Journal of the American Society of Echocardiography
Volume 23, Issue 6 , Pages 643-652 , June 2010

Independent Predictors of Survival in Primary Systemic (AL) Amyloidosis, Including Cardiac Biomarkers and Left Ventricular Strain Imaging: An Observational Cohort Study

  • Diego Bellavia, MD, PhD, MS

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Patricia A. Pellikka, MD

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Ghormallah B. Al-Zahrani, MBBS

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Theodore P. Abraham, MD

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Angela Dispenzieri, MD

      Affiliations

    • Department of Hematology, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Chinami Miyazaki, MD

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Martha Lacy, MD

      Affiliations

    • Department of Hematology, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Christopher G. Scott, MS

      Affiliations

    • Department of Biostatistics, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Jae K. Oh, MD

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • ,
  • Fletcher A. Miller Jr., MD

      Affiliations

    • Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota
    • Corresponding Author InformationReprint requests: Fletcher A. Miller, Jr, MD, Mayo Clinic and Foundation, Division of Cardiovascular Disease, 200 First Street SW, E-16-B, Rochester, MN 55905.

References 

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  2. Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, et al. Prognostication of survival using cardiac troponins and N-terminal pro-brain natriuretic peptide in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood. 2004;104:1881–1887
  3. Dispenzieri A, Lacy MQ, Katzmann JA, Rajkumar SV, Abraham RS, Hayman SR, et al. Absolute values of immunoglobulin free light chains are prognostic in patients with primary systemic amyloidosis undergoing peripheral blood stem cell transplantation. Blood. 2006;107:3378–3383
  4. Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation. 2003;107:2446–2452
  5. Bellavia D, Pellikka PA, Abraham TP, Al-Zahrani GB, Dispenzieri A, Oh JK, 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
  6. Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN, et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18-22 April 2004. Am J Hematol 2005;79:319-28.
  7. Bellavia D, Pellikka PA, Abraham TP, Al-Zahrani G, Dispenzieri A, Oh J, et al. “Hypersynchronization” by tissue velocity imaging in patients with cardiac amyloidosis. Heart. 2009;95:234–240
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  11. Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med. 2004;140:85–93
  12. Austin BA, Duffy B, Tan C, Rodriguez ER, Starling RC, Desai MY. Comparison of functional status, electrocardiographic, and echocardiographic parameters to mortality in endomyocardial-biopsy proven cardiac amyloidosis. Am J Cardiol. 2009;103:1429–1433
  13. Berk JL, Keane J, Seldin DC, Sanchorawala V, Koyama J, Dember LM, et al. Persistent pleural effusions in primary systemic amyloidosis: etiology and prognosis. Chest. 2003;124:969–977
  14. Klein AL, Hatle LK, Taliercio CP, Oh JK, Kyle RA, Gertz MA, et al. Prognostic significance of Doppler measures of diastolic function in cardiac amyloidosis. A Doppler echocardiography study. Circulation. 1991;83:808–816
  15. Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009;120:1203–1212
  16. Bursi F, Weston SA, Redfield MM, Jacobsen SJ, Pakhomov S, Nkomo VT, et al. Systolic and diastolic heart failure in the community. JAMA. 2006;296:2209–2216
  17. Migrino RQ, Mareedu RK, Eastwood D, Bowers M, Harmann L, Hari P. Left ventricular ejection time on echocardiography predicts long-term mortality in light chain amyloidosis. J Am Soc Echocardiogr. 2009;22:1396–1402
  18. Al-Zahrani GB, Bellavia D, Pellikka PA, Dispenzieri A, Hayman SR, Oh JK, et al. Doppler myocardial imaging compared to standard two-dimensional and Doppler echocardiography for assessment of diastolic function in patients with systemic amyloidosis. J Am Soc Echocardiogr. 2009;22:290–298
  19. Bellavia D, Abraham TP, Pellikka PA, Al-Zahrani GB, Dispenzieri A, Oh JK, et al. Detection of left ventricular systolic dysfunction in cardiac amyloidosis with strain rate echocardiography. J Am Soc Echocardiogr. 2007;20:1194–1202
  20. Comenzo RL, Vosburgh E, Falk RH, Sanchorawala V, Reisinger J, Dubrey S, et al. Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients. Blood. 1998;91:3662–3670
  21. Dispenzieri A, Lacy MQ, Kyle RA, Therneau TM, Larson DR, Rajkumar SV, et al. Eligibility for hematopoietic stem-cell transplantation for primary systemic amyloidosis is a favorable prognostic factor for survival. J Clin Oncol. 2001;19:3350–3356

PII: S0894-7317(10)00274-9

doi: 10.1016/j.echo.2010.03.027

Journal of the American Society of Echocardiography
Volume 23, Issue 6 , Pages 643-652 , June 2010

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