Relative to dilated and hypertrophic cardiomyopathies, restrictive cardiomyopathy (RCM) happens at a low frequency in the developed world. Specific forms of RCM such as endomyocardial disease are important causes of morbidity and mortality common in specific geographic region, particularly in underdeveloped countries. The pathophysiologic feature that defines RCM is increased stiffness in ventricular walls, which causes heart failure due to impaired ventricular filling. In the early stages of the syndrome, the systolic function may be normal, though deterioration in the systolic function is generally observed as the disease progresses.
Early in the disease, the left ventricular (LV) systolic function is typically preserved, while the LV diastolic function is abnormal, causing an increased atrial pressure and marked bi-atrial enlargement. The characteristic features of primary RCM on 2D echocardiography include a normal ventricular cavity size, normal wall thicknesses, and usually a preserved global systolic function with bi-atrial enlargement. RCM is accompanied by restrictive diastolic filling; this is, however, a nonspecific finding. The typical LV diastolic pressure tracing in RCM is the dip and plateau or square root configuration, which corresponds to a shortened deceleration time of the mitral inflow E velocity. The increase in the left atrial pressure (a) causes the mitral valve to open at a higher pressure, reducing the isovolumic relaxation time; (b) increases the transmitral pressure gradient with a concomitant rise in the mitral E velocity; and (c) decreases the pulmonary venous flow velocity during systole and increases it during diastole. With a high LV end-diastolic pressure, atrial contribution to ventricular filling is minimal and A velocity is usually decreased, causing the E/A ratio to increase markedly (>2). In contrast to constrictive pericarditis, the hepatic vein diastolic flow reversal in RCM is remarkable during inspiration. Myocardial relaxation is universally impaired, so that the mitral annulus E′ velocity obtained from the septal annulus is frequently less than 7 cm/s.
KeywordsRestrictive cardiomyopathy Echocardiography Diastolic pressure Constrictive pericarditis Myocardial relaxation Endomyocardial fibrosis
- Idiopathic RCM
Idiopathic restrictive cardiomyopathy
Isovolumic relaxation time
Cardiomyopathies, restrictive cardiomyopathy
(Related to Fig. 23.1): Typical echocardiography finding in Amyloidosis. Concentric increased myocardial thickness in apical 4 chamber and parasternal short axis views (AVI 13084 kb)
(Related to Fig. 23.1): Typical echocardiography finding in Amyloidosis. Concentric increased myocardial thickness in apical 4 chamber and parasternal short axis views (AVI 2913 kb)
(Related to Fig. 23.3): Loffler syndrome, Dilated atria, small pericardial effusion and thickened obliterate apex (AVI 10423 kb)
(Related to Fig. 23.3): Loffler syndrome, Dilated atria, small pericardial effusion and thickened obliterate apex (AVI 6816 kb)
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