Restrictive Cardiomyopathies: The Importance of Noninvasive Cardiac Imaging Modalities in Diagnosis and Treatment—A Systematic Review

Figures

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  Figure 1. Echocardiographic images from a patient with restrictive cardiomyopathy. Two-dimensional 4-chamber view in diastole (a) and systole (b), showing normal left ventricular volume, wall thickness, and systolic function (EF 62%). There is marked biatrial enlargement. Pulse wave Doppler from the left ventricular inflow (c) showing restrictive filling pattern with an E wave velocity of 1 m/sec, an A wave velocity of 0.4 m/sec, and an E wave deceleration time of 145 msec. Spectral tissue Doppler from the lateral (d) and septal (e) mitral annulus. There is marked reduction in systolic annular velocities indicative of latent systolic dysfunction. E/e′ (e′ measured as the average between the two annular e′ velocities) is 16, indicative of increased left ventricular filling pressure. Note the marked reduction in lateral a′ velocity (<4 cm/sec) indicative of left atrial systolic dysfunction.

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  Figure 2. ECG from a patient with cardiac amyloidosis. There are low voltage QRS complexes with left axis deviation and marked 1st-degree atrioventricular block.

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  Figure 3. Parasternal long (a) and short axis (b) view from the patient whose ECG appears in Figure 2. There is marked LV hypertrophy. Also note the granular, speckled myocardial appearance.

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  Figure 4. Strain curves from the interventricular septum of the same patient appearing in Figures 2 and 3. In this example, strain curves were constructed with the Doppler Myocardial Imaging technique. There is reduced peak systolic strain of the basal (yellow curve) and mid (green curve) septal segments, compared to the apical segment (red curve) with an apex to base ratio > 2.1.

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  Figure 5. CMR short axis (a) and four-chamber view (b). LGE images showing diffuse, nonhomogenous myocardial enhancement involving both ventricles and atria. The pattern of enhancement is consistent with cardiac amyloidosis. Note the presence of a right atrial thrombus. Images courtesy of Professor Dr. Jan Bogaert, University Hospital Leuven.

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  Figure 6. LGE images of a patient with pulmonary sarcoidosis with cardiac involvement. Four-chamber view (a) showing mid-wall focal enhancement in the lateral wall of the left ventricle and in the apex. Note also the enhancement of the mediastinal lymph nodes. Short axis view (b) demonstrating enhancement of the LVOT and possibly of the RVOT. Images courtesy of Professor Dr. Jan Bogaert, University Hospital Leuven.

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  Figure 7. Echocardiographic images from a patient with advanced Anderson-Fabry disease and end-stage renal failure. Parasternal long axis (a), short axis (b), and apical four-chamber view (c). There is left ventricular hypertrophy, more pronounced at the interventricular septum. There is also thickening of the right ventricular free wall apparent at the modified apical four-chamber view (d).