidelines, and the diagnostic accuracy of CMR for the diagnosis of CS has not yet been evaluated. METHODS: We performed an analysis of 12-lead electrocardiograms (ECGs), 24-h ambulatory ECGs, echocardiograms, thallium scintigrams, and gadolinium-enhanced CMR studies in 58 biopsy-proven pulmonary sarcoidosis patients assessed for CS. The diagnostic accuracy of CMR for CS was determined using modified Japanese guidelines as the gold standard. RESULTS: The diagnosis of CS was made in 12 of 58 patients (21%); CMR revealed late gadolinium enhancement (LGE), mostly involving basal and lateral segments (73%), in 19 patients. In 8 of the 19 patients, scintigraphy was normal, while patchy LGE was present. The sensitivity and specificity of CMR were 100% (95% confidence interval, 78% to 100%) and 78% (95% confidence interval, 64% to 89%), and the positive and negative predictive values were 55% and 100%, respectively, with an overall accuracy of 83%. CONCLUSIONS: In patients with sarcoidosis, CMR is a useful diagnostic tool to determine cardiac involvement. New diagnostic guidelines should include CMR.

Cardiac MR Imaging of Nonischemic Cardiomyopathies: Imaging Protocols and Spectra of Appearances

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