MRI to assess arrhythmia and cardiomyopathies

md.manar al_mohammad

17 April 2020

Razan abdalrahman

Abstract
Cardiovascular magnetic resonance (CMR) is highly precise for morphological
and functional analyses of the myocardium, and has been used to assess different
types of cardiomyopathies. Its ability to characterize tissue, especially with
gadolinium (Gd) delayed enhancement techniques, has shown promising results for
the diagnosis of arrhythmogenic cardiomyopathies. In this review we discuss the
background and potential of this approach, as well as its usefulness for assessing
arrhythmias and cardiomyopathies.

Interduction
CARDIOMYOPATHIES ARE DISEASES of the myocardium associated with
cardiac dysfunction. The latest report of the World Health Organization classifies
them according to the dominant pathophysiology (Table 1).The diagnosis of
cardiomyopathies has been greatly improved in recent years by imaging techniques.

Table 1. WHO Classification of Cardiomyopathy

 

Dilated Ischemic  Metabolic

Hypertrophic Unclassified Inflammatory

Restrictive Specific Valvular
 Echocardiography is the most commonly used method for functional evaluation
and classification of cardiomyopathy because it is widely available. However,
cardiovascular magnetic resonance (CMR) has recently gained acceptance as a
useful tool for both functional evaluation and tissue characterization. CMRI is now
firmly established in clinical and research cardiovascular medicine as a method for
evaluating cardiomyopathy. CMRI is unrivaled in its ability to characterize soft
tissue structures, and its versatility makes it a promising new diagnostic tool for
cardiomyopathies. It is also redefining our understanding of the conditions that can
adversely affect the myocardium because it has the ability to provide high resolution
images of the heart in any desired plane. CMRI is not a single technique; consists of
several techniques that can be performed separately or in various combinations
during a patient examination. For example, cine CMRI can provide accurate
assessments of cardiac morphology and function, first pass contrast enhanced
perfusion CMRI with and without vasodilators can assess myocardial perfusion
reserve, and delayed contrast enhanced (DCE) . CMRI can be used to
noninvasively detect scar tissue. The latter feature can be of value in establishing
the appropriate diagnosis, and with such a diagnosis one can sometimes interrupt
the progression of disease to overt heart failure by instituting the appropriate
treatment .

The presence of scar tissue in cardiomyopathy has been related to the genesis of
arrhythmia, particularly in ischemic heart disease, and some cardiomyopathies are
also associated with severe arrhythmias that can be life threatening. The
electrophysiological effects of cardiomyopathy have been studied in several different
animal models, as well as in human tissue from biopsies and explanted hearts.
These studies reveal that electrical remodeling occurs in myopathic hearts. Globally,
cell necrosis and replacement of myocytes with scar tissue occur. The remaining
cells develop hypertrophy and altered ion channel and gap ‐junction expression.
These changes affect ventricular mechanical function and promote arrhythmia ,Scar
tissue can be detected with the use of DCE,CMR.
DILATED CARDIOMYOPATHY (DCM)

DCM is characterized by left ventricular (LV) or biventricular dilatation and

impaired contraction. Although a significant proportion of cases are of unknown
etiology, various pathological conditions (e.g., viral, genetic, toxic, and immune
deficient) can lead to this condition . The clinical presentation usually involves heart
failure, which is often progressive . The anatomical and functional abnormalities of
dilated cardiomyopathy are clearly demonstrated and measured by CMRI (Fig.1).
Functional cine CMRI can provide precise measurements and usually shows a
uniform wall thickness that appears thin due to increased ventricular volumes.
However, there are increased left ventricular (LV) mass, reduced stroke volume,
reduced ejection fraction, and usually diffuse hypokinesis, although this is not
mandatory . Here CMRI is superior to echocardiography for follow up evaluations
because it does not use mathematical models to estimate mass or function. This
feature has been increasingly used to monitor progression of disease because it
provides accuracy and is less operator dependent. Myocardial tagging has been
reported to provide evidence of severe reductions in fiber shortening and the
absence of normal systolic LV wall thickening from base to apex . Tagging
noninvasively alters the local magnetization with radiofrequency (RF) pulses by
generating a grid consisting of nulled orthogonal lines. Because saturated rows of
protons comprise this grid structure, the tagging is “embedded” in the tissue and its
motion can be reliably tracked throughout the cardiac cycle .

Figure 1

blood four chamber image of the heart in diastole (a) and systole (b) showing LV
enlargement in a patient with DCM .

HYPERTROPHIC CARDIOMYOPATHY (HCM)

 HCM is characterized by LV and/or right ventricular (RV) hypertrophy. This is
caused mainly by an inherited defect in the protein components of the cardiac
sarcomere. The pathologically characteristic feature is localized, but otherwise
unexplained myocardial hypertrophy is associated with significant myofibrillar
disarray. Accurate and early diagnosis of HCM is essential because many of these
patients are at risk for recurrent arrhythmias and premature cardiac death. The
genetic nature of the disorder has important implications with respect to the
screening of family members .

Echocardiography is the standard technique for evaluating this entity , and it is

important to obtain precise measurements of the LV wall thickness to establish a
prognosis. CMR is an appropriate alternative to confirm the diagnosis or identify
atypical cases . CMR is highly accurate for measuring wall thickness (Fig. 3).
Echocardiography may be limited by the acoustic window available, and is limited in
the evaluation of the LV apex, a common site of hypertrophy.

Figure 3

blood long axis image of the heart showing the LV (arrow) of a patient with HCm .
Figure 4

HCM a: Bright blood LV long axis image of the heart showing septal hypertrophy
(arrow) in a patient with HCM.

b: Bright blood LV short axis image of the heart showing septal hypertrophy (arrow)
in a patient with HCM.

c: Delayed Gd enhanced short axis image of the heart showing septal hypertrophy
with diffuse enhancement (arrows), indicative of collagen deposition .

In a recent longitudinal study , life threatening arrhythmias occurred at a rate of

5% per year for primary prevention, and twice that in secondary prevention cases.
The same study confirmed ventricular tachycardia (VT) or fibrillation as the
arrhythmias that led to sudden cardiac death. Although it has not been proven
definitively, slow conduction and re entry occurring in the presence of scar tissue are
possible mechanisms of these arrhythmias. Varnava et al demonstrated in a series
of 75 patients that the risk for re entry arrhythmias was related to the degree of
fibrosis. Using Holter monitoring, Teraoka et al showed that VT occurred more
frequently in patients with greater degrees of hyperenhancement .

Another potential application of CMR is in the evaluation of flow dynamics in the

LV outflow tract in patients with dynamic obstruction , morphological and functional
changes associated with surgical myectomy , and, more recently, alcohol septal
ablation . The latter technique is gaining widespread acceptance, and CMR can be
used to identify results of the procedure in the acute phase by evaluating the site
and extension of infarction (Fig. 5). At follow ‐up, CMR has shown changes in LV
mass

Figure 5

HCM after treatment. a:

First pass perfusion MRI,
LV short axis image of the
heart, showing septal
perfusion defect (arrow)
following septal alcohol
ablation. b: Delayed Gd
enhanced LV short axis image of the heart showing enhancement of the septum
(arrows) due to myonecrosis following alcohol ablation .

RESTRICTIVE CARDIOMYOPATHY (RCM)

RCM is the least common of the cardiomyopathies. RCM is characterized by

restrictive filling and reduced diastolic volume of either or both ventricles with normal
near normal systolic function and wall thickness . This disease may be idiopathic or
associated with other infiltrative diseases, such as amyloidosis (Fig. 7) and
endomyocardial disease with or without hypereosinophilia . RCMs cause symptoms
and signs of left and/or right side failure because they affect both ventricles. Some
patients may have complete heart blockage due to fibrosis encasing the sinoatrial or
atrioventricular nodes . On MRI images, features caused by impaired ventricle filling
include small or normal sized ventricles and dilation of the atria, superior and inferior
vena cava, and hepatic veins. Masui et al demonstrated that CMR can serve as a
noninvasive examination for the definitive diagnosis of constrictive pericarditis, and
can help distinguish between constrictive pericarditis and RCM on the basis of
pericardial thickness. The normal pericardium, seen as a hypointense line less than
4 mm thick, is very clearly visible on turbo spin‐echo T1 sequences. The diagnostic
accuracy of the existence of pericardial thicknesses of over 4 mm on MR images is
93%. This MR finding is the diagnostic clue for differentiating between constrictive
pericarditis and RCM .

Figure 7

Delayed Gd enhanced four

chamber image of the heart
showing areas of diffuse
enhancement (arrows), indicative
of amyloid deposition .

ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

(ARVC)
 ARVC is a disease characterized by enlargement, dysfunction, and fibrofatty
infiltration of the RV. It is recognized clinically by ventricular tachyarrhythmia,
abnormal RV morphology, and RV dysfunction. Although it is rare, it may be
responsible for 10–20% of sudden cardiac death due to arrhythmias in young people
in certain populations .

Many methods are available to assess the RV, but techniques (such as CMR)
that facilitate comprehensive coverage of the RV are essential . Combined with its
ability to characterize fatty/fibrofatty infiltration of the RV (Fig. 8), CMR has rapidly
evolved into the diagnostic standard for identifying ARVC in experienced centers
Fibrofatty tissue may have a role in the development of cardiac arrhythmias. Several
investigators have looked at the clinical value of tissue characterization in ARVC.
Tandri et al assessed consecutive patients referred for diagnostic evaluation. RV
late Gd enhancement was observed in 100% of the patients identified as having
ARVC by RV biopsy. Furthermore, DCE,CMR was found to predict inducible
sustained VT at electrophysiology study, although the prognostic value of this
finding remains unclear . DCE CMR for fibrosis/scar assessment in the RV has to
be done with optimized techniques. Desai et al evaluated the inversion time (TI) for
signal suppression in the RV compared to the LV. The TI for myocardial signal
suppression appears to differ between the LV and RV. Potential mechanisms
include partial volume averaging with fat or blood pool (related to increased
trabeculation) in the RV. Alternatively, increased blood pool signal (within Thesbian
veins or arterioluminal communications) in the RV compared to the LV leads to
altered TI times due to similar partial volume effects .

Figure 8

a: Fast
spin‐echo
T1 axial
image of
the heart
showing
signal
abnormality
present in
the RV and LV walls (white arrows). b: Fast spin echo, axial, fat saturated T1 image
of the heart, confirming fat infiltration of the RV and LV walls (white arrows) .

 Conclusion

CMR is an invaluable tool, not only in diagnosis between cardiomyopathy, but

also in aiding the accurate diagnosis of the subtype of cardiomyopathy. CMR should
routinely be integrated in the diagnostic workup of various cardiomyopathies .