Multimodality Cardiac Imaging in Cardiomyopathies: From Diagnosis to Prognosis

Abstract

Cardiomyopathies are a group of structural and/or functional myocardial disorders which encompasses hypertrophic, dilated, arrhythmogenic, restrictive, and other cardiomyopathies. Multimodality cardiac imaging techniques are the cornerstone of cardiomyopathy diagnosis; transthoracic echocardiography should be the first-line imaging modality due to its availability, and diagnosis should be confirmed by cardiovascular magnetic resonance, which will provide more accurate morphologic and functional information, as well as extensive tissue characterization. Multimodality cardiac imaging techniques are also essential in assessing the prognosis of patients with cardiomyopathies; left ventricular ejection fraction and late gadolinium enhancement are two of the main variables used for risk stratification, and they are incorporated into clinical practice guidelines. Finally, periodic testing with cardiac imaging techniques should also be performed due to the evolving and progressive natural history of most cardiomyopathies.

Keywords: cardiomyopathy; diagnosis; late gadolinium enhancement; left ventricular ejection fraction; multimodality cardiac imaging techniques; prognosis.

Figure 1

A parasternal long-axis view of a patient with septal HCM. Note the marked increase in septal wall thickness and the asymmetry compared to the posterior wall.

Figure 2

Different hypertrophic cardiomyopathy phenotypes. (A) Septal HCM. (B) Apical HCM. (C) Diffuse HCM.

Figure 3

Ancillary signs in HCM. (A) Myocardial crypts in the inferior wall. (B) Apical aneurysm in a patient with apical HCM. (C) Apical insertion of the papillary muscles in a patient with septal HCM. (D) Outflow tract obstruction measured on Doppler CW, note the dagger-shaped curve. (E) SAM evidenced on M-mode: see the movement of the anterior mitral leaflet towards the septum in systole.

Figure 4

CMR study of a patient with septal HCM. (A) Maximal wall thickness measured on the short-axis cine stack at end-diastole. (B) Post-contrast T1 sequences showing extensive basal and mid-anteroseptal LGE with an intramyocardial pattern.

Figure 5

CMR study of a patient with septal HCM. (A,B) LGE sequences with heterogeneous contrast uptake in the basal and mid-anteroseptal segments. (C) Native T1 mapping sequence with an increased native T1 value of 1108 ms in the basal anteroseptal segment (reference range 950–1050 ms).

Figure 6

Stress echocardiography in a patient with HCM. Note the marked increase in SAM (B, white arrow) and the significant increase in LVOTO (D) at peak stress compared to the basal situation (A,C).

Figure 7

Transthoracic echocardiography of a patient with dilated cardiomyopathy, showing a parasternal long-axis view (A) and an apical 4-chambers view (B). Note the marked dilatation of the left ventricle and the spherical pattern.

Figure 8

Three-dimensional transthoracic echocardiography of the left ventricle. (A) Triplane view of the LV allows simultaneous and single-beat acquisition of the three apical views. By tracing the endocardial borders, the LV volume is obtained (surface rendering, bottom right). (B) Real-time single-beat 3D acquisition of the LV from the apical window. Volume renders allow for offline reconstructions. (C) Tomographic multislice obtained from a multiple-beat apical view. Wall motion abnormalities can be assessed with this technique.

Figure 9

Longitudinal strain analysis of a patient with DCM: 4 chambers (A), 2 chambers (B), 3 chambers (C), and bull’s eye plot (D). Note the diffusely affected longitudinal strain, consistent with depressed LVEF, and positive values in the basal septum suggestive of dyskinesia in these segments.

Figure 10

Myocardial work analysis in a patient with DCM. (A) Strain—pressure loop. (B) Comparison of constructive work (green) and wasted work (blue). (C) Bull’s eye plot of myocardial work index (mmHg%). (D) Bull’s eye plot of myocardial work efficiency (%).

Figure 11

CMR study of a patient with DCM. (A) Short-axis cine stack. (B,C) Post-contrast T1 sequences showing a typical LGE pattern with a lineal mid-septum uptake as well as a focal uptake in the septum-free wall inferior junction.

Figure 12

Strain analysis by CMR obtained with feature-tracking software in a patient with DCM. Endocardial and epicardial segmentation of short and long-axis is required, both at end-diastole and end-systole. Thus, longitudinal (A), radial (B), and circumferential (C) strain are simultaneously acquired.

Figure 13

Three-dimensional transthoracic echocardiography of the right ventricle. (A) By tracing the endocardial RV borders (top and bottom left), a 3D volume of the RV (top right) is obtained throughout the cardiac cycle (bottom right) and 3D RVEF is calculated. Note the RV inflow and outflow tract in the 3D model. (B) Real-time single-beat 3D acquisition of the RV from the dedicated apical window. (C) Tomographic multislice obtained from a multiple-beat apical view.

Figure 14

Apical 4-chamber (A), 3-chamber (B), and 2-chamber (C) views in a patient with cardiac amyloidosis (left ventricular wall thickness and sparkling). On the bull’s eye strain images (D), the apical sparing pattern is displayed (right image).

Figure 15

CMR findings in a patient with CA: left ventricular concentric hypertrophy (left), increased native T1 mapping values (red color in the central image), and diffuse LGE (right image).

Figure 16

Echocardiographic signs in Fabry disease. (A) Binary septum (arrows), (B) concentric hypertrophy, and inferolateral fibrosis (*), (C) abnormal longitudinal strain more pronounced in the inferolateral wall (blue area) correlated with the fibrosis.

Figure 17

Late gadolinium enhancement pattern in a patient with Fabry disease. (A) Presence of LGE in the inferolateral wall. (B) Evolution of the same patient 5 years later, the presence of a thinning of the wall, a more extensive and transmural LGE pattern is observed.

Figure 18

CMR findings in a patient with Fabry disease: (Left panel) presence of concentric left ventricular hypertrophy with no LGE. (Central panel) T1 mapping image of the same patient. (Right panel) bull’s eye representation of the T1 mapping showing a short native T1 in the septum (glycosphingolipid accumulation) and a long T1 value in the inferolateral wall (diffuse fibrosis). T1 mapping abnormalities appear earlier than LGE.

Figure 19

(A) Patchy LGE distribution in the septum (arrows) and (B) subepicardial LGE (arrows) in a patient with sarcoidosis. (C) 18F-FDG uptake in a patient with sarcoidosis (arrow). (D) Perfusion-metabolism mismatch in a patient with sarcoidosis (perfusion defect in the upper image, metabolism uptake in the mid image, and mismatch in the lower image).

Figure 20

Transthoracic echocardiography (A,B) and cardiac magnetic resonance (C,D) of patients with endomyocardial fibrosis. Note the marked endocardial thickening of the mid and apical segments and apical obliteration of the left ventricle (A,B), the apical thrombus (C,D), and the endomyocardial fibrosis on LGE sequences (D).

Figure 21

Transthoracic echocardiography of a patient with right ventricular arrhythmogenic cardiomyopathy. (A) RV dedicated apical 4-chamber view shows severe dilatation of the RV. RV dedicated apical 4-chamber view (B) and subcostal view (C) show the presence of aneurysms in the RV free wall (arrow).

Figure 22

CMR findings in a patient with ACM. (A) RV aneurysms (arrows) in cine images. (B) Fibrofatty infiltration in T1-weighted turbo spin-echo sequences (arrow). (C) Right ventricular enlargement and left ventricular wall thinning (subepicardial fatty infiltration) (arrows) in a 3-chamber view cine. (D) Subepicardial LGE (arrows) and (E) subepicardial annular (arrows) patterns.

Figure 23

TTE (A) and CMR (B) images of patients with left ventricular noncompaction showing marked hypertrabeculation and deep intertrabecular recesses.

Figure 24

TTE parasternal short-axis mid-ventricular view of a patient with LVNC (A). Measurement of the compacted and non-compacted layers at end-diastole after echocontrast administration, fulfilling LVNC diagnostic criteria (B).

Figure 25

CMR 4-chambers view of a patient with LVNC (A). Measurement of the compacted and non-compacted layers at end-diastole on a long-axis view (Petersen criteria, B). Measurement of the trabeculated mass on a short-axis view (Jacquier criteria, C).