Imaging in Dilated Cardiomyopathy

Overview

The World Health Organization (WHO) defines dilated cardiomyopathy as a condition in which the ventricular chambers exhibit increased diastolic and systolic volume and a low (< 40%) ejection fraction.^{[1, 2]} In the WHO/International Society and Federation of Cardiology classification, dilated cardiomyopathy in its primary (eg, idiopathic or familial) and secondary forms is the most common cause of the clinical syndrome of chronic heart failure.

See the images below depicting cardiomyopathy.

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Chest radiograph shows a large heart. Image does not provide any clue to the etiology of the dilated cardiomyopathy; however, the healing rib fracture....

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Magnified right chest radiograph shows an area of right lower lobe aspiration pneumonia in a patient with dilated cardiomyopathy (same patient as in t....

The prevalence of heart failure is approximately 1-1.5% in the adult population of Western countries. Dilated cardiomyopathy is associated with a large number of systemic or cardiac diseases, including specific heart muscle diseases (eg, ischemic cardiomyopathy, diabetic cardiomyopathy, alcoholic cardiomyopathy).

Dilated cardiomyopathy comprises approximately 90% of all cardiomyopathies; approximately 25% of all cases of dilated cardiomyopathy are of unknown etiology.^[3] Despite improved treatment, the mortality rate for dilated cardiomyopathy remains high, with a median period of survival of 1.7 years for men and 3.2 years for women. The natural history of the condition is progressive, and its cost, disability, and morbidity are among the highest of any disease.^{[4, 5, 6]}

For excellent patient education resources, visit eMedicineHealth's Heart Health Center. Also, see eMedicineHealth's patient education articles Atrial Fibrillation, Lupus (Systemic Lupus Erythematosus), Alcoholism, Sickle Cell Disease, and Growth Failure in Children.

Preferred radiologic examination

Currently available radiologic investigations are as varied in technique and sophistication as they are in cost. Studies vary from the noninvasive to the invasive and from the dynamic to the static. Radiologic tests are used to help make a diagnosis, to assess the degree of cardiac dysfunction, to identify a cause (though this is unusual), and to guide therapy.

It is difficult to make an accurate clinical identification of heart failure resulting from poor ventricular function, but it is important to do so because of the need to relieve symptoms. A substantial number of patients with heart failure have normal ECG results.^[7] In other patients with apparent heart failure, echocardiography provides extra information on the nature of the cardiac disease that affects management.^[8] In addition, providing appropriate treatment is important in patients affected more severely (ejection fraction < 35-40%), in whom treatment can significantly reduce the mortality rate.^{[9, 10, 11]}

The initial chest radiograph should usually be followed by echocardiography. These studies may be the only investigations required, and this approach is by far the most common method for diagnosing dilated cardiomyopathy. Subsequent evaluation of cardiac function may be performed by using cine computed tomography (CT) scanning (in rare cases), nuclear scintigraphy, or magnetic resonance imaging (MRI).^{[12, 13]}

MRI also is rarely used to diagnose or investigate dilated cardiomyopathy, because the information obtained during echocardiography is often adequate. Although MRI has a valuable role in the diagnosis of specific causes of dilated cardiomyopathy (eg, myocarditis, sarcoidosis, hemochromatosis), the expertise required to evaluate these conditions is not widely available.^[14]

Limitations of techniques

Chest radiography is good for assessing the effects of cardiac dysfunction on pulmonary perfusion and the development of pulmonary edema. Radiographs seldom help in identifying the etiology of the dysfunction.

Echocardiography is an excellent method for assessing cardiac function, determining the presence of valve lesions, detecting complications such as thrombus or pericardial effusion, and assessing response to treatment. Echocardiography may be limited in 10-20% of patients by restricted acoustic access and is dependent on operator experience.

Cine CT scanning and CT angiography scanning require radiation and intravenous contrast enhancement; therefore, these techniques are seldom used.

Nuclear scintigraphy is a repeatable technique that often is used when serial examination of cardiac function is required. It is not as valuable as echocardiography, since it provides little anatomic information.

MRI and magnetic resonance angiography (MRA) are the most accurate methods for assessing cardiac anatomy or function. MRI/MRA is used when echocardiography is inadequate, but this study is often not used because of its relatively high cost and limited availability, as well as contraindications if ferromagnetic metallic foreign bodies are present in the patient.

• References

Radiography

Despite the development of cross-sectional imaging techniques, chest radiography remains useful in evaluating cardiac disease. Radiography is inexpensive and poses low risk to the patient. Moreover, it may provide useful information in guiding further investigations.

If the presenting symptom is dyspnea, radiographic findings may differentiate cardiac etiologies (eg, alcoholic cardiomyopathy [as in the first 3 images below]) from pulmonary ones. Cardiomegaly (cardiac shadow >50% of thorax width on the posteroanterior view), Kerley B lines (prominent lines in the peripheral parts of the lungs, secondary to interstitial edema [see the fourth image below]), pleural effusions, and cephalization of the pulmonary vasculature (as in the fifth image below) are consistent with a cardiac origin of dyspnea.

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Chest radiograph shows a large heart. Image does not provide any clue to the etiology of the dilated cardiomyopathy; however, the healing rib fracture....

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Magnified right chest radiograph shows an area of right lower lobe aspiration pneumonia in a patient with dilated cardiomyopathy (same patient as in t....

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Magnified left chest radiograph in a patient with dilated cardiomyopathy shows rib fractures (arrow) resulting from repeated chest injuries that occur....

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Magnified chest radiograph of the right costophrenic angle in a patient with dilated cardiomyopathy and interstitial pulmonary edema, as demonstrated ....

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Chest radiograph in a patient with dilated cardiomyopathy shows redistribution of pulmonary blood flow toward the upper lungs (cephalization).

The pattern of cardiac and great vessel enlargement, as well as of alveolar shadowing, may indicate an etiology.

In dilated cardiomyopathy, nonspecific cardiomegaly is usually found, with dilatation of the superior vena cava (see the images below) and the azygos vein.^[15]

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Chest radiograph in a patient presenting with heart failure, which was subsequently found to be the result of dilated cardiomyopathy. Although cardiac....

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Five-year follow-up chest radiograph in a patient with dilated cardiomyopathy (same patient as in the previous image). The heart is enlarged, but sign....

Development of cardiomegaly is easily tracked using chest radiography and is associated with a worse prognosis.

Although coronary artery calcification may be a clue to ischemic pathology, it is infrequently identified.

Limitations

Chest radiograph is a good method to detect a large heart and pulmonary vascular changes in heart failure; however, it often provides little information on etiology, on the degree of cardiac dysfunction, on complicating factors such as valve dysfunction, or on pericardial effusion. For this reason, radiography is usually augmented with a cross-sectional imaging test, usually echocardiography.

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Computed Tomography

Although ultrafast CT scanners (electron beam) and multidetector-row CT scanners can be used with ECG gating to assess ventricular function, little reason exists to employ this equipment. CT scanners require ionizing radiation and injection of relatively large amounts of iodinated contrast agents.^[16]

Gated CT scanning is an accurate means of evaluating cardiac function, especially with the use of ultrafast CT scanning and 50-millisecond image acquisition. Only 1 short breath-hold period is required, and definition of the endocardial margins is excellent, allowing a degree of automation for defining the ventricular volumes. However, coverage of the entire heart may be difficult.

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Magnetic Resonance Imaging

MRI and MRA offer excellent noninvasive means of displaying cardiac and coronary anatomy.^[14] They provide high spatial and contrast resolution, which can be used to evaluate congenital and acquired abnormalities and assess results of therapy.^[17] Pulse sequences (eg, cine MRA sequences) that provide high temporal resolution can be used to identify and quantify functional abnormalities.

Myocardial edema and inflammation play an important role in dilated cardiomyopathy and have been identified using cardiovascular MRI. T2 mapping has shown that myocardial water content is greater in DCM patients and that the myocardial water content increases as the disease progresses.^[18]

Currently, cine MRA is well established as the most accurate modality (some refer to it as the criterion standard) for validating other imaging techniques and for evaluating the effects of medical therapy on ventricular function.^{[19, 20]} MRA can be used to assess cardiac dimensions and systolic function, and phase-contrast techniques can be used to evaluate diastolic dysfunction.^[21] Provided ECG gating and breath-holding are effective, reliable and accurate imaging of cardiac function and anatomy is typical.

Contraindications

Contraindications to cardiac MRI are identical to MRI contraindications at any other anatomic site. They include the presence of a pacemaker or other implanted electrical stimulator, ferromagnetic material in the eye, or intracerebral aneurysm clips. Except for the pre-6000 series of Starr-Edwards valves (few of which, if any, exist), no cardiac valve prostheses are a contraindication at the magnetic strengths (< 2 T) currently used for diagnostic MRI.^[5] Retained pacemaker wires, surgical clips, and wire sternal sutures are not contraindications to MRI.^[22] Coronary and pulmonary artery stents may be imaged safely, although some authors believe it prudent to exclude patients with new (< 6-wk-old) stents from MRI.

Pregnancy is a relative contraindication. If MRI is necessary, the unknown risk to the fetus must be weighed against the benefit to the mother.

Gadolinium-based contrast agents have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the Medscape Reference topic Nephrogenic Systemic Fibrosis. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.

Technique

MRI of the heart requires an adequate ECG tracing to provide a gating signal for image acquisition at the same part of the cardiac cycle, although some newer sequences (such as true fast imaging with steady-state free precession) are fast enough to be implemented without gating.^[23] The quality of the examination probably depends more on this parameter than on any other. The effects of strong magnetic fields on the character of the ECG tracing are unpredictable and may compromise gating.

The hydromagnetic effect of blood flowing in a strong magnetic field may distort the ECG trace and make ECG gating difficult or impossible. Spin-echo MRI has long been used as an accurate method for assessing cardiac dimensions. Phantom studies have shown a high degree of correlation, which is superior to that of other non-MRI techniques.

Spin-echo MRI is the most accurate method for assessing cardiac dimensions, but it is time consuming and provides only static information.^[24] Evaluation of cardiac function is improved with cine MRA. For this, either breath-hold or non–breath-hold techniques can be used.^[23]

Left ventricular function is optimally evaluated in the following 3 planes (2 of which are demonstrated in the images below):

• Right anterior oblique equivalent
• Four-chamber equivalent or oblique axial
• Multilevel short axis

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  Oblique coronal (right anterior oblique perspective) end-diastolic cine magnetic resonance angiogram in a patient in the recovery phase of postpartum ....

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  Oblique coronal end-systolic cine magnetic resonance angiogram in a patient recovering from postpartum dilated cardiomyopathy.

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  Oblique axial end-diastolic breath-hold cine magnetic resonance angiogram in a patient with dilated ischemic cardiomyopathy. The left ventricle (LV) i....

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  Oblique axial end-systolic breath-hold cine magnetic resonance angiogram in a patient with dilated ischemic cardiomyopathy. Only moderate contraction ....

   

  

   

Once the right anterior oblique equivalent and long-axis 4-chamber equivalents are acquired, they can be used to set up short-axis acquisitions. Usually, the authors acquire the images one third and two thirds of the distance between the mitral valve annulus and the apex of the LV, although contiguous sections can be acquired, if necessary, for accurate assessment of left ventricular mass and volume.

As demonstrated in the image below, a variety of mathematical models can be used to derive ventricular dimensions from long-axis views when time is more important than extreme accuracy for measuring volume and mass.^[25] For most clinical purposes, the accuracy of long-axis methods compares well with alternative non-MRI techniques.^[24] Long-axis views also show signal voids resulting from mitral regurgitation and may be used to assess aortic regurgitation.^[26] Short-axis views usually provide better definition of the endocardium, with higher intraluminal signal intensity and a good assessment of septal, inferior, and posterior wall function. Software to automatically calculate ejection fractions and ventricular volumes from data on the images is available.^[27]

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Cine magnetic resonance angiogram in a patient with postpartum cardiomyopathy. Using the area/length method and the Simpson rule, the ejection fractio....

Breath-hold tagging sequences have been developed (see the images below). The use of linear or cross tags enhances appreciation of subtle regional wall motion abnormalities.^{[28, 29]}

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Oblique axial end-diastolic breath-hold linear-tagged cine magnetic resonance angiogram in a patient with dilated ischemic cardiomyopathy. Linear tags....

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Oblique axial end-systolic breath-hold linear-tagged cine magnetic resonance angiography in a patient with dilated ischemic cardiomyopathy. The linear....

Cardiac MRI is excellent for assessing cardiac function in dilated cardiomyopathy because of the high intrinsic contrast between blood and myocardium, especially on cine MRA.^[30] Cine MRA is a repeatable examination, as compared with echocardiography, with less risk of intertest variation resulting from differences in operator or technique.^[31] Cine MRA is especially suitable for evaluating the right ventricle, which is difficult to reliably image with other techniques.^[32]

Multilevel cine MRA is the most accurate method for assessing cardiac function and dimensions.^[33] In addition to providing a means of assessing cardiac function, signal abnormalities may indicate a metabolic or inflammatory etiology for dilated cardiomyopathy.^{[34, 35]}

In some patients, endocardial surfaces may be defined poorly or intraluminal signal may be lost, as in areas with a slow flow related to ventricular dyskinesia. This may occur especially on long-axis views, which increase the likelihood of in-plane signal saturation. This problem can be overcome by using intravenous contrast media (eg, gadolinium diethylenetriamine pentaacetic acid), which increase signal intensity for up to 20 minutes.^[36] However, this technique reduces the extent of any flow void resulting from valve regurgitation.

Findings

Causes of abnormal myocardial signal intensity on T1-weighted images include the following^[37] :

• Hypertrophic cardiomyopathy (variable signal)
• Hemochromatosis/hemosiderosis (low signal intensity, also shortened T2 relaxation)
• Arrhythmogenic right ventricular dysplasia (high signal intensity in right ventricular wall)
• Transplant rejection
• Myocarditis (after gadolinium enhancement)

Causes of high myocardial signal on T2-weighted images include the following:

• Myocardial infarction
• Metastases
• Myocarditis (variable)
• Abscesses
• Sarcoidosis
• Amyloidosis
• Toxoplasmosis
• Systemic lupus erythematosus
• Wegener granulomatosis
• Transplant rejection

• References

Ultrasonography

Echocardiography is the next and, usually, most useful investigation after chest radiography. Echocardiograms (seen in the images below) are useful for excluding other causes of a large heart shadow, such as pericardial effusion or multiple valve disease. In dilated cardiomyopathy, all of the heart chambers are typically dilated, and both ventricles are diffusely hypokinetic.

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Two-dimensional end-diastolic echocardiogram in a patient with dilated cardiomyopathy (congestive cardiomyopathy at diastole) shows the transverse dia....

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Two-dimensional end-diastolic echocardiogram in a patient with dilated cardiomyopathy (congestive cardiomyopathy at systole). The transverse diameter ....

A feature that may help distinguish idiopathic dilated cardiomyopathy from ischemic cardiomyopathy is the presence of global, rather than regional, dysfunction. However, in some patients with dilated cardiomyopathy, regional dysfunction may be seen as a result of the preservation of systolic function at the base of the LV or the presence of left bundle branch block, which causes paradoxical septal motion.

Right ventricular function often is relatively preserved in ischemic cardiomyopathy. Ventricular walls may be normal, increased, or decreased in thickness. The left atrium usually is dilated early on, while right ventricular and right atrial enlargement occur later.

The most precise left ventricular cavity and wall-thickness measurements at end diastole, end systole, and ejection fraction are obtained by using M-mode echocardiography (shown in the image below), which has the best temporal resolution of any imaging technique. Measurements obtained in a standardized way (at the tips of the mitral valve leaflets) are important to allow for useful comparison with published reference values.

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M-mode echocardiogram acquired at the level of the tips of the mitral valve leaflets shows dilated and poorly contracting left ventricle in a patient ....

Two-dimensional echocardiography, with its superior spatial coverage, is used to guide the positioning of the M-mode sample for direct measurements of ventricular wall thickness, left ventricular volumes, and ejection fraction. An advantage of 2-dimensional echocardiography (compared with M-mode imaging) is that the myocardial mass, chamber volumes, and ejection fraction of an abnormally shaped ventricle can be measured. However, because of the subjective nature of 2-dimensional echo examination, its risk of error is higher than that of other cross-sectional imaging techniques.

In most patients, images of the mitral and tricuspid valves appear normal but with an abnormally central position with nearly equal excursion of the anterior and posterior leaflets. Significant atrioventricular regurgitation may occur because of incomplete closure of the mitral and tricuspid leaflets as a result of annular dilatation. The motion of the mitral valve may reflect the high diastolic pressure, with early opening of the valve leaflets appreciated on M-mode echo (so-called C-hump). Pericardial effusions are common, but they tend to be relatively small and do not cause significant hemodynamic problems. Mural thrombi may be seen in dilated chambers, especially those with severe hypokinesia. The above characteristics are demonstrated in the images below.

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M-mode echocardiogram in a patient with dilated cardiomyopathy shows the mitral valve (MV) centrally placed in the left ventricle (LV), with near equa....

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Apical 2-dimensional echocardiogram with color Doppler display (systolic image) in a patient with dilated cardiomyopathy shows a jet of mitral regurgi....

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Parasternal long-axis 2-dimensional systolic echocardiogram with color Doppler display. Compare this and the previous image, noting the eccentric natu....

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M-mode C-hump (arrow) echocardiogram in a patient with dilated cardiomyopathy. The tips of the mitral valve leaflets are displaced toward the center o....

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Two-dimensional echocardiogram in a patient with ischemic cardiomyopathy shows extensive thrombus (Th) in the apex of the left ventricle (LV).

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Nuclear Imaging

The most important contribution of nuclear cardiology is the ability to assess myocardial perfusion and ventricular function. Nuclear ventriculography is a reliable and reproducible method for assessing left ventricular function, an especially important consideration when one performs sequential studies to assess the effects of therapy.^{[38, 39]}

Radionuclide ventriculography (multiple-gated acquisition or first-pass scanning; shown in the images below) is used to estimate ventricular size and ejection fraction and to assess wall motion abnormalities. Diastolic dysfunction and the response to exercise also can be reliably evaluated.^{[39, 40]} In idiopathic dilated cardiomyopathy, the global ejection fraction is decreased and wall motion is uniformly poor, with the exception of possible sparing of the septal and anterior basal segments.

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End-diastolic cardiac nuclear ventriculogram (obtained with technetium-99m–tagged red blood cells) in a patient with dilated cardiomyopathy. The left ....

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Nuclear image of the heart in end systole, with the left ventricle outlined and the background unchanged, in a patient with dilated cardiomyopathy. No....

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Left ventricular time-activity curve, with end diastole (ED) and end systole (ES) demarcated, in a patient with dilated cardiomyopathy. The curve is s....

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Measured global left ventricular ejection fraction is 25.8% in a patient with dilated cardiomyopathy. This value is severely depressed (reference rang....

Radionuclide perfusion imaging can help in differentiating ischemic causes and other etiologies. A perfusion defect of greater than 40% strongly suggests ischemic etiology. Unfortunately, large and fixed (especially apical) perfusion defects have been reported in dilated cardiomyopathy.^[41]

Radionuclide ventriculography is frequently used to follow the cardiotoxic effects of drugs (eg, doxorubicin) that cause cardiomyopathy. Dilated cardiomyopathy caused by doxorubicin usually produces severe, irreversible left ventricular dysfunction symptoms of congestive heart failure; therefore, it is essential to detect ventricular dysfunction early, well before any irreversible changes occur. Because radionuclide ventriculography is a reproducible technique, it is used widely for this purpose.^[42] The technique can also be used to assess the response to therapy in patients with any form of idiopathic cardiomyopathy.

Gated single-photon emission CT (SPECT) has been used to successfully identify etiologies of dilated cardiomyopathy, because of its ability to depict myocardial perfusion and function. Myocardial perfusion and function imaging has been used to accurately classify cardiomyopathies as ischemic or nonischemic, depending on the gated SPECT and perfusion images.^[43] Ischemic cardiomyopathies tend to have reversible or fixed perfusion defects along with regional wall motion abnormalities on gated SPECT, while nonischemic cardiomyopathies demonstrate mild perfusion defects with diffuse wall motion abnormalities.

Degree of confidence

The degree of confidence is high. Nuclear ventriculography is a relatively accurate and repeatable method for assessing left ventricular function. It has been used for longitudinal studies to assess the response to medical treatment and to detect early changes resulting from drug toxicity, which may lead to dilated cardiomyopathy in patients undergoing chemotherapy with anthracycline cytotoxic agents.

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Angiography

Determining ischemic heart disease as a cause of cardiomyopathy is important because it may help in identifying treatable pathology. Coronary angiography remains the best technique for assessing epicardial coronary artery disease and for determining whether revascularization can potentially reverse the cardiomyopathy. Multiple studies of patients with symptomatic heart failure, coronary artery disease suitable for revascularization, and poor systolic function (with or without angina) have demonstrated a survival and ventricular function benefit in surgical treatment in comparison with medical therapy.

The need for coronary angiography depends on the following factors:

• A history of angina, prior myocardial infarction, or antecedent viral syndrome or family history of cardiomyopathy
• A high probability of coronary artery disease in the patient, as based on age, sex, and risk factors
• Generalized ischemic heart disease, as indicated on noninvasive tests, such as echocardiography or nuclear medicine studies at rest or after stress

Coronary angiography is performed in multiple projections because it is a projectional technique, and atherosclerotic lesions typically are eccentric. Many groups have correlated the degree of stenosis with ultimate clinical or pathologic outcome. The severity of obstructive disease is assessed in each coronary artery segment by comparing the arterial diameter at a point of maximum lumen reduction with that of a proximal or distal normal-appearing artery.

Nonatherosclerotic coronary disease occasionally causes ischemic heart disease. Examples include causes of arteritis (eg, Takayasu disease, giant cell arteritis, polyarteritis nodosa), infections (eg, syphilitic stenoses of the coronary ostia, aortic root abscess), congenital coronary anomalies, and Kawasaki disease.^[44]

During cardiac catheterization, a left ventriculogram usually is obtained to assess the LV, which demonstrates diffuse enlargement and reduced contractility. Abnormalities of segmental wall motion are common, and mural thrombi may be found in the apex.

Cine angiography was once regarded as the reference standard for assessing LV function. Currently, this is not the case; as a result of its projectional nature and the need to inject contrast agent, cine angiography may be inaccurate. In particular, it can fail to demonstrate regional wall motion abnormalities. Three-dimensional echo imaging and cine MRA are more accurate in assessing global and regional ventricular function.^[45] However, a few contraindications to cardiac catheterization have been identified.

• References

Author

Haroon R Afridi, MBBS, Interventional Radiologist, RCI Iowa Plc, Cedar Rapids, Iowa

Disclosure: Nothing to disclose.

Specialty Editors

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand

Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Attending Radiologist, Teaching Coordinator for Cardiac Imaging, Radiology Residency Program, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Justin D Pearlman, MD, ME, PhD, FACC, MA, Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Chair of Institutional Review Board, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

George Hartnell, MBChB Professor of Radiology, Tufts University School of Medicine; Director of Cardiovascular and Interventional Radiology, Department of Radiology, Baystate Medical Center

George Hartnell, MBChB is a member of the following medical societies: American College of Cardiology, American College of Radiology, American Heart Association, Association of University Radiologists, British Institute of Radiology, British Medical Association, Massachusetts Medical Society, Radiological Society of North America, Royal College of Physicians, Royal College of Radiologists, andSociety of Cardiovascular and Interventional Radiology

Disclosure: Nothing to disclose.

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