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Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

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Nội dung chi tiết: Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2ic or a systemic myocardial disorder in the absence of underlying atherosclerotic coronary artery disease. valvular disease, congenital heart disease,

or systemic hypertension, which is characterized by abnormal left ventricular fillinjL. and is associated with normal or reduced left ventricle (IV) Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

and right ventricle (RV) volumes and function.1 The term is not precise, but it incorporates infiltrative and fibrotic cardiac pathology. which are de

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

alt with in this chapter. While the majority of patients with infiltrative and fibrotic cardiomyopathies develop a restrictive filling pattern, especi

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2th other types of heart disease, such as dilated cardiomyopathy. In patients with dilated cardiomyopathy the restrictive filling pattern is often a re

versible phenomenon, related to worsening heart failure, and morphologically tlse ventricle is dilated, usually with severe reduction in ejection frac Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

tion. Although the clinical presentation of RCM may be similar to dilated cardiomyopathy, the nondilatcd. stiff ventricles often result in highly sodi

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

um-sensitive heart failure symptoms, associated in the late stage of the disease with a low cardiac output due to the small stroke volume. Because of

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2nt with symptoms of exercise intolerance.Diastolic dysfunction in the presence of preserved left ventricular ejection fraction (LVEF) is the key compo

nent of pathophysiology of R('.M. Initial stages of R('M demonstrate preserved LVEF with noncompliant walls that impair the normal diastolic filling o Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

f the ventricle. This restriction can be isolated to cither ventricle, or show biventricular involvement. Biventricular volumes arc cither normal or r

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

educed. Over a period of time, the chronically elevated IV diastolic pressure leads to increased atrial size, which may be considerable. Although seve

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2larly- if associated with long-standing atrial fibrillation. In later stages of the disease, as the compliance of the IV decreases, a small change in

LV volume is associated with a steep rise in IV pressure. A reduced ejection fraction may occur in the very late stages of the disease. It is importan Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

t to recognize that, although the left ventricle may show diastolic dysfunction with a normal election fraction. longitudinal systolic function may be

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

significantly impaired, and thus a normal ejection fraction should not be considered Q synonymous with normal systolic function (Videos 24.1 and 24.2

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2ers and storage disorders. Infiltrative disorders primarily affect the interstitial space of the myocardium. whereas storage diseases are associated w

ith deposits within the cardiac myocytes. In addition, endomyocardial involvement, leading to restriction, may occur in a variety- of uncommon conditi Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

ons (Box 24.1).Diagnosis of Restrictive CardiomyopathyDue to the varied pathophysiology and clinical manifestations of the underlying systemic process

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

, a systematic approach, beginning with a comprehensive history and detailed systemic evaluation, can help guidefurther management. Among patients wit

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2ial. ('.linical screening of first-degree relatives should be considered, and abnormalities, if present, may include hypertrophic and dilated cardiomy

opathy. Comprehensive genetic screening should also be considered, particularly if family members with suspicious cardiac abnormalities arc identified Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

.ECHOCARDIOGRAPHY IN RESTRICTIVE CARDIOMYOPATHY Cardiac imaging plays a pivotal role in establishing the diagnosis of RCM. Despite the availability of

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

multiple cardiac imaging options, including cardiac magnetic resonance (CMR) imaging and nuclear cardiology, echocardiography- remains the initial im

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2it can also provide vital dues to the diagnosis of the underlying etiology. The first step in cardiac assessment when interpreting an echocardiogram i

n suspected restrictive heart disease involves a thorough evaluation of the overall and regional anatomy of the left ventricle with regard to underlyi Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

ng wall thickness, altered myocardial texture, and wall motion abnormality, IV mass assessed by using three-dimensional (3D) echocardiogram is more re

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

producible, and mirrors the mass obtained by cardiac MR more closely. Similarly, while the quantitative assessment of overall left ventricular volumes

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2ejection fraction, when feasible and available, is encouraged since it docs not rely on underlying geometric assumptions leading to superior accuracy

and reproducibility. Nevertheless, two-dimensional (2D) echocardiography can give extremely useful diagnostic information, and five use of contrast fo Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

r better delineation of the endocardium when two or more contiguous IV endocardial segments are poorly visualized in apical view* improves accuracy an

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

d reduces interreader variability of IV functional analyses. In "primary" R( JM. ventricular wall thickness is usually normal, whereas the myocardium

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2 wall thickness and function, as involvement of right ventricle may have prognostic significance in a number of diseases.Doppler FeaturesDiastolic fun

ctional assessment of myocardium plays an important role in the diagnosis of RCM. In tlsc early stages of restrictive heart diseases, the myocardial r Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

elaxation (e") is reduced, resulting in septal C’ less than 7 cm/s and lateral e" less than 10 cm/s (11g. 21.1A and B). In early stages of the disease

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

, the mitral inflow pulse-wave IXippk-t shows an abnormal relaxation pattern, is characterized by an E/A ratio of <0.8. an increased mitral inflow E-w

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2l or mildly dilated in size, and the patient is rarely symptomatic. As this pattern is common in older patients in the general population, it is nondi

agnostic even in a gene-positive patient. With progression of disease, the mitral inflow pulse wave Doppler pattern shows pscudonormal filling pattern Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

, where the E/A ratio is 0.8-2. and this ratio reverses with Valsalva maneuver. Due to the elevated left ventricular245246(VEchocardiography fOR Disea

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

ses Of ĨHE Myocardiumtilling pressures. there is an increase of the E/e' ratio (a 10) and the left atrial volume index is elevated, 234 inlJm*. There

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2<1, while normal S/D is >1; see Fig. 24.1C and D). With further deterioration ofBOX 24.1 Cardiac Diseases Associated With Restrictive PathophysiologyP

rimary RCM•Idiopathic and familial RCM•Mitochondrial cardiomyopathyInfiltrative Diseases•Amyloidosis•Mucopolysaccharoidoses (Hurler syndrome. Gaucher Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

disease)Storage Diseases•Anderson-Fabry disease•Glycogen storage disorders•Hemochromatosis (may present with restrictive or, more commonly, dilated ph

Ebook Essential echocardiography - A companion to braunwald’s heart disease: Part 2

enotype)Endomyocardial Involvement•Endomyocardial fibrosis and Loftier endocarditis•Carcinoid syndrome•Postradiation

24Restrictive and InfiltrativeCardiomyopathiesVikram Agarwal, Rodney H. FalkINTRODUCTIONRestrictive cardiomyopathy (RCM) refers

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