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Transthyretin amyloidosis in aortic stenosis: clinical and therapeutic implications

Gioele Fabbri1, Matteo Serenelli1, Anna Cantone1

  • 1Centro Cardiologico, Università di Ferrara, via Aldo Moro 8, Cona, 44124 Ferrara, Italy.

European Heart Journal Supplements : Journal of the European Society of Cardiology
|October 15, 2021
PubMed

Insights

About 14% of elderly patients with severe aortic stenosis also have transthyretin amyloid cardiomyopathy. This association may stem from valve infiltration triggering calcification or aortic stenosis-induced strain promoting amyloidosis. Valve replacement is viable for these patients.

Area of Science:

  • Cardiology
  • Geriatrics
  • Biomedical Science

Background:

  • Severe calcific aortic stenosis (AS) frequently coexists with transthyretin amyloid cardiomyopathy (AC-TTR) in elderly individuals.
  • The underlying mechanisms linking AS and AC-TTR are not fully understood but may involve amyloid infiltration triggering valve calcification or AS-induced strain promoting amyloidogenesis.

Purpose of the Study:

  • To explore the association between AS and AC-TTR.
  • To investigate the diagnostic clues for coexisting AC-TTR in AS patients.
  • To evaluate the prognostic implications of AC-TTR in patients undergoing transcatheter aortic valve replacement (TAVR).

Main Methods:

  • Clinical data analysis of elderly patients with severe AS and AC-TTR.
  • Echocardiogram and ECG findings in patients with coexisting AS and AC-TTR.
  • Bone scintigraphy for AC-TTR diagnosis.
  • Prognostic evaluation of TAVR candidates with AS and AC-TTR.

Main Results:

  • Approximately 14% of elderly patients with severe AS also have AC-TTR.
  • Echocardiography, ECG, and carpal tunnel syndrome history can suggest coexisting AC-TTR.
  • Bone scintigraphy confirms AC-TTR diagnosis.
  • AC-TTR does not significantly impact TAVR procedural outcomes but is linked to increased long-term heart failure risk.

Conclusions:

  • AS and AC-TTR commonly coexist in the elderly, with potential shared pathophysiological links.
  • Early suspicion and diagnostic confirmation of AC-TTR are crucial in AS patients.
  • Transcatheter aortic valve replacement remains a valid option for patients with AS and AC-TTR, provided standard clinical indications are met.

Keywords:
AmyloidosisAortic stenosisTAVITransthyretin

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