1. Cardiac Magnetic Resonance in Hypertrophic Cardiomyopathy
Andrew C.Y. To, Ashwat Dhillon, and Milind Y. Desai
J. Am. Coll. Cardiol. Img. 2011;4;1123-1137
doi:10.1016/j.jcmg.2011.06.022
This information is current as of November 21, 2011
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://imaging.onlinejacc.org/cgi/content/full/4/10/1123
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4. JACC: CARDIOVASCULAR IMAGING, VOL. 4, NO. 10, 2011 To et al. 1125
OCTOBER 2011:1123–37 CMR in HCM
the details and limitations of the typical CMR atomical assessment. TTE and TEE are the current
study for HCM. standards in assessing LVOT anatomy and flow
In this review article, we discuss the role of profile. The main advantage of CMR is in iden-
CMR in the diagnosis, treatment, and prognosis tifying the anatomy of the septal-systolic anterior
of HCM, with a focus on the complementary motion contact and subvalvular apparatus. Iso-
value of CMR in relation to standard imaging lated or concomitant mid-ventricular obstruction
modalities, and we examine some of the emerging related to mid-ventricular hypertrophy is also
roles of CMR. easily demonstrated.
The CMR studies have illustrated the contribu-
tion of abnormal mitral subvalvular morphology in
CMR and Diagnosis LVOT obstruction (20,21) (Fig. 6, Online Video
2). Compared with control subjects, HCM patients
The phenotypic heterogeneity of HCM is well- have a higher incidence of papillary muscle anom-
recognized. This is further complicated because not alies such as bifid or multiple accessory papillary
all patients with LVH have HCM, whereas HCM- muscles, as well as anteroapical papillary muscle
like pathophysiology with dynamic LVOT obstruc- displacement that encroaches into the
tion can be observed without LVH, in a subgroup LVOT during systole (19,22). Figure 7 ABBREVIATIONS
of patients with mitral valve and/or papillary muscle schematically illustrates the common pap- AND ACRONYMS
abnormalities. Figure 1 summarizes the diagnostic illary muscle anatomical variations that
challenges faced by clinicians in both established contribute to LVOT obstruction. During
CMR cardiac magnetic
resonance
and suspected HCM. Figure 2 highlights the areas CMR acquisition, careful attention is paid ECG electrocardiography
where CMR potentially has incremental utility. on the short-axis cine images, with addi-
Although a more comprehensive algorithm detail- tional long-axis cine images specifically
HCM hypertrophic
cardiomyopathy
ing the step-by-step diagnostic approach in HCM planned to demonstrate subvalvular anat- ICD implantable cardioverter
has been detailed elsewhere (17), a simplified ap- omy. This is especially important in pa- defibrillator
proach to the differential diagnosis of HCM has tients with dynamic LVOT obstruction LGE late gadolinium
been outlined in Figure 3. CMR enables the precise without classic asymmetric septal hyper- enhancement
characterization of subtle disease phenotypic varia- trophy. A 3-dimensional dataset of the LV left ventricle/ventricular
tions (Figs. 4, 5, 6, and 7, Online Videos 1, 2, and LV with high spatial resolution is ob- LVH left ventricular
3), especially important for characterizing LVOT, tained with a respiratory navigator ECG- hypertrophy
papillary muscle, subvalvular anatomy, and diagnos- gated whole-heart sequence that allows LVOT left ventricular outflow
ing of atypical HCM. High image quality and offline multiplanar reconstruction of pap- tract
tissue characterization accurately identify the vari- illary and subvalvular anatomy. RV right ventricle/ventricular
ous conditions that mimic the morphological ap- Although CMR assessment of the SCD sudden cardiac death
pearance of HCM (Figs. 8 and 9, Online Videos 4 LVOT is primarily anatomical, LVOT ac- TEE transesophageal
and 5). Reproducible volume and mass quantification celeration and flow turbulence can be diag- echocardiography
might also identify at-risk individuals with a family nosed as systolic signal void in flow-sensitive TTE transthoracic
history of HCM and can be used to screen for gradient echo sequences, and LVOT gradi-
echocardiography
pre-clinical disease. ent can be quantified with phase contrast
Disease characterization: LVOT, papillary muscle, and
flow-sensitive sequences. However, this is often tech-
subvalvular anatomy. Resting or provocable LVOT
nically challenging in HCM for a variety of reasons.
obstruction is present in 70% of cases and is an
Proper alignment of the imaging plane to obtain the
important manifestation of HCM (18). It relates to
highest flow velocities can be time consuming and
the complex anatomical relationships between the
septum, LVOT, mitral valve, and papillary muscles. prone to errors. Intravoxel dephasing and signal loss
In the majority of HCM patients, septal hypertro- due to phase offset errors also make the accurate
phy leads directly to LVOT obstruction (Fig. 4, quantification of turbulent flow difficult. Imaging with
Online Video 1). However, some present with hyper- provocation and during exercise is also difficult with
trophy without obstruction, whereas others pres- CMR. New CMR sequences under development
ent with dynamic LVOT obstruction and mini- might allow the routine 3-dimensional acquisition of
mal septal hypertrophy. The latter is likely due to the flow pattern and velocities not limited by imaging
a variety of papillary muscle and subvalvular planes (23), real-time velocity encoding (24), as well as
abnormalities (19) (Fig. 5, Online Video 2). Such accurate measurement of turbulent jet velocities (25).
complexity highlights the importance of accurate an- Until then, echocardiography remains the “gold stan-
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CMR in HCM OCTOBER 2011:1123–37
Table 1. Typical Dedicated CMR Study for HCM: Potential Advantages and Limitations
Potential Advantages Over Limitations of CMR
Typical Sequences Technical Details Information Obtained Echocardiography Techniques
Bright blood cine image Balanced SSFP Septal thickness Image quality superior to Availability and portability of
echocardiography echocardiography is unlikely
to be matched by CMR
Optionally, 3D SSFP Relationship between the No limitation of imaging window 3D cine sequences are currently
septum, mitral valve, and and imaging plane limited by acquisition time,
subvalvular apparatus in the inferior spatial and temporal
LVOT obstruction resolution
Global and regional Quantification of ventricular Functional information on
ventricular function volumes, function, and mass dynamic LVOT obstruction
with excellent reproducibility might not be easily obtained
Ventricular mass Compared with transesophageal
echocardiography, CMR is
noninvasive
LGE images Phase-sensitive Extent and location of Tissue characterization for Limited role in patients with
inversion recovery myocardial fibrosis myocardial fibrosis is chronic renal failure due to
gradient echo unique to CMR concern over nephrogenic
sequence systemic fibrosis
Quantification of myocardial
fibrosis is time consuming
Detection of diffuse myocardial
fibrosis remains challenging
Selection of wrong nulling time
on LGE might make
measurement of myocardial
fibrosis inaccurate
3D SSFP whole Respiratory navigator Papillary muscle anatomy Localization of papillary muscle 3D information with a high
heart dataset gated ECG gated 3D number, extent, proximal and spatial resolution is not easily
SSFP sequences distal attachments obtainable
Coronary artery anatomy Exclusion of coronary anomalies as
alternative cause of cardiac
arrhythmia and SCD
Tagged cine images SPAMM sequence Regional wall deformation Accurate characterization of regional Data analysis to obtain strain
deformation: strain and strain rate and strain rate remains time
consuming
Limited clinical utility
Flow quantification Velocity-encoded cine Aortic flow velocities, Quantification of flow velocities Accuracy of flow measurements
sequences sequences profile, and volume and volume in HCM has not been
validated
LVOT flow velocities and profile Quantification of mitral regurgitation
Mitral regurgitant volumes and
fractions
Perfusion images—rest 90° saturation recovery Myocardial perfusion Information on myocardial perfusion Data analysis to quantify
pre-pulse followed is easily obtained with CMR, at myocardial perfusion remains
by gradient echo the time of contrast injection for the realm of advanced
readout sequences LGE assessment research laboratory
Clinical implications of
abnormal findings not
well-established
3D 3-dimensional; CMR cardiac magnetic resonance; ECG electrocardiography; HCM hypertrophic cardiomyopathy; LGE late gadolinium enhancement; LVOT left ventricular
outflow tract; SCD sudden cardiac death; SPAMM spatial modulation of magnetization; SSFP steady state free precession.
dard” for flow quantification of LVOT obstruction in forward aortic flow derived from the velocity-
HCM. encoded phase contrast sequence, from stroke
CMR assesses mitral regurgitation with diverse volume derived from LV volume measurements
techniques. Gauging severity on the basis of (26,27). Although CMR also demonstrates mi-
turbulence-related signal void in various cine tral leaflet abnormalities, echocardiography re-
sequences is fraught with errors, because it is mains the test of choice because of superior
highly dependent on pulse sequence parameters. temporal resolution and various Doppler tech-
Most commonly, visualizing the regurgitant jet niques for hemodynamic information.
on flow-sensitive gradient echo sequences is com- Disease characterization: atypical forms of HCM. In
plemented with quantification, by subtracting the past, it was presumed that HCM is synonymous
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OCTOBER 2011:1123–37 CMR in HCM
Genotypic Delayed disease expression in gene carriers,
Genotype
Heterogeneity hence issues with screening
Classic LVOT obstruction
may occure without
septal hypertrophy
Phenotypic
Heterogeneity Phenotype
HYPERTROPHY LVOT OBSTRUCTION
Hypertrophic
Obstructive
Atypical Cardiomyopathy
distribution Obstructive
of hypertrophy Non-obstructive cardiomyopathy without
hypertrophic hypertrophy
cardiomyopathy
Concurrent mitral
valve abnormalities
Other conditions may
mimic the hypertrophic Concurrent papillary
cardiomyopathy muscle abnormalities
Figure 1. Diagnostic Challenges Faced by Clinicians in Suspected and Established HCM
This figure demonstrates the potential difficulties in diagnosis of hypertrophic cardiomyopathy (HCM) due to phenotypic and genotypic
heterogeneity. Patients within the same family might have different phenotypic expressions, ranging from gross hypertrophy with severe
left ventricular outflow tract (LVOT) obstruction to minimal hypertrophy and no LVOT obstruction.
with asymmetric septal hypertrophy, and hence a spectrum to focal segmental hypertrophy on the other
septal to posterior wall ratio 1.3 is diagnostic of end. The focal hypertrophy variant sometimes in-
HCM (28,29). Subsequent studies, including those volves only 1 to 2 myocardial segments, often with a
with CMR, showed that atypical cases of HCM are noncontiguous pattern of hypertrophy where hyper-
more common than previously thought (30,31). These trophied segments are separated by regions of normal
range from diffuse global hypertrophy on 1 end of the thickness (30). Normal LV mass does not exclude
Potential Utility of CMR Evaluation of HCM
Diagnosis Treatment Prognosis
If diagnosis of If diagnosis If there is a Pre- Post- Risk
HCM is of HCM is family history procedural procedural prediction
established uncertain of HCM planning planning
Disease Differential Screening for LV septal
characterization and diagnosis preclinical
phenotypic expression Myectomy dimension
•Hypertensive disease LVOT
Atypical forms of HCM heart disease anatomy
•Asymptomatic
•Apical HCM •Aortic stenosis positive gene LV mass
•Apical aneurysm •Athlete’s heart carrier
Papillary
•Atypical non-contiguous •Asymmetric muscle
pattern of hypertrophy •Asymptomatic
septal suspected remodeling
•Right ventricular hypertrophy gene carrier LV function
involvement of the elderly Mitral valve
•Non-compaction and papillary
LVOT obstruction,
Mitral valve muscle
mitral valve and •Infiltrative Late gadolinium
papillary muscle heart disease repair anatomy
enhancement
anatomy
Figure 2. Potential Role of CMR in Management of HCM
This figure explains the potential utility of cardiac magnetic resonance (CMR) in the diagnosis and management of HCM. It has a
potential role in establishing the diagnosis, pre-procedural planning, and prognostication. LV left ventricular; other abbreviations
as in Figure 1.
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CMR in HCM OCTOBER 2011:1123–37
Figure 3. Simplified Approach to the Differential Diagnosis of HCM
This figure is a simplified diagnostic approach in a patient with increased LV thickness. Please note that for such an approach to be suc-
cessful, multimodality imaging is necessary. Abbreviations as in Figures 1 and 2.
HCM in these patients. Such a focal noncontiguous HYPERTENSIVEHEARTDISEASEANDAORTICSTENOSIS.
pattern of hypertrophy is not usually seen in secondary Hypertensive heart disease and aortic stenosis both
forms of hypertrophy (e.g., hypertension). In 12% of present with concentric rather than asymmetrical
HCM patients, focal segmental LV hypertrophy is LVH. HCM and hypertensive heart disease occa-
limited to the anterolateral free wall, posterior septum, sionally might be difficult to differentiate, but in
or apex (30,32). These areas are technically challeng- general, LV wall thickness of hypertensive heart
ing for TTE, due to imaging window limitation, and disease is 15 to 16 mm. Specific studies compar-
in 1 study, the diagnosis of HCM was missed in 6% ing hypertensive heart disease and HCM with
of patients by echocardiography (32). CMR are sparse, although with improved image
Apical HCM (Fig. 6, Online Video 3) with quality, CMR is more sensitive in detecting differ-
predominantly LV apical hypertrophy is commonly ences in segmental wall thickness. Interestingly,
missed on TTE, because of limited acoustic windows although myocardial fibrosis on LGE has tradition-
and foreshortening, and CMR has incremental utility ally been considered rare in hypertensive heart
here (33). Similarly, apical aneurysm can be missed on disease and aortic stenosis, a recent study demon-
noncontrast TTE in 40% of cases and is best visual- strated patchy LGE in more than 50% of hyper-
ized on CMR (34). Apical aneurysms present as tensive heart disease and aortic stenosis patients
dyskinesis or akinesis with a thin rim of myocardium, with significant LVH (36). Another report also
often with transmural scarring on LGE, and are suggested a potential prognostic role for LGE in
associated with adverse outcomes with an annual aortic stenosis (37). As such, LGE itself might not
event rate of 11%. In addition, recent reports found be specific for HCM, and its prognostic utility in
that HCM patients often have significant right ven- other disorders needs to be studied further. In
tricular (RV) involvement with increased RV wall addition, in a small group of patients with concom-
thickness and mass compared with control subjects itant valvular aortic stenosis and LVOT obstruction
(35). Assessment of RV by CMR is superior to from asymmetric septal hypertrophy, CMR can
echocardiography. identify the site of jet turbulence and distinguish the
relative contributions of the 2 disease processes.
Differential diagnosis. Accurate diagnosis of HCM
is important, because of the significant lifestyle ATHLETE’S HEART. Athlete’s heart is character-
altering and familial implications. ized by a mildly enlarged LV cavity, symmetric
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OCTOBER 2011:1123–37 CMR in HCM
Figure 4. Patient With “Typical” HCM and LVOT Obstruction
(A) Patient with “typical” HCM with marked basal septal hypertrophy on CMR and (B) LVOT obstruction (arrow) on Doppler echocardiog-
raphy after amyl nitrite. Patient with “garden-variety” hypertrophic obstructive cardiomyopathy with severe basal septal hypertrophy and
LVOT obstruction. Also note the myocardial fibrosis. Late gadolinium enhancement in long-axis (C) and short-axis (D) views showed myo-
cardial fibrosis (arrows). See Online Video 1. Abbreviations as in Figures 1 and 2.
thickening of the LV wall—typically 15 mm— nonaffected segments as well as diagnoses the com-
and normal diastolic function on Doppler echo- monly associated LV thrombus.
cardiography. CMR complements TTE in this
INFILTRATIVE HEART DISEASES. Infiltrative heart
condition, because it accurately measures LV
diseases mimic HCM with LVH and its functional
volumes, mass, and function, with high reproduc-
consequences. CMR plays an important role in
ibility (9,38). Researchers used wall thickness
excluding these conditions.
indexed to end-diastolic ventricular volume to
Fabry’s disease. Fabry’s disease is an X-linked reces-
distinguish athlete’s heart from HCM (39). De-
sive glycolipid storage disease with deficient alpha-
spite this, such differentiation remains difficult,
galactosidase activity. The resulting phenotype is of
and some subjects might have to undergo a period
concentric hypertrophy, with LGE found in 50% of
of deconditioning to document reverse remodel-
patients, typically in the basal inferolateral segment in
ing as a definitive proof of athlete’s heart (40).
a mid-myocardial distribution (41) (Fig. 8, Online
NONCOMPACTION. Noncompaction is character- Video 4).
ized by prominent LV trabeculations, and differenti- Hypereosinophilic syndrome. Hypereosinophilic syn-
ation of compacted and noncompacted layers is often drome presents with apical fibrosis and mural
difficult in echocardiography, especially without con- thrombus, frequently leading to apical cavity oblit-
trast. CMR is ideal for delineating compacted and eration, and therefore can sometimes mimic apical
noncompacted layers. An end-diastolic ratio between HCM on TTE (42). Areas of increased subendo-
noncompacted and compacted layers of more than cardial signal intensity are often observed.
2.4:1.0 is a proposed imaging criterion for noncom- Sarcoidosis. Sarcoidosis usually presents with a re-
paction. CMR also precisely delineates the character- strictive cardiomyopathy with generalized LV thick-
istic abrupt transition zone between affected and ening, but asymmetric basal septal involvement can
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CMR in HCM OCTOBER 2011:1123–37
Figure 5. Patient With “Obstructive Cardiomyopathy” With Minimal LVH
The main pathology is the abnormal hypermobile bifid papillary muscle (arrows, A and B) resulting in systolic anterior motion of
the anterior mitral valve leaflet (arrow, C), seen on CMR (diastole [A] and systole [B]) and echocardiography (C). This causes severe
post exercise LVOT obstruction (D). See Online Videos 1 and 2. LVH left ventricular hypertrophy; other abbreviations as in
Figures 1 and 2.
mimic HCM (43). The LGE pattern is variable, most penetrance, and delayed disease presentation some-
commonly affecting the basal and lateral segments. times until adulthood also make it challenging to
Amyloidosis. Amyloidosis presents with diffuse LV screen for suspected carriers and detect preclinical disease
wall thickening and diffuse LGE associated with a in definite carriers. Current strategy involves a combina-
characteristic shortening of myocardial nulling time tion of clinical assessment, ECG, and TTE at 12- to
on inversion recovery sequences (44,45) (Fig. 9, 18-month intervals from age 12 to adulthood, although
Online Video 5). negative clinical and imaging tests cannot fully exclude
Screening. Despite advances in gene testing in HCM, the risks of future disease development (46).
mutations are only identified in 60% of index HCM Although a large prospective study of HCM
cases (3,4). Phenotypic heterogeneity, incomplete screening with CMR has not been performed, CMR
Figure 6. Apical HCM
Apical HCM with marked mid and apical hypertrophy on cine CMR (A). Patchy late gadolinium enhancement (arrow) is observed in the
hypertrophied apex on late gadolinium enhancement sequences (B). See Online Videos 1 and 3. Abbreviations as in Figures 1 and 2.
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OCTOBER 2011:1123–37 CMR in HCM
Figure 7. Schematic Diagram of the Common Variations in Papillary Muscle Anatomy in HCM
Schematic diagram of the common variations in papillary muscle anatomy in HCM (arrows). The left image represents the myocardium
during diastole, the right image represents systole. (A) Normal papillary muscle orientation; (B) bifid papillary muscles; (C) apical displace-
ment of the papillary muscles; (D) hypertrophied papillary muscles with mainly mid-cavity obstruction during systole; (E) abnormal
chordal attachment to the mid-portion of the mitral valve (MV); and (F) elongated anterior MV leaflet. See Online Video 2. Ao aorta;
LA left atrium; LV left ventricle; other abbreviation as in Figure 1.
might detect subtle abnormalities and/or serial LVH, as compared with control subjects (49). Parallel
changes that are otherwise not observed on echocar- to the research in strain imaging by echocardiography to
diography, enabling the detection of pre-clinical dis- detect subclinical contractile dysfunction in carriers
ease. In small studies, CMR detected abnormal wall (50,51), CMR myocardial tagging techniques have also
thickening in approximately 20% of asymptomatic been investigated; however, studies remain sparse (52).
gene carriers not appreciated by echocardiography.
Pre-hypertrophic crypts in the basal and mid infero- CMR and Treatment Strategies
septum have been suggested as a sign of a mutation
carrier (47,48). In a recent study, high levels of serum Symptomatic patients with obstructive HCM intrac-
C-terminal propeptide of type I procollagen were table to medical therapy can either undergo surgical
found in subjects with HCM-mutations without myectomy or alcohol septal ablation. CMR is an
Figure 8. Patient With Fabry’s Disease
Cine sequence in the 3-chamber view demonstrates the concentric hypertrophy (A), associated with the basal posterolateral segment
(arrow) late gadolinium enhancement (B). See Online Video 4.
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CMR in HCM OCTOBER 2011:1123–37
Figure 9. Patient With Cardiac Amyloidosis
Cine sequence in the 4-chamber view demonstrates diffuse concentric left ventricular hypertrophy (A). Late gadolinium enhancement
images showed extensive amyloid infiltration (arrows) with generalized gadolinium uptake, resulting in high signal intensity and a
reduced myocardial nulling time on inversion recovery sequences (B). See Online Video 5.
important adjunct in the pre-procedural planning correlate of LGE in HCM seems to be increased
for both procedures. We perform pre-operative myocardial collagen rather than myocardial dis-
CMR and perioperative TEE to precisely measure array, which is also observed on histological
the degree and extent of the anteroseptal and specimens. Increased myocardial collagen is pos-
inferoseptal hypertrophy as well as the relationship tulated to reflect microvascular ischemia and
of the septum with the anterior mitral valve leaflet, microscopic replacement fibrosis due to small
subvalvular apparatus, and papillary muscle mor- intramural coronary arteriole dysplasia (57,58).
phology. Accurate pre-operative anatomical assess- The latter finding correlates with LGE in myec-
ment of the subvalvular anatomy has led to the tomy specimens from patients who underwent
increasing recognition that septal myectomy might surgery for LVOT obstruction (58). An alterna-
need to be combined with mitral valve and chordae tive hypothesis for LGE in HCM suggested that
remodeling and/or papillary muscle reorientation to the causative sarcomeric gene mutations might
optimally relieve LVOT obstruction (53,54). lead to a phenotypic expression of increased
CMR is extensively used to assess the effective- myocardial connective tissue deposition (59).
ness of alcohol septal ablation (55,56). CMR after The prevalence of LGE is variable in different
surgical myectomy or alcohol septal ablation pro- cohorts. In those with manifest HCM, it varies
vides insights on the effect of the respective proce- between 40% and 80% (10 –14,60). The commonly
dures on the interventricular septum (55). Surgical found LGE pattern is patchy, multifoci mid-
myectomy predictably leads to a discrete resected myocardial fibrosis, especially in regions of hyper-
area in the anteroseptum, whereas alcohol septal trophy (Figs. 4B, 4D, and 6B). Other observed
ablation leads to a variable pattern of myocardial patterns include diffuse confluent transmural septal
scar, usually inferiorly in the basal septum with fibrosis and patchy septal fibrosis at RV insertion
extension to the RV side of the septum. The points.
improvement of LVOT obstruction is also more LGE correlates with LV wall thickening
variable after alcohol septal ablation. (10,61) and inversely correlates with LV ejection
fraction (61– 63). It also correlates with other
known clinical markers of SCD (64). The asso-
CMR and Prognosis ciation between LGE and the detection of ven-
tricular arrhythmia on Holter monitoring sug-
LGE. There is a growing body of published re- gests the potential pathophysiologic link between
ports on the role of LGE on CMR in HCM risk HCM, myocardial fibrosis, arrhythmia, and ulti-
stratification, but a large prospective study on mately SCD (10 –14,60). Recent longitudinal
how the data should be interpreted to alter studies suggest a strong association between LGE
management is still lacking. The histological and SCD (Table 2) (12–14). LGE shows prom-
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Table 2. Summary of the Recent Prognostic Studies on the Role of LGE in HCM
O’Hanlon et al. (13) Bruder et al. (14) Rubinshtein et al. (12)
N (% women) 217 (29) 243 (39) 424 (41)
Follow-up, yrs 3.1 3.0 3.6
Clinical (%)
NYHA functional class III/IV 14 8 53
Wall thickness 30 mm 6 4 7
History of syncope 16 6 16
History of sustained VT/VF 3 6 10
CMR
Prevalence of LGE (%) 63 61 56
Quantification of LGE FWHM 2 SD Qualitative manual tracing
Outcome
Primary endpoint: LGE vs. no LGE Primary combined endpoint (25% vs. 7%); HR 3.37 LGE is associated with all-cause SCD and appropriate ICD
Cardiovascular deaths (5.9% vs. 1.2%); HR 4.45 mortality (OR: 5.47) and discharge (3.4% vs.
cardiac mortality (OR: 8.01) 0.0%)
FWHM full-width at half maximum; HR hazard ratio; ICD implantable cardioverter defibrillator; NYHA New York heart association; OR odds ratio; VF ventricular fibrillation; VT
ventricular tachycardia; other abbreviations as in Table 1.
ise, but there is insufficient evidence for inserting With respect to LGE and prognosis, the relative
an ICD on the basis of LGE alone. Further importance of the severity, extent, and location of
studies should establish the role of LGE in LGE as well as whether there is a threshold effect
identifying high-risk patients from among those below which fibrosis does not impact on prognosis
who are currently classified as intermediate-risk is uncertain.
with clinical criteria and do not otherwise qualify Septal thickness and LV mass. The current guidelines
for ICD insertion. include LV thickness 30 mm on TTE as an
Certain aspects of LGE in HCM prognostica- important prognostic criterion (6). The improved
tion are technically challenging and worthy of accuracy of CMR in measuring LV thickness will
mention. Error in the appropriate myocardial null- likely refine this. In addition, CMR provides accu-
ing time might over- or underestimate true fibrosis rate and reproducible information on overall LV
burden. The use of phase sensitive inversion recov- mass. Investigators have studied the relative prog-
ery sequences has greatly improved this aspect (65). nostic value of LV wall thickness and mass by
Although LGE is assessed qualitatively in routine CMR. HCM patients typically have a “thickness-
clinical practice, LGE in relation to overall LV mass” mismatch because of the differing extent of
myocardial volume can be quantified with auto- hypertrophy in individual LV segments. It was
mated software. Various methods exist and most found that LV mass indexed to body surface area
commonly calculate the total areas of signal inten- above 2 SDs of a healthy control cohort is a
sity above a certain number of SDs (n 2 to 6) over sensitive but not specific predictor of outcome,
that of the mean signal intensity of nulled myo- whereas an LV wall thickness 30 mm is a more
cardium (61,66 – 68). These differences in meth- specific but less sensitive predictor (71). Future
odology translate into differences in the quanti- studies will clarify how best to use this information
fied area of LGE and potentially impact on the in management.
ability to generalize individual research studies.
The current assessment of myocardial fibrosis
contrasts areas of LGE with areas of presumed New Developments in CMR Imaging of HCM
“normal” nulled myocardium. Histological stud-
ies, however, suggest a global increase in myocar- Several new developments in CMR imaging of
dial fibrosis that current LGE imaging tech- HCM are worth discussing, but their clinical ap-
niques cannot detect. New techniques such as T1 plications remain undecided.
mapping (69) and equilibrium contrast CMR There has been increasing awareness of the
(70) might offer alternatives to quantify the importance of ventricular vascular interactions in
overall extent of myocardial fibrosis. various cardiac disorders. HCM patients were
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CMR in HCM OCTOBER 2011:1123–37
found to have a higher pulse wave velocity than myocardial segments and is inversely related to
matched control subjects, indicating increased severity (52,78). These findings are analogous to
aortic stiffness (72). This was independently as- strain measured by speckle tracking on echocar-
sociated with lower peak oxygen consumption on diography, where impaired longitudinal strain
cardiopulmonary exercise testing (73). Whole- was shown to correlate with fibrosis severity (79).
heart CMR sequences also provided insight that
HCM patients have a steep angle between the
aortic root and the LV long axis, compared with Conclusions
control subjects. The acuteness of this LV-aortic
root angle correlates with age and the observed HCM is a heterogeneous disease with complex mor-
LVOT gradient (74). These early findings high- phological expression that requires accurate disease
light the potential impact HCM has on the aortic characterization for optimal therapeutic planning and
vasculature and the usefulness of CMR in inves- risk-stratification. CMR has emerged as a useful
tigating this relationship. adjunct for these purposes. With the increasing incor-
CMR perfusion studies in HCM investigated poration of multimodality imaging in the clinical
the role of microvascular dysfunction in intramu- assessment of HCM, our understanding of the signif-
ral coronary arteriole dysplasia and subsequently icance of subtle morphological differences will con-
myocardial fibrosis as well as in blunting myocar- tinue to grow, and further research will define new
dial blood flow during vasodilator stress, which prognostic markers and improve current treatment
has been observed in HCM, especially subendo- strategies.
cardially (75). Furthermore, CMR spectroscopy
with 31-phosphorus demonstrated an altered Acknowledgments
myocardial energy metabolic profile in HCM that The authors would like to acknowledge Marion
correlated with the severity of LGE (76). With Tomasko for her graphical support and Kathryn
CMR spectroscopy, perhexiline, a modulator of Brock for her editorial support. Dr. To acknowl-
substrate metabolism, was shown to ameliorate edges the support from the Overseas Fellowship
cardiac energetic impairment, correct diastolic Award from the National Heart Foundation of
dysfunction, and increase exercise capacity in New Zealand.
symptomatic HCM patients (77).
Reprint requests and correspondence: Dr. Milind Y. Desai,
Myocardial tagging quantifies myocardial me- Tomsich Department of Cardiovascular Medicine, Heart
chanics parameters such as strain, strain rate, and and Vascular Institute, Cleveland Clinic, J1-5, 9500
torsion and has been studied in HCM. Not Euclid Avenue, Cleveland, Ohio 44195. E-mail:
unexpectedly, strain is reduced in hypertrophied desaim2@ccf.org.
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atic hypertrophic cardiomyopathy. sessed by speckle tracking echocardiog- For supplementary videos and their legends,
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17. Cardiac Magnetic Resonance in Hypertrophic Cardiomyopathy
Andrew C.Y. To, Ashwat Dhillon, and Milind Y. Desai
J. Am. Coll. Cardiol. Img. 2011;4;1123-1137
doi:10.1016/j.jcmg.2011.06.022
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