ESC Congress 2011, Paris Imaging Unusual Causes of Cardiomyopathy Restrictive Cardiomyopathy Kazuaki Tanabe, MD, PhD Professor of Medicine Chair, Division of Cardiology Izumo, Japan
I Have No Disclosures
Cardiomyopathy Basic Categories Dilated Hypertrophic Restrictive
Restrictive Cardiomyopathy Etiology Idiopathic Infiltrative Disease: - Amyloidosis - Sarcoidosis - Hemochromatosis Post-radiation therapy Endocardial fibroelastosis
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
21 Years Old Man
21 Years Old Man
21 Years Old Man E A
21 Years Old Man S E A
Echocardiographic Assessment of Diastolic Function Oh JK, et al. Circ Cardiovasc Imaging. 2011;4:444-455
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 Idiopathic RCM
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
Idiopathic RCM Mayo Clinic 1979-1996 Ammash NM, et al. Circulation 2000;101:2490
73 Years Old Woman
73 Years Old Woman
Idiopathic RCM Mayo Clinic 1979-1996 Ammash NM, et al. Circulation 2000;101:2490
Idiopathic RCM Mayo Clinic 1979-1996 Male-sex NYHA class Congestion LAD>60mm Ammash NM, et al. Circulation 2000;101:2490
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
Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84
Amyloidosis Organ involvement and extent of deposition Dubrey SW, et al. Heart 2011;97:75-84
Cardiac Amyloidosis
AL Amyloidosis Klein AL, et al. Circulation 1991;83:808
Cardiac Amyloidosis Speckle Tracking Longitudinal Strain
HCM vs. Cardiac Amyloidosis Circumferential strain (%) Normal LVH HCM Amyloidosis Longitudinal strain (%) Normal LVH HCM Amyloidosis Sun JP, et al. Am J Cardiol 2009;103:411 415
Prognostic Significance of Strain Imaging in AL Amyloidosis All Death Cardiac Death Koyama J, et al. JACC Imaging 2010;3:333
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.
Cardiac Amyloidosis Cardiovascular Magnetic Resonance Maceira AM, et al. Circulation 2005;111:186 93
Cardiac Amyloidosis
Cardiac Amyloidosis
TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333
TDI, SRI and Strain in AL Amyloidosis Koyama J, et al. JACC Imaging 2010;3:333
RCM vs. Constrictive Pericarditis Garcia MJ, et al. JACC 1996;27:108 E >8.0 cm/s
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
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