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Restrictive Cardiomyopathy

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Part of the book series: Clinical Cases in Cardiology ((CCC))

Abstract

A 57 year-old female with a history of nonischemic cardiomyopathy was referred for management of heart failure. Five months ago, the patient was evaluated for 2 months of fevers, dyspnea on exertion and lower extremity edema. A transthoracic echocardiogram (TTE) was completed which revealed a left ventricular ejection fraction (LVEF) of 20% with global hypokinesis. A nuclear stress test was normal. A repeat TTE 1 month later showed improved LVEF to 35–40%.

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References

  1. Banypersad SM, Moon JC, Whelan C, Hawkins PN, Wechalekar AD. Updates in cardiac amyloidosis: a review. J Am Heart Assoc. 2012;1:e000364.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Gertz MA, Dispenzieri A, Sher T. Pathophysiology and treatment of cardiac amyloidosis. Nat Rev Cardiol. 2015;12:91–102.

    Article  CAS  PubMed  Google Scholar 

  3. Castaño A, Drachman BM, Judge D, Maurer MS. Natural history and therapy of TTR-cardiac amyloidosis: emerging disease-modifying therapies from organ transplantation to stabilizer and silencer drugs. Heart Fail Rev. 2015;20:163–78.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Dispenzieri A, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol Off J Am Soc Clin Oncol. 2004;22:3751–7.

    Article  CAS  Google Scholar 

  5. Dubrey SW, Comenzo RL. Amyloid diseases of the heart: current and future therapies. QJM. 2012;105:617–31.

    Article  CAS  PubMed  Google Scholar 

  6. Bokhari S, Castano A, Pozniakoff T, Deslisle S, Latif F, Maurer MS. 99mTc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses. Circ Cardiovasc Imaging. 2013;6(2):195–201.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Phelan D, Collier P, Thavendiranathan P, Popovic ZB, Hanna M, Plana JC, et al. Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart. 2012;98(19):1442–8.

    Article  PubMed  Google Scholar 

  8. Phelan D, Thavendiranathan P, Popovic Z, Collier P, Griffin B, Thomas JD, et al. Application of a parametric display of two-dimensional speckle-tracking longitudinal strain to improve the etiologic diagnosis of mild to moderate left ventricular hypertrophy. J Am Soc Echocardiogr. 2014;27(8):888–95.

    Article  PubMed  Google Scholar 

  9. Koyama J, Ikeda S, Ikeda U. Echocardiographic assessment of the cardiac amyloidoses. Circ J. 2015;79:721–34.

    Article  PubMed  Google Scholar 

  10. Estep JD, et al. Heart transplantation and end-stage cardiac amyloidosis: a review and approach to evaluation and management. Methodist Debakey Cardiovasc J. 2012;8:8–16.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Fikrle M, et al. The diagnostic performance of cardiac magnetic resonance in detection of myocardial involvement in AL amyloidosis. Clin Physiol Funct Imaging. 2014;36:218. https://doi.org/10.1111/cpf.12216.

    Article  PubMed  Google Scholar 

  12. Falk RH. Cardiac amyloidosis: a treatable disease, often overlooked. Circulation. 2011;124:1079–85.

    Article  PubMed  Google Scholar 

  13. Varr BC, et al. Implantable cardioverter-defibrillator placement in patients with cardiac amyloidosis. Heart Rhythm. 2014;11:158–62.

    Article  PubMed  Google Scholar 

  14. Sanchorawala V. Light-chain (AL) amyloidosis: diagnosis and treatment. Clin J Am Soc Nephrol. 2006;1:1331–41.

    Article  PubMed  Google Scholar 

  15. Wechalekar AD, et al. Guidelines on the management of AL amyloidosis. Br J Haematol. 2015;168:186–206.

    Article  PubMed  Google Scholar 

  16. Gertz MA, et al. First-in-human phase I/II study of NEOD001 in patients with light chain amyloidosis and persistent organ dysfunction. J Clin Oncol Off J Am Soc Clin Oncol. 2016;34:1097. https://doi.org/10.1200/JCO.2015.63.6530.

    Article  CAS  Google Scholar 

  17. Wechalekar AD, Gillmore JD, Hawkins PN. Systemic amyloidosis. Lancet. 2015;387:2641. https://doi.org/10.1016/s0140-6736(15)01274-x.

    Article  PubMed  Google Scholar 

  18. Herlenius G, Wilczek HE, Larsson M, Ericzon B-G, Familial Amyloidotic Polyneuropathy World Transplant Registry. Ten years of international experience with liver transplantation for familial amyloidotic polyneuropathy: results from the Familial Amyloidotic Polyneuropathy World Transplant Registry. Transplantation. 2004;77:64–71.

    Article  PubMed  Google Scholar 

  19. Maurer MS, et al. Tafamidis in transthyretin amyloid cardiomyopathy: effects on transthyretin stabilization and clinical outcomes. Circ Heart Fail. 2015;8:519–26.

    Article  PubMed  Google Scholar 

  20. Berk JL, et al. Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial. JAMA. 2013;310:2658–67.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Coelho T, et al. Safety and efficacy of RNAi therapy for transthyretin amyloidosis. N Engl J Med. 2013;369:819–29.

    Article  CAS  PubMed  Google Scholar 

  22. Swiecicki PL, et al. Left ventricular device implantation for advanced cardiac amyloidosis. J Heart Lung Transplant. 2013;32:563–8.

    Article  PubMed  Google Scholar 

  23. Grupper A, et al. Role of ventricular assist therapy for patients with heart failure and restrictive physiology: improving outcomes for a lethal disease. J Heart Lung Transplant. 2015;34:1042–9.

    Article  PubMed  Google Scholar 

  24. Dubrey SW, Burke MM, Hawkins PN, Banner NR. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant. 2004;23:1142–53.

    Article  PubMed  Google Scholar 

  25. Lacy MQ, et al. Autologous stem cell transplant after heart transplant for light chain (Al) amyloid cardiomyopathy. J Heart Lung Transplant. 2008;27:823–9.

    Article  PubMed  Google Scholar 

  26. Dey BR, et al. Cardiac transplantation followed by dose-intensive melphalan and autologous stem-cell transplantation for light chain amyloidosis and heart failure. Transplantation. 2010;90:905–11.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Varr BC, et al. Heart transplantation and cardiac amyloidosis: approach to screening and novel management strategies. J Heart Lung Transplant. 2012;31:325–31.

    Article  PubMed  Google Scholar 

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Correspondence to Amanda R. Vest .

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Chokshi, A., Vest, A.R. (2018). Restrictive Cardiomyopathy. In: Shah, R., Abbasi, S. (eds) Clinical Cases in Heart Failure. Clinical Cases in Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-319-65804-9_2

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  • DOI: https://doi.org/10.1007/978-3-319-65804-9_2

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-65803-2

  • Online ISBN: 978-3-319-65804-9

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