Delayed Enhancement Magnetic Resonance Imaging Findings in Cardiac Amyloidosis

심장 아밀로이드증의 지연 조영증강 MR 영상소견

  • Song, Jin Hwa (Department of Radiology, Seoul National University Hospital) ;
  • Park, Eun-Ah (Department of Radiology, Seoul National University Hospital) ;
  • Lee, Whal (Department of Radiology, Seoul National University Hospital) ;
  • Chung, Jin Wook (Department of Radiology, Seoul National University Hospital) ;
  • Park, Jae Hyung (Department of Radiology, Seoul National University Hospital)
  • 송진화 (서울대병원 영상의학과) ;
  • 박은아 (서울대병원 영상의학과) ;
  • 이활 (서울대병원 영상의학과) ;
  • 정진욱 (서울대병원 영상의학과) ;
  • 박재형 (서울대병원 영상의학과)
  • Received : 2012.04.13
  • Accepted : 2012.12.27
  • Published : 2013.04.30

Abstract

Purpose : To evaluate late gadolinium enhancement (LGE) pattern of left ventricular (LV) myocardium and presence or absence of LGE in other regions of the heart on cardiac magnetic resonance (CMR) imaging in patients diagnosed with cardiac amyloidosis. Materials and Methods: From 2009 to 2011, 9 patients who were suspected cardiac amyloidosis underwent CMR. We retrospectively analyzed the presence or absence of LGE and enhancement pattern in LV myocardium, and the presence or absence of LGE in other chambers as well. Also we measured interatrial septal thickness (IST), relative signal intensities of atrial septum and epicardial fat over the left atrial (LA) cavity on delayed enhanced images. MRI parameters in these patients were compared to those of control group of patients with ischemic heart disease by Wilcoxon rank sum test. Results: Of nine patients, LGE were found in 8; subendocardial circumferential pattern in 4 and diffuse pattern in 4. LGE in right ventricle was observed in 7. IST was significantly increased in patients with cardiac amyloidosis (P = 0.02). Ratio of atrial septum to LA cavity and ratio of epicardial fat to LA cavity showed a significant difference (P = 0.0002 and P = 0.0006, respectively). Conclusion: In LGE CMR, subendocardial or diffuse enhancement pattern is a typical finding for patients with cardiac amyloidosis. Atrial septum and epicardial fat show relatively increased signal intensities over LA blood cavity.

목적 : 심장 아밀로이드증 환자의 심장 자기공명영상의 지연 조영증강영상에서 좌심근의 조영증강 패턴과 다른 심장부위의 조영증강 유무를 평가하고자 하였다. 대상 및 방법 : 2009년 9월부터 2011년 6월 사이 심장 아밀로이드증이 의심되어 심장 자기공명영상을 촬영한 9명의 환자를 후향적으로 분석하였다. 좌심근의 조영증강 여부 및 패턴, 그리고 좌우심방 및 우심실의 조영증강 유무를 평가하였다. 또한 심방중격 두께, 좌심방 혈액신호 강도에 대한 심방중격과 심외막 지방의 상대적 신호강도 비를 계산하여 허혈성 심질환 대조군 16명과 Wilcoxon rank sum test를 통해 통계적으로 비교분석 하였다. 결과 : 총 9명중 8명에서 좌심근 조영증강소견을 보였으며, 8명중 4명의 환자에서 심내막하 원형성, 나머지 4명에서 미만성 패턴을 보였다. 심방중격 두께는 심장 아밀로이드증 환자에서 허혈성 심질환 환자에 비해서 증가되어 있었고 (p = 0.02), 심장 아밀로이드증 환자군에서 심방중격 대 혈액 신호 강도비 (p = 0.0002) 및 심외막 지방 대 혈액 신호 강도비 (p = 0.0006) 모두 의미있게 높았다. 결론 : 좌심실의 심내막하 원형성 혹은 미만성 조영증강 패턴은 심장 아밀로이드증 환자의 지연 조영 심장 자기공명영상의 특징적인 소견이며, 심방중격 및 심외막 지방 대 혈액 신호 강도비 신호강도가 상대적으로 증가된다.

Keywords

References

  1. Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med 2003;349:583-596 https://doi.org/10.1056/NEJMra023144
  2. Smith TJ, Kyle RA, Lie JT. Clinical significance of histopathologic patterns of cardiac amyloidosis. Mayo Clin Proc 1984;59:547-555 https://doi.org/10.1016/S0025-6196(12)61493-1
  3. Rodney HF, Dubry SW. Amyloid heart disease. Prog Cardiovasc Dis 2010;52:347-361 https://doi.org/10.1016/j.pcad.2009.11.007
  4. Falk RH. Diagnosis and management of the cardiac amyloidosis. Circulation 2005;112:2047-2060 https://doi.org/10.1161/CIRCULATIONAHA.104.489187
  5. Pellikka PA, Holmes Jr DR, Edwards WD, et al. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med 1988;148:662-666 https://doi.org/10.1001/archinte.1988.00380030168027
  6. Yilmaz A, Kindermann I, Kindermann M, et al. Comparative evaluation of left and right ventricular endomyocardial biopsy: differences in complication rate and diagnostic performance. Circulation 2010;122:900-909 https://doi.org/10.1161/CIRCULATIONAHA.109.924167
  7. Perugini E, Rapezzi C, Piva T, et al. Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance. Heart 2006;92:343-349
  8. Syed IS, Glockner JF, DaLi F, et al. Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. J Am Coll Cardiol Cardiovasc Im 2010;3:155-164
  9. Vogelsberg H, Mahrholdt H, Deluigi CC, et al. Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy. J Am Coll Cardiol 2008;51:1022-1030 https://doi.org/10.1016/j.jacc.2007.10.049
  10. Austin BA, Wilson Tang WH, Rodriguez R, et al. Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis. J Am Coll Cardiol 2009;12:1369-1377
  11. Bellavia D, Pellikka PA, Abraham TP, et al. Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography. Am J Cardiol 2008;101:1039- 1045 https://doi.org/10.1016/j.amjcard.2007.11.047
  12. Cummings KW, Bhalla S, Javidan-Nejad C, Bierhals AJ, Gutierrez FR, Woodard PK. A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR Imaging. Radiographics 2009;29:89-103 https://doi.org/10.1148/rg.291085052
  13. Cerqueira MD, Weissman NJ, Diilsizian V, et al. Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart: a statement for healthcare professionals from the cardiac imaging committee of the council on clinical cardiology of the American heart association. Circulation 2002;105:539-542 https://doi.org/10.1161/hc0402.102975
  14. Maceira AM, Joshi J, Prasad SK, et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2005;111:186-193 https://doi.org/10.1161/01.CIR.0000152819.97857.9D
  15. Ruberg FL, Appelhaum E, Davidoff R, et al. Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-chain cardiac amyloidosis. Am J Cardiol 2009;103:544- 549 https://doi.org/10.1016/j.amjcard.2008.09.105
  16. Mekinian A, Lions C, Leleu X, et al. Prognosis assessment of cardiac involvement in systemic AL amyloidosis by magnetic resonance imaging. J Am Med 2010;123:864-868 https://doi.org/10.1016/j.amjmed.2010.03.022
  17. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT and Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 1981;63:188-196 https://doi.org/10.1161/01.CIR.63.1.188
  18. Di Bella G, Minutoli F, Mazzeo A, et al. MRI of cardiac involvement in transthyretin familial amyloid polyneuropathy. AJR Am J Roentgenol 2010;195:394-399 https://doi.org/10.2214/AJR.09.3721
  19. Berk JL, Keane J, Seldin DC. Persistent pleural effusions in primary systemic amyloidosis - etiology and prognosis. Chest 2003;124;969-977 https://doi.org/10.1378/chest.124.3.969
  20. Falk RH, Plehn JF, Deering T, et al. Sensitivity and specificity of the echocardiographic features of cardiac amyloidosis. Am J Cardiol 1987;59:418-422 https://doi.org/10.1016/0002-9149(87)90948-9
  21. Fattori R, Rocchi G, Celletti F, Bertaccini P, Rapezzi C, Gavelli G. Contribution of magnetic resonance imaging in the differential diagnosis of cardiac amyloidosis and symmetric hypertrophic cardiomyopathy. Am Heart J 1998;136:824-830 https://doi.org/10.1016/S0002-8703(98)70127-9
  22. Westermark P, Stenkvist B. A new method for the diagnosis of systemic amyloidosis. Arch Intern Med 1973;132:522-523 https://doi.org/10.1001/archinte.1973.03650100040007
  23. Migrino RQ, Christenson R, zabo A, Bright M, Truran S, Hari P. Prognostic implication of late gadolinium enhancement on cardiac MRI in light chain (AL) amyloidosis on long term follow up. BMC Med Phys 2009;9:5