Elsevier

Progress in Cardiovascular Diseases

Volume 54, Issue 3, November–December 2011, Pages 253-265
Progress in Cardiovascular Diseases

The Current and Emerging Role of Cardiovascular Magnetic Resonance in the Diagnosis of Nonischemic Cardiomyopathies

https://doi.org/10.1016/j.pcad.2011.08.007Get rights and content

Abstract

Imaging plays a crucial role in the diagnosis, management, and prognosis assessment of patients with nonischemic cardiomyopathies. Over the past decade, the role of cardiovascular magnetic resonance imaging in clinical practice has been rapidly expanding. The technique's unsurpassed accuracy in defining cardiac morphology and function and ability to provide tissue characterization make it particularly well suited for the study of patients with nonischemic cardiomyopathies. In this review article, we provide an overview of the main cardiovascular magnetic resonance features of nonischemic cardiomyopathies, highlighting the diagnostic and prognostic utility of the technique in this heterogenous group of diseases.

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.

References (86)

  • A.S. Adabag et al.

    Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance

    J Am Coll Cardiol

    (2008)
  • T. Papavassiliu et al.

    CMR findings in patients with hypertrophic cardiomyopathy and atrial fibrillation

    J Cardiovasc Magn Reson

    (2009)
  • O. Bruder et al.

    Myocardial scar visualized by cardiovascular magnetic resonance imaging predicts major adverse events in patients with hypertrophic cardiomyopathy

    J Am Coll Cardiol

    (2010)
  • U.S. Valeti et al.

    Comparison of surgical septal myectomy and alcohol septal ablation with cardiac magnetic resonance imaging in patients with hypertrophic obstructive cardiomyopathy

    J Am Coll Cardiol

    (2007)
  • T.D. Karamitsos et al.

    Ischemic heart disease: comprehensive evaluation by cardiovascular magnetic resonance

    Am Heart J

    (2011)
  • W.C. Roberts et al.

    Idiopathic dilated cardiomyopathy: Analysis of 152 necropsy patients

    Am J Cardiol

    (1987)
  • A. Jain et al.

    Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia

    J Cardiovasc Magn Reson

    (2008)
  • H. Tandri et al.

    Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging

    J Am Coll Cardiol

    (2005)
  • S. Sen-Chowdhry et al.

    Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity

    J Am Coll Cardiol

    (2008)
  • M.G. Friedrich et al.

    Cardiovascular magnetic resonance in myocarditis: a JACC White paper

    J Am Coll Cardiol

    (2009)
  • H. Abdel-Aty et al.

    Diagnostic performance of cardiovascular magnetic resonance in patients with suspected acute myocarditis: comparison of different approaches

    J Am Coll Cardiol

    (2005)
  • S.E. Petersen et al.

    Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging

    J Am Coll Cardiol

    (2005)
  • I.S. Syed et al.

    Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis

    JACC Cardiovasc Imaging

    (2010)
  • B.A. Austin et al.

    Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis

    JACC Cardiovasc Imaging

    (2009)
  • A.M. Maceira et al.

    Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis

    J Cardiovasc Magn Reson

    (2008)
  • I. Noth et al.

    Churg-Strauss syndrome

    Lancet

    (2003)
  • R. Wassmuth et al.

    Cardiovascular magnetic resonance imaging detects cardiac involvement in Churg-Strauss syndrome

    J Card Fail

    (2008)
  • J.C. Wood

    Cardiac iron across different transfusion-dependent diseases

    Blood Rev

    (2008)
  • T.D. Karamitsos et al.

    Tako-tsubo cardiomyopathy presenting with features of left ventricular non-compaction

    Int J Cardiol

    (2008)
  • H. Abdel-Aty et al.

    Myocardial edema is a feature of Tako-Tsubo cardiomyopathy and is related to the severity of systolic dysfunction: insights from T2-weighted cardiovascular magnetic resonance

    Int J Cardiol

    (2009)
  • A.A. Elesber et al.

    Transient cardiac apical ballooning syndrome: prevalence and clinical implications of right ventricular involvement

    J Am Coll Cardiol

    (2006)
  • N.B. Ntusi et al.

    Aetiology and risk factors of peripartum cardiomyopathy: a systematic review

    Int J Cardiol

    (2009)
  • J. Marmursztejn et al.

    Delayed-enhanced cardiac magnetic resonance imaging features in peripartum cardiomyopathy

    Int J Cardiol

    (2009)
  • F.X. Lescure et al.

    Chagas disease: changes in knowledge and management

    Lancet Infect Dis

    (2010)
  • C.E. Rochitte et al.

    Myocardial delayed enhancement by magnetic resonance imaging in patients with Chagas' disease: a marker of disease severity

    J Am Coll Cardiol

    (2005)
  • M.A. McAteer et al.

    An approach to molecular imaging of atherosclerosis, thrombosis, and vascular inflammation using microparticles of iron oxide

    Atherosclerosis

    (2010)
  • P. Richardson et al.

    Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies

    Circulation

    (1996)
  • B.J. Maron et al.

    Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention

    Circulation

    (2006)
  • P.S. Douglas et al.

    ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 Appropriate Use Criteria for Echocardiography. A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance American College of Chest Physicians

    J Am Soc Echocardiogr

    (2011)
  • T.D. Karamitsos et al.

    Operator induced variability in left ventricular measurements with cardiovascular magnetic resonance is improved after training

    J Cardiovasc Magn Reson

    (2007)
  • O. Catalano et al.

    Intra-observer and interobserver reproducibility of right ventricle volumes, function and mass by cardiac magnetic resonance

    J Cardiovasc Med (Hagerstown)

    (2007)
  • L.J. Anderson et al.

    Cardiovascular T2-star (T2⁎) magnetic resonance for the early diagnosis of myocardial iron overload

    Eur Heart J

    (2001)
  • T.D. Karamitsos et al.

    Cardiovascular magnetic resonance in heart failure

    Curr Cardiol Rep

    (2011)
  • Cited by (0)

    Statement of Conflict of Interest: see page 262.

    View full text