Restrictive Cardiomyopathy
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1 ESC Congress 2011, Paris Imaging Unusual Causes of Cardiomyopathy Restrictive Cardiomyopathy Kazuaki Tanabe, MD, PhD Professor of Medicine Chair, Division of Cardiology Izumo, Japan
2 I Have No Disclosures
3 Cardiomyopathy Basic Categories Dilated Hypertrophic Restrictive
4 Restrictive Cardiomyopathy Etiology Idiopathic Infiltrative Disease: - Amyloidosis - Sarcoidosis - Hemochromatosis Post-radiation therapy Endocardial fibroelastosis
5 Idiopathic RCM Characteristics Primary disease of the myocardium Increased resistance to ventricular filling Increased myocardial stiffness (decreased ventricular compliance): - Elevated ventricular diastolic pressure - Increased atrial pressure Global systolic function may be preserved Conduction abnormalities
6 21 Years Old Man
7 21 Years Old Man
8 21 Years Old Man E A
9 21 Years Old Man S E A
10 Echocardiographic Assessment of Diastolic Function Oh JK, et al. Circ Cardiovasc Imaging. 2011;4:
11 Mayo Clinic Echocardiographic criteria: Biatrial enlargement Nondilated ventricles Normal wall thickness Exclusion criteria: - IHD, HT(>5 years), VHD, congenital, - pericardial disease, carcinoid syndrome, - connective tissue disease, amyloidosis, - hemochromatosis, eosiophilic syndrome, - malignancy, radiation, alcohol abuse, - cardiotoxic drugs Ammash NM, et al. Circulation 2000;101:2490 Idiopathic RCM
12 Idiopathic RCM Mayo Clinic patients (61% women) years old (mean, 64 years) 28% in NYHA III/IV 74% in Af 16% in systolic dysfunction Echocardiographic characteristics LA diameter, mm (50±9) LA volume, ml (142±76) LVEDD, mm (47±6) LVEF, % (59±13) Ammash NM, et al. Circulation 2000;101:2490
13 Idiopathic RCM Mayo Clinic Ammash NM, et al. Circulation 2000;101:2490
14 73 Years Old Woman
15 73 Years Old Woman
16 Idiopathic RCM Mayo Clinic Ammash NM, et al. Circulation 2000;101:2490
17 Idiopathic RCM Mayo Clinic Male-sex NYHA class Congestion LAD>60mm Ammash NM, et al. Circulation 2000;101:2490
18
19 Cardiac Amyloidosis Increased LV wall thickness Increased RV wall thickness Small, well, or poorly contracting LV Valve thickening Mitral regurgitation Thickened atrial septum E/A ratio >1 Pericardial effusion
20 Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84
21 Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84
22 Cardiac Amyloidosis
23 AL Amyloidosis Klein AL, et al. Circulation 1991;83:808
24 Cardiac Amyloidosis Speckle Tracking Longitudinal Strain
25 HCM vs. Cardiac Amyloidosis Circumferential strain (%) Normal LVH HCM Amyloidosis Longitudinal strain (%) Normal LVH HCM Amyloidosis Sun JP, et al. Am J Cardiol 2009;103:
26 Prognostic Significance of Strain Imaging in AL Amyloidosis All Death Cardiac Death Koyama J, et al. JACC Imaging 2010;3:333
27 Summary The presence of dilated atria with nonhypertrophied, nondilated ventricles in patients with congestive heart failure should raise the suspicion of RCM. Making an early diagnosis of amyloidosis is critical because, once clinically significant heart disease is present, the prognosis is poor. Strain imaging is sensitive to demonstrate the impairment of longitudinal contraction despite apparently preserved ejection fraction and may have potential for evaluating the prognosis in cardiac amyloidosis.
28
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30 Cardiac Amyloidosis Cardiovascular Magnetic Resonance Maceira AM, et al. Circulation 2005;111:186 93
31 Cardiac Amyloidosis
32 Cardiac Amyloidosis
33 TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333
34 TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333
35 RCM vs. Constrictive Pericarditis Garcia MJ, et al. JACC 1996;27:108 E >8.0 cm/s
36 Myocardial Deformations and Twist Control DHF SHF LV twist Circumferential strain Longitudinal strain Radial strain Wang J, et al: Eur Heart J 2008;29:1283
37 RCM vs. Constrictive Pericarditis Sengupta PP, et al. J Am Coll Cardiol Img 2008;1:29 38
38 Contemporary Definitions and Classification of the Cardiomyopathies Maron BJ, et al. Circulation 2006;113:
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