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ESC Congress 2011, Paris Imaging Unusual Causes of Cardiomyopathy Restrictive Cardiomyopathy Kazuaki Tanabe, MD, PhD Professor of Medicine Chair, Division of Cardiology Shimane University Faculty of Medicine Izumo, Japan I Have No Disclosures Cardiomyopathy “Basic Categories” • Dilated • Hypertrophic • Restrictive Shimane University Faculty of Medicine Restrictive Cardiomyopathy “Etiology” • Idiopathic • Infiltrative Disease: - Amyloidosis - Sarcoidosis - Hemochromatosis • Post-radiation therapy • Endocardial fibroelastosis Shimane University Faculty of Medicine 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 Shimane University Faculty of Medicine 21 Years Old Man Shimane University Faculty of Medicine 21 Years Old Man Shimane University Faculty of Medicine 21 Years Old Man E A Shimane University Faculty of Medicine 21 Years Old Man S’ E’ A’ Shimane University Faculty of Medicine Echocardiographic Assessment of Diastolic Function Oh JK, et al. Circ Cardiovasc Imaging. 2011;4:444-455 Shimane University Faculty of Medicine Idiopathic RCM Mayo Clinic 1979-1996 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 Shimane University Faculty of Medicine Idiopathic RCM Mayo Clinic 1979-1996 • 94 patients (61% women) • 10-90 years old (mean, 64 years) • 28% in NYHA III/IV • 74% in Af • 16% in systolic dysfunction Echocardiographic characteristics LA diameter, mm 40-82 (50±9) LA volume, mL 62-476 (142±76) LVEDD, mm 30-55 (47±6) LVEF, % 18-80 (59±13) Ammash NM, et al. Circulation 2000;101:2490 Shimane University Faculty of Medicine Idiopathic RCM Mayo Clinic 1979-1996 Ammash NM, et al. Circulation 2000;101:2490 Shimane University Faculty of Medicine 73 Years Old Woman Shimane University Faculty of Medicine 73 Years Old Woman Shimane University Faculty of Medicine Idiopathic RCM Mayo Clinic 1979-1996 Ammash NM, et al. Circulation 2000;101:2490 Shimane University Faculty of Medicine Idiopathic RCM Mayo Clinic 1979-1996 Male-sex Congestion Ammash NM, et al. Circulation 2000;101:2490 NYHA class LAD>60mm Shimane University Faculty of Medicine 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 Shimane University Faculty of Medicine Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84 Shimane University Faculty of Medicine Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84 Shimane University Faculty of Medicine Cardiac Amyloidosis Shimane University Faculty of Medicine AL Amyloidosis Klein AL, et al. Circulation 1991;83:808 Shimane University Faculty of Medicine Cardiac Amyloidosis Speckle Tracking Longitudinal Strain Shimane University Faculty of Medicine HCM vs. Cardiac Amyloidosis Circumferential strain (%) Normal LVH HCM Amyloidosis LVH HCM Amyloidosis Longitudinal strain (%) Normal Sun JP, et al. Am J Cardiol 2009;103:411– 415 Shimane University Faculty of Medicine Prognostic Significance of Strain Imaging in AL Amyloidosis All Death Cardiac Death Koyama J, et al. JACC Imaging 2010;3:333 Shimane University Faculty of Medicine 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. Shimane University Faculty of Medicine Shimane University Faculty of Medicine Cardiac Amyloidosis Cardiovascular Magnetic Resonance Maceira AM, et al. Circulation 2005;111:186 –93 Shimane University Faculty of Medicine Cardiac Amyloidosis Cardiac Amyloidosis TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333 Shimane University Faculty of Medicine TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333 Shimane University Faculty of Medicine RCM vs. Constrictive Pericarditis E’ >8.0 cm/s Garcia MJ, et al. JACC 1996;27:108 Shimane University Faculty of Medicine 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 Shimane University Faculty of Medicine RCM vs. Constrictive Pericarditis Sengupta PP, et al. J Am Coll Cardiol Img 2008;1:29–38 Contemporary Definitions and Classification of the Cardiomyopathies Maron BJ, et al. Circulation 2006;113:1807-1816
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