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Inherited Cardiomyopathies

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Magnetic Resonance Imaging of Congenital Heart Disease

Abstract

In order to plan optimal management and treatment strategies in patients with a suspected cardiomyopathy, the key initial factor is to establish the diagnosis and underlying etiology at an early stage. While many patients will present with symptoms and demonstrable ventricular dysfunction on echocardiography, often the underlying cause is not apparent, thus necessitating many “routine” invasive and non-invasive investigations such as angiography, echocardiography, holter monitoring, treadmill testing, and nuclear studies. There has been considerable progress in recent years in the development of imaging technologies which are now able to characterize a much wider number of cardiomyopathic processes than ever before in a non-invasive manner. The advent of gadolinium enhanced cardiac magnetic resonance imaging (CMR) has dramatically changed the non-invasive work-up of patients with a suspected cardiomyopathy. In a single scan setting it is now possible to provide a comprehensive assessment of both ischemic and non-ischemic cardiomyopathies providing detailed information on cardiac anatomy, function, tissue characterization, assessment of epicardial and microvascular perfusion, valvular flows, and coronary and peripheral angiography (Fig. 13.1). This comprehensive examination can be completed in a short period of time, typically 30–45 min, without the need for prolonged breath holds (5–10 s) or 2onizing radiation. establish definitive diagnoses with the greatest degree of clarity, helps guide and monitor therapeutic response, and assists in optimal risk stratification. Gadolinium based contrast agents are remarkably safe and the incidence of adverse reactions or nephrogenic systemic sclerosis (NSF) is exceedingly low. Follow up imaging to monitor progression and response to interventions can be performed safely and without any concern regarding cumulative radiation exposure.

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Author information

Authors and Affiliations

  1. Department of Cardiology, St. Vincent’s University Hospital, Dublin, Ireland

    Rory O’Hanlon M.D., MRCPI

  2. Centre for Cardiovascular Magnetic Resonance, Blackrock Clinic, Rock Road, Dublin, Ireland

    Rory O’Hanlon M.D., MRCPI

  3. Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, Sydney Street, London, SW3 6NP, UK

    Raad H. Mohiaddin M.D., Ph.D

Authors
  1. Rory O’Hanlon M.D., MRCPI
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  2. Raad H. Mohiaddin M.D., Ph.D
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Correspondence to Raad H. Mohiaddin M.D., Ph.D .

Editor information

Editors and Affiliations

  1. , Stritch School of Medicine, Loyola University Chicago, S. First Ave. 2160, Maywood, 60153, Illinois, USA

    Mushabbar A. Syed

  2. Magnetic Resonance Unit, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, United Kingdom

    Raad H. Mohiaddin

13.1 Electronic Supplementary Material

Below is the link to the electronic supplementary material.

Mid to apical HCM with significant apical aneurysm formation due to obstruction. Wall thinning in the aneurysmal segment noted (AVI 1728 kb)

Mid ventricular HCM with apical aneurysm formation (AVI 1728 kb)

LVOT view of basal and mid septal asymmetrical hypertrophy secondary to HCM with cavity obliteration in systole. Note no evidence of LVOT obstruction or systolic anterior motion of the mitral valve (AVI 1584 kb)

Four chamber view of marked asymmetrical HCM with near cavity obliteration in systole. No LVOT obstruction or SAM seen. The RV is also free of hypertrophy (AVI 1584 kb)

Short axis view in mid ventricle of HCM with marked asymmetrical septal hypertrophy and relative septal hypokinesis (AVI 1321 kb)

Apical HCM in two-chamber view with more marked focal apical inferior wall hypertrophy and apical obliteration in systole noted (AVI 1394 kb)

LVOT view of apical HCM with mid to apical obliteration in systole. No aneurysm seen. Note the presence of septal wall clefts (AVI 1394 kb)

Four chamber view of apical HCM with asymmetrical apical hypertrophy affecting both LV and RV apical segments. Apical obliteration of LV and RV noted in systole (AVI 1394 kb)

Dilated cardiomyopathy: Four chamber view demonstrating markedly dilated LV and RV with severe global hypokinesis and reduced EF. Note the increased trabecular pattern, a common feature of DCM. Mitral and tricuspid annular dilatation with mild to moderate central mitral and tricuspid valve regurgitation (AVI 1674 kb)

Dilated Cardiomyopathy: Two chamber view of DCM with Lv dilatation and severe global hypokinesis. Left atrial dilatation seen. The left atrial appendage is also seen in this cine (AVI 1674 kb)

218193_1_En_13_MOESM11_ESM.mpg

SSFP long axis of ARVC demonstrating a dilated RV with reduced RVEF and focal dyskinesis and aneurysmal dilatation of the RV free wall. Reduced left ventricular systolic function also noted (MPG 233 kb)

218193_1_En_13_MOESM12_ESM.avi

RVOT view in a case of ARVC demonstrating significant aneurysmal dilatation of the RVOT (AVI 10986 kb)

Four chamber view of LVNC demonstrating prominent lateral and apical trabeculations with moderate reduction in LV systolic function. The ratio of non-compact to compact myocardium in these regions is >2:1 in systole and >2.3:1 in diastole (AVI 1430 kb)

LVOT view of LVNC demonstrating marked trabecular pattern in the inferior and inferolateral walls from base to apex. Note the mitral papillary muscles attach into non-compact myocardium (AVI 1430 kb)

Representative SA view of LVNC demonstrating prominent trabecular meshwork in the lateral and inferior walls meeting criteria for LVNC in both systole and diastole (AVI 1430 kb)

Movie 13.11

SSFP long axis of ARVC demonstrating a dilated RV with reduced RVEF and focal dyskinesis and aneurysmal dilatation of the RV free wall. Reduced left ventricular systolic function also noted (MPG 233 kb)

Movie 13.12

RVOT view in a case of ARVC demonstrating significant aneurysmal dilatation of the RVOT (AVI 10986 kb)

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O’Hanlon, R., Mohiaddin, R.H. (2012). Inherited Cardiomyopathies. In: Syed, M., Mohiaddin, R. (eds) Magnetic Resonance Imaging of Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-4267-6_13

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