Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

Implantation of a cardioverter-defibrillator in a patient with cardiac amyloidosis

Abstract

Background A 49-year-old woman presented with chest pain of 10 days' duration. Initial physical examinations and laboratory investigations were normal. The patient received symptomatic treatment with β-blockers, which continued following normal findings on coronary angiogram. About 7 months later the patient developed ventricular arrhythmias, with clinical evidence of left ventricular heart failure. Her arrhythmia symptoms persisted despite pharmacological therapy with atenolol, carvedilol and amiodarone.

Investigations Physical examination, electrocardiography, laboratory testing, serologic testing, exercise-tolerance testing, coronary angiography, chest radiography, cardiac MRI, tongue biopsy, bone-marrow biopsy, CT scan, iodine-123-labeled serum-amyloid-P-component scintigraphy.

Diagnosis Systemic primary amyloidosis (AL amyloidosis), with predominant cardiac involvement.

Management Pharmacological antiarrhythmic therapy and cardioverter-defibrillator implantation. Chemotherapy was planned but, despite intervention, the patient died before this treatment could begin.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Initial electrocardiogram of the patient at presentation with no signs of cardiac dysfunction.
Figure 2: An electrocardiogram of the patient showing evidence of infiltration and pseudo-infarction, with anterolateral Q waves and small QRS complexes in the standard leads.

References

  1. Falk R (2005) Diagnosis and management of the cardiac amyloidoses. Circulation 112: 2047–2060

    Article  Google Scholar 

  2. Falk R et al. (1997) The systemic amyloidoses. N Engl J Med 337: 898–909

    Article  CAS  Google Scholar 

  3. Kyle R et al. (1997) A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 336: 1202–1207

    Article  CAS  Google Scholar 

  4. Kholová I and Niessen H (2005) Amyloid in the cardiovascular system: a review. J Clin Pathol 58: 125–133

    Article  Google Scholar 

  5. Comenzo R and Gertz M (2002) Autologous stem cell transplantation for primary systemic amyloidosis. Blood 99: 4276–4282

    Article  CAS  Google Scholar 

  6. Falk R et al. (1984) Cardiac arrhythmias in systemic amyloidosis: correlation with echocardiographic abnormalities. J Am Coll Cardiol 3: 107–113

    Article  CAS  Google Scholar 

  7. Skadberg B et al. (1988) Sudden death caused by heart block in a patient with multiple myeloma and cardiac amyloidosis. Acta Med Scand 223: 379–383

    Article  CAS  Google Scholar 

  8. Dubery S et al. (2004) Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant 23: 1142–1153

    Article  Google Scholar 

  9. Falk R et al. (2000) Failure of the implantable defibrillator to prevent sudden death in cardiac amyloidosis [abstract #396]. Circulation 102 (Suppl 2)

  10. Hess E and White R (2004) Out-of-hospital cardiac arrest in patients with cardiac amyloidois: presenting rhythmns, management and outcomes in four patients. Resuscitation 60: 105–111

    Article  Google Scholar 

  11. Mercando A et al. (1988) Survival of patients with the automatic implantable cardioverter defibrillator. Pacing Clin Electrophysiol 11: 2059–2063

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ben LC Wright.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wright, B., Grace, A. & Goodman, H. Implantation of a cardioverter-defibrillator in a patient with cardiac amyloidosis. Nat Rev Cardiol 3, 110–114 (2006). https://doi.org/10.1038/ncpcardio0461

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpcardio0461

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing