Senile Systemic Amyloidosis: An Underdiagnosed Disease

  • Marta Catarino Manso Internal Medicine Department, Hospital de Egas Moniz, Lisbon
  • Diogo Paixão Marques Internal Medicine Department, Hospital de Egas Moniz, Lisbon
  • Sara L. Rocha Internal Medicine Department, Hospital de Egas Moniz, Lisbon
  • Simão C. Rodeia Emergency Department, Hospital Prof Doutor Fernando da Fonseca, Amadora
  • Raquel Domingos Internal Medicine Department, Hospital de Egas Moniz, Lisbon
Keywords: Cardiac amyloidosis, senile systemic amyloidosis, amyloidosis, heart failure with preserved ejection fraction.

Abstract

Senile systemic amyloidosis is caused by a non-mutated form of transthyretin with the heart being the major organ involved. This infiltrative cardiomyopathy usually presents as slowly progressive heart failure.
An 82-year-old female patient was admitted for newly diagnosed heart failure. A year later she presented with decompensated heart failure and syncope. Inpatient work-up showed persistently elevated troponin and N-terminal-pro BNP levels, rapid progression to severe left ventricular concentric hypertrophy, and sinus pauses on the Holter. Cardiac MRI revealed diffuse late gadolinium enhancement in the left ventricle. The demonstration of amyloid protein with the clinical findings and complementary investigations allowed for the diagnosis of senile systemic amyloidosis.

References

  • Mohty D, Damy T, Cosnay P, et al. Cardiac amyloidosis: updates in diagnosis and management. Arch Cardiovasc Dis 2013;106:528–240.

  • Fikrle M, Paleček T, Kuchynka P, et al. Cardiac amyloidosis: a comprehensive review. Cor et Vasa 2013;55:160–175.

  • Esplin BL, Gertz MA. Current trends in diagnosis and management of cardiac amyloidosis. Curr Prob Cardiol 2013;38:53–96.

  • Aljaroudi W, Desai MY, Tang WH, et al. Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: state of the art review and focus on emerging nuclear techniques. J Nucl Cardiol 2014;21:271–283.

  • Fine N, Arruda-Olson AM, Dispenieri A, et al. Yield of noncardiac biopsy for the diagnosis of transthyretin cardiac amyloidosis. Am J Cardiol 2014;113:1723–1727.
  • Published: 2017-09-21

    Issue: Vol 4 No 9 (view)

    Section: Articles

    How to cite:
    Manso, M., Paixão Marques, D., Rocha, S., Rodeia, S., & Domingos, R. (2017). Senile Systemic Amyloidosis: An Underdiagnosed Disease. European Journal of Case Reports in Internal Medicine, 4(9). https://doi.org/https://doi.org/10.12890/2017_000725