Suspected transthyretin cardiac amyloidosis by cardiac scintigraphy in heart failure with preserved ejection fraction, left ventricular hypertrophy and red flag manifestations

Main Article Content

Mariano Ezequiel Napoli Llobera Lilia Luz Lobo Marquez Kari Kostiw Dave Webster Atilio Eugenio Costa Vitali

Abstract

Background


Transthyretin Cardiac Amyloidosis (ATTR) is a type of restrictive cardiomyopathy, which typically manifests as Heart Failure with Preserved Ejection Fraction (HFpEF).


The presence of unexplained left ventricular hypertrophy (LVH) associated with HF and red flag manifestations could increase the diagnostic probability. However, the diagnostic prevalence of this triad by cardiac scintigraphy remains uncertain.


Methods


From August 1st to December 31st, 2021, 22 consecutive patients diagnosed with a HF (ejection fraction more than 40%), LVH with unexplained etiology and at least one red flag clinical manifestation, underwent pyrophosphate scintigraphy (99mTc-PYP). The patients were divided into two groups: “Positive” and “Negative” (as defined by grade 2 or 3 uptake). Multiple logistic models were made with variable 99mTC-PYP and explanatory variables.


Results


Among 22 patients, 15 had a positive 99mTc-PYP study for ATTR. The prevalence of ATTR using the triad of HFpEF, unexplained LVH and at least one red flag was 68% (CI 95%; 45-86%). Patients with 99mTc-PYP positive tended to be male, older, and with an aortic mean gradient and interventricular septum higher, as compared to the group with a negative study. The most frequent red flag clinical manifestations were proteinuria (55%) and pseudoinfarction pattern (55%). The presence of 2 or more red flags could increase the diagnostic probability of the test (OR 1.6 (CI95% 0.52-4.89).


Conclusions


The diagnostic probability of ATTR by 99mTc-PYP scan could increased when a clinical manifestation of a red flag was added to the suspected diagnosis of heart failure and left ventricular hypertrophy. The use of non-invasive techniques allows early identification and treatment of this underdiagnosed disease.

Article Details

How to Cite
NAPOLI LLOBERA, Mariano Ezequiel et al. Suspected transthyretin cardiac amyloidosis by cardiac scintigraphy in heart failure with preserved ejection fraction, left ventricular hypertrophy and red flag manifestations. Medical Research Archives, [S.l.], v. 10, n. 3, mar. 2022. ISSN 2375-1924. Available at: <https://esmed.org/MRA/mra/article/view/2739>. Date accessed: 28 nov. 2022. doi: https://doi.org/10.18103/mra.v10i3.2739.
Section
Research Articles

References

1. García-Pavía P, Tomé -Esteban MT, Rapezzi C. Amiloidosis. También una enfermedad del corazón. Rev Esp Cardiol. 2011; 64:797–808.
2. Rapezzi C, Lorenzini M, Longhi S, et al. Cardiac amyloidosis: the great pretender. Heart Fail Rev. 2015; 20:117–24.
3. Fontana M, Ćorović A, Scully P, Moon JC, et al. Myocardial Amyloidosis: The Exemplar Interstitial Disease. JACC. 2019; 12:23-45.
4. Gonzalez-Lopez E, Gallego-Delgado M, Guzzo-Merello G, et al. Wild Type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J. 2015; 36:2585-94.
5. Inomata T, Tahara N, Nakamura K, Endo J, et al. Diagnosis of wild-type transthyretin amyloid cardiomyopathy in Japan: red-flag symptom clusters and diagnostic algorithm. ESC Heart failure. 2021; 8:2647-2659.
6. Garcia-Pavia P, Rapezzi C, Adler Y, Arad M, et al. Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. ESC Heart Failure. 2021; 10:1-15.
7. Lindmark K, Pilebro B, Sundström T, Lindqvist P. Prevalence of wild type transthyretin cardiac amyloidosis in a heart failure clinic. ESC Heart Failure. 2021; 8:745-749.
8. Gertz MA, Benson MD, Dyck PJ, Grogan M, et al. Diagnosis, Prognosis, and Therapy of Transthyretin Amyloidosis. JACC. 2015; 66:24-51.
9. AbouEzzeddine O, Davies D, Scott C, Fayyaz A, et al. Prevalence of Transthyretin Amyloid Cardiomyopathy in Heart Failure With Preserved Ejection Fraction. JAMA Cardiol. 2021; 6:1267-1274.
10. Adams D, Ando Y, Melo Beirao J, Coelho T, et al. Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy. Journal of Neurology. 2021;268:2109-2122.