The Current and Emerging Role of Cardiovascular Magnetic Resonance in the Diagnosis of Nonischemic Cardiomyopathies
Section snippets
The CMR examination in cardiomyopathies
A comprehensive CMR protocol for patients suspected of having cardiomyopathy would include a variety of sequences in multiple imaging planes to assess specific features. Cardiovascular and extracardiac anatomy is assessed on black and/or bright blood images in standard orthogonal (eg, transverse, coronal, sagittal) and sometimes in individualized double-angulated (eg, oblique sagittal) planes. The evaluation of cardiac volumes, function, and mass using steady-state free precession sequences is
Hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy, affecting 0.2% of the general population.2 It is inherited in an autosomal dominant manner and shows a wide variation of phenotypic expression. Asymmetric involvement of the interventricular septum is the most common form of the disease. A common diagnostic criterion is a maximal focal LV wall thickness of greater than or equal to 15 mm.16 However, this is not an absolute requirement, and patients with lesser degrees
Dilated cardiomyopathy
Dilated cardiomyopathy (DCM) is characterized by ventricular chamber enlargement and systolic dysfunction with usually low-normal LV wall thickness.2 The differentiation between DCM and ischemic heart disease causing heart failure is important for therapeutic and prognostic purposes. The demonstration of normal coronary arteries is not sufficient on its own to exclude coronary artery disease as the underlying cause of cardiomyopathy. In contrast, a CMR scan with LGE in combination with coronary
Arrhythmogenic RV cardiomyopathy
Arrhythmogenic RV cardiomyopathy is an uncommon form of inheritable heart muscle disease (estimated prevalence, 1:5000).2 It primarily involves the right ventricle with progressive loss of myocytes and fibrofatty replacement of the myocardium, resulting in regional or global thinning and wall motion abnormalities. It should be noted that the diagnosis of ARVC cannot only be based on imaging criteria. In contrast, a combination of structural, histologic, electrocardiographic (ECG), arrhythmic,
Myocarditis
Myocarditis is an acute or a chronic inflammatory process affecting the myocardium produced by a wide variety of infectious agents (viruses, bacteria, etc), toxins, and drugs.2 In some instances, an episode of (frequently subclinical) viral myocarditis may trigger an autoimmune reaction resulting in LV dysfunction often appearing clinically as DCM. As an inflammatory process, myocarditis causes tissue changes including hyperemia and increased capillary permeability, edema, and, in severe cases,
Left ventricular noncompaction
Noncompaction of ventricular myocardium is a recently recognized cardiomyopathy characterized by prominent LV trabeculations with deep intertrabecular recesses, resulting from an arrest in the normal embryogenesis.2 The natural history of LV noncompaction is largely unresolved but includes LV remodeling with systolic dysfunction and heart failure, thromboembolic events, arrhythmias, and sudden death.45 Both familial and nonfamilial cases have been described. It is controversial whether LV
Amyloidosis
Cardiac amyloidosis describes clinically significant involvement of the heart by amyloid deposition, which may or may not be associated with involvement of other organs. Echocardiographic indicators of amyloidosis (ie, measurement of LV wall thickness, subjective assessment of myocardial appearance, and evaluation of diastolic function/restrictive physiology) are neither sensitive nor specific, and endomyocardial biopsy is invasive and subject to potential sampling error. Cardiovascular
Sarcoidosis
Sarcoidosis most commonly involves granuloma formation in the lungs. Other frequently involved organs include the lymph nodes, skin, eyes, heart, and the nervous, musculoskeletal, renal, and endocrine systems. Cardiac involvement in sarcoidosis, although common (reported in up to 1/4 of autopsy cases with proven extracardiac sarcoidosis), is extremely difficult to diagnose clinically antemortem. Cardiovascular magnetic resonance has unique value in the demonstration of cardiac involvement in
Anderson-Fabry disease
Up to 6% of patients who are initially diagnosed as HCM actually have evidence of Anderson-Fabry disease.55 This is an X-linked disorder of lysosomal metabolism that results in a deficiency of the lysosomal enzyme α-galactosidase A. Although Fabry disease is rare in the general population, it has a prevalence of around 3% in middle-aged male patients with otherwise unexplained LV hypertrophy.56 The implications of an incorrect diagnosis are considerable because patients with Anderson-Fabry
Endomyocardial fibrosis
Endomyocardial fibrosis (EMF) is the most frequent form of restrictive cardiomyopathy, affecting about 12 million people in the world, mainly children from poor areas in developing countries.59 It is characterized by fibrotic tissue deposition in the endocardium of the inflow tract and apex of one or both ventricles. Ventricular morphology is usually distorted with normal or reduced volumes, whereas atrial volumes are increased. Echocardiography is unable to fully characterize EMF, and it may
Churg-Strauss syndrome
Churg-Strauss syndrome is a rare type of necrotizing vasculitis characterized by granulomas and eosinophilia affecting nearly all organ systems but specifically the lungs and heart.62 Cardiac involvement is an important predictor of adverse prognosis and, once detected, should trigger intensified therapy. However, conventional diagnostic tests (ECG, echocardiography, and clinical symptoms) are nonspecific. Recognized cardiovascular manifestations are eosinophilic vasculitis, myocarditis,
Iron overload cardiomyopathy
Iron overload occurs in patients who require regular blood transfusions to correct genetic and acquired anemias, such as β-thalassemia major, sickle cell disease, and myelodysplastic syndromes.64 Although iron overload causes damage in many organs, accumulation of cardiac iron (cardiac siderosis) is a major cause of morbidity and mortality in patients with β-thalassemia major. Siderotic cardiomyopathy is reversible if chelation is commenced early, but diagnosis is often delayed because of the
Stress (“Tako-Tsubo”) cardiomyopathy
Stress cardiomyopathy, also known as apical ballooning syndrome, broken heart syndrome, and “Tako-Tsubo” cardiomyopathy, is a recently described clinical entity characterized by acute but rapidly reversible LV systolic dysfunction in the absence of atherosclerotic coronary disease.2 The term Tako-Tsubo cardiomyopathy was used to describe the syndrome because of the similarity between a Japanese octopus trap and the shape of the left ventricle during systole with its characteristic apical
Pericardial constriction
The normal pericardium appears as a hypointense line surrounding the heart with a minimal fluid layer. It is usually less than 2-mm thick and is indeed sometimes not visible on CMR images. Cardiovascular magnetic resonance can diagnose constrictive pericarditis by providing morphological evidence of an abnormally thickened pericardium associated with constrictive physiology (Fig 6).71 On black-blood anatomical CMR images, a pericardial thickness of more than 4 mm is suggestive of constriction.
Brugada syndrome—channelopathies
The Brugada syndrome is a relatively new clinical entity associated with cardiac sudden death in young people. It is identified by distinctive ECG patterns consisting of right bundle-branch block and coved-type or saddleback-type ST-segment elevation in the anterior precordial leads (V1-V3), which is often concealed and may be unmasked by the administration of sodium channel blockers.2 Although cardiac function may appear normal on echocardiography, recent CMR studies suggest that some patients
Peripartum cardiomyopathy
Peripartum cardiomyopathy is defined as a cardiomyopathy characterized by the development of heart failure in the last month of pregnancy and up to 5 months postpartum without any demonstrable cause of heart failure and in the absence of any preexisting heart disease that may be adversely affected by the stress of pregnancy.75 About 30% to 50% of patients with peripartum cardiomyopathy recover without complications, with their baseline LV systolic function at rest returning to normal. If LV
Mitochondrial myopathies
Mitochondrial disorders result in impaired oxidative phosphorylation, limiting the production of adenosine triphosphate, and most frequently affect the brain, heart and skeletal muscle, which are particularly vulnerable to defects in energy metabolism.79 A recent study demonstrated echocardiographic evidence for cardiomyopathy in 25% of patients with mitochondrial disease, most commonly in the form of concentric LV hypertrophy.80 There are only isolated case reports describing CMR findings in
Chagas disease
Chagas disease is caused by Trypanosoma cruzi infection. Cardiac involvement is the most serious long-term complication, occurring in approximately one third of seropositive individuals, and is the commonest cause of death from heart failure in many Latin American countries.83 It is characterized by progressive myocardial inflammation, resulting in a chronic disease characterized by fibrosis and cardiac dysfunction. The left ventricle typically shows areas of wall thinning, frequently leading
Future perspective
Over the past decade, CMR has developed from an exciting novelty to become an integral part of today's clinical routine. The unique ability of CMR to comprehensively assess cardiac morphology, function, and tissue structure has provided new pathophysiologic insights, improving our understanding of nonischemic cardiomyopathies and facilitating early diagnosis. Improvement in CMR software and hardware will further shorten scan times and allow the use of real-time imaging with better spatial and
Statement of Conflict of Interest
All authors declare that there are no conflicts of interest.
Acknowledgments
The authors acknowledge support from the British Heart Foundation (PG/08/101/26126) and the Oxford National Institute for Health Research Biomedical Research Centre program. Stefan Neubauer acknowledges support from the Oxford British Heart Foundation Centre for Research Excellence.
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Statement of Conflict of Interest: see page 262.