Cardiomyopathies: focus on cardiovascular magnetic resonance

Abstract

Cardiomyopathies (CMPs) are a group of often inherited diseases characterised by abnormalities and associated dysfunction of heart muscle. In the past decade, cardiovascular magnetic resonance (CMR) has emerged as a powerful tool in their assessment, providing data that are complementary to other aspects of clinical evaluation. Key advantages of CMR are three-dimensional visualisation of the heart and its relationship to thoracic structures; gold-standard quantification of cardiac volumes and function, which can safely be repeated over time (no ionising radiation is involved); and tissue characterisation to detect focal scar and fatty infiltration. This paper reviews the role of CMR in the clinical assessment of patients with CMPs.

Figures

Figure 1

Hypertrophic cardiomyopathy. Note left ventricular hypertrophy and, after contrast, late gadolinium enhancement of the hypertrophied areas and right ventricular insertion points (arrows). (a, c) Steady-state free precession cine in diastole [(a) four-chamber view; (c) short-axis view]. (b, d) Inversion recovery after gadolinium bolus [(b): four-chamber view; (d) short-axis view].

Figure 2

Cardiac amyloidosis. Note the bilateral pleural effusions and small pericardial effusion (arrows). The patient has mildly impaired radial systolic left ventricular function but severely impaired long-axis function with concentric left ventricular hypertrophy and dilated atria. (a) Steady-state free precession cine image in diastole, four-chamber view. (b) Inversion recovery after gadolinium bolus, four-chamber view; note the dark blood pool and bright myocardium.

Figure 3

A patient with sarcoidosis and paroxysmal complete heart block on 24 h electrocardiography. (a) Transverse multislice half-Fourier acquisition single-shot turbo spin echo images, showing extensive lung parenchymal abnormalities (right>left) and lymphadenopathy (arrow). (b) Short axis inversion recovery late gadolinium enhancement image shows patchy fibrosis (arrows).

Figure 4

Short-axis still steady-state free precession cine in diastole of a patient with Anderson–Fabry disease, showing (a) concentric left ventricular hypertrophy (15 mm, white line) and right ventricular hypertrophy. (b) Typical subepicardial late gadolinium enhancement in the inferolateral wall (arrow).

Figure 5

Hypereosinophilic syndrome. (a) Still four-chamber steady-state free precession cine in diastole shows increased left and right ventricular wall thickness (particularly at the apex) (white arrows), moderate left atrium dilatation, severe tricuspid regurgitation (not shown), right atrium (RA) dilatation and pericardial effusion (white star). (b) Inversion recovery early gadolinium image shows diffuse left and right subendocardial thrombi. These were confirmed histologically. (c) Inversion recovery late gadolinium image shows diffuse right and left subendocardial fibrosis.

Figure 6

Ischaemic cardiomyopathy in a patient with triple vessel disease. Inversion recovery images after gadolinium bolus show extensive transmural late gadolinium enhancement and apical thrombi in the left and right ventricles (arrows). (a, b) Four-chamber (left) and left ventricular outflow tract (right) views. (c–e) Short axis views.

Figure 7

Dilated cardiomyopathy. Note the dilated left ventricular cavity and poor systolic function. Late gadolinium enhancement reveals mid-wall hyperenhancement (arrows). The images are steady-state free precession cine in diastole (a) and systole (b) and inversion recovery after gadolinium bolus (c).

Figure 8

Arrhythmogenic right ventricular (RV) cardiomyopathy: note the dilated right ventricle (larger than the left ventricle) which had impaired systolic function on cine imaging. (a) Inversion recovery after gadolinium, four-chamber view, shows enhancement of the RV free wall. (b) Inversion recovery after gadolinium image confirms the RV free wall late gadolinium enhancement and shows left ventricular involvement with mid-ventricular inferolateral subepicardial enhancement. (c, d) T1 weighted (T1W) images without (c) and with (d) fat suppression from another patient with arrhythmogenic right ventricular cardiomyopathy shows fat infiltration in the right and left ventricles (arrows).

Figure 9

Pericardial constriction. (a) T1 weighted four-chamber view showing marked concentric pericardial thickening. Middle and right panels: still diastolic frames from real-time sequence during expiration (b) and deep inspiration (c). With inspiration, there is preferential right ventricular filling and consequent underfilling of the coupled left ventricle, resulting in flattening of the ventricular septum.

Figure 10

Single breath-hold spoiled gradient multi-echo T2 weighted sequence in a patient with thalassaemia major and severe cardiac iron loading. The left panel shows signal decay in the myocardium at different echo times (TE, in ms) that is due to iron overload. A region of interest is traced in the mid-septum and plotting of a curve of the signal intensity vs TE (right panel) allows calculation of cardiac T2* (in this case 7.4 ms) from the equation of the curve (the reciprocal of the exponent).

Figure 11

Left ventricular non-compaction. Steady-state free precession cine images in diastole. Note the dilated left ventricle (LV), which has impaired function, and the marked hypertrabeculation of both ventricles (arrowed). (a) Four-chamber view, (b) short axis view. RV, right ventricle.