Introduction:Cardiac amyloidosis is a rare and under-diagnosed
disease. The objective of this study was to collect cases of cardiac amyloidosis
in patients hospitalized in the cardiology department of the Centre Hospitalier
Intercommunal Alençon-Mamers (CHICAM). Patients and Methods: This was a
retrospective descriptive study of the records of patients diagnosed with
cardiac amyloidosis. The study took place in the cardiology department of the
CHICAM over 12 months (from January 1, 2020 to December 31, 2020).We included the files of
patients in whom the diagnosis of cardiac amyloidosis was confirmed on the
basis of clinical, echocardiographic, biological, scintigraphic and cardiac MRI
evidence. Results: Seven patients were included. The mean age was 86.71
years. Six men for one woman. Most of the patients were in heart failure. There
was one case of periorbital ecchymosis. Troponinemia was increased in three
patients, NTproBNP was always high with a mean of 1000ng/L. Cardiac echography showed a hyperbright septum
in four cases, concentric hypertrophy of the left ventricle with a mean
interventricular septum in diastole of 13.85mm. Six cases were senile transthyretin amyloidosis
(TTR), one case was mutated transthyretin amyloidosis (TTRm). Two cases of
death were noted. Conclusion: Cardiac amyloidosis has a poor prognosis
due to delayed diagnosis.
References
[1]
Magy-Bertranda, N. (2016) Actualités sur les amyloses. La Revue de Médecine Interne, 37, 529-535. https://doi.org/10.1016/j.revmed.2015.12.029
[2]
Goïorania, F., Dagrenata, C., Brocchia, J., Couppieb, P. and Leddet, P. (2020) Le diagnostic de l’amylose cardiaque par résonance magnétique: les éléments discriminants. Annales de Cardiologie et d'Angéiologie, 69, 262-267. https://doi.org/10.1016/j.ancard.2020.09.023
Kyle, R.A. and Gertz, M.A. (1995) Primary Systemic Amyloidosis: Clinical and Laboratory Features in 474 Cases. Seminars in Hematology, 32, 45-59.
[5]
Bodez, D., Galat, A., Guellich, A., Deux, J.-F., Rosso, J., Le Bras, F., et al. (2016) Les amyloses cardiaques: les reconnaître et les prendre en charge. La Presse Médicale, 45, 845-855. https://doi.org/10.1016/j.lpm.2016.07.001
[6]
Eicher, J.C., Audia, S. and Damy, T. (2020) L'amylose cardiaque à transthyrétine. La Revue de Médecine Interne, 41, 673-683. https://doi.org/10.1016/j.revmed.2020.07.002
[7]
Damy, T., Mohty, D., Deux, J.-F., Rosso, J., Benhaiem, N., Lellouche, N., et al. (2013) Amylose systémique sénile: définition, diagnostic, pourquoi y penser? La Presse Médicale, 42, 1003-1014. https://doi.org/10.1016/j.revmed.2020.07.002
[8]
Cornwell, G. and Westermark, P. (1980) Senile Amyloidosis: A Protean Manifestation of the Aging Process. Journal of Clinical Pathology, 33, 1146-1152. https://doi.org/10.1136/jcp.33.12.1146
[9]
Cornwell, G., et al. (1983) Frequency and Distribution of Senile Cardiovascular Amyloid. A Clinicopathologic Correlation. The American Journal of Medicine, 75, 618-623. https://doi.org/10.1016/0002-9343(83)90443-6
[10]
Pitkanen, P., Westermark, P. and Cornwell, G. (1984) Senile Systemic Amyloidosis. The American Journal of Pathology, 117, 391-399.
[11]
Ueda, M., et al. (2011) Clinicopathological Features of Senile Systemic Amyloidosis: An Ante- and Post-Mortem Study. Modern Pathology, 24, 1533-1544. https://doi.org/10.1038/modpathol.2011.117
[12]
Isabel, C., Georgin-Laviallea, S., Aoubab, A., Delarueb, R., Nochyc, D., Karrasd, A., et al. (2013) Amylose cardiaque: à propos d’une série de 14 patients, description et facteurs pronostiques. La Revue de Médecine Interne, 34, 671-678. https://doi.org/10.1016/j.revmed.2013.05.003
[13]
Phelan, D., Collier, P., Thavendiranathan, P., Popovic, Z.B., Hanna, M., Plana, J.C., et al. (2012) Relative Apical Sparing of Longitudinal Strain Using Two-Dimensional Speckle-Tracking Echocardiography Is Both Sensitive and Specific for the Diagnosis of Cardiac Amyloidosis. Heart, 98, 1442-1448. https://doi.org/10.1136/heartjnl-2012-302353
[14]
Connors, L.H., Doros, G., Sam, F., Badiee, A., Seldin, D.C. and Skinner, M. (2011) Clinical Features and Survival in Senile Systemic Amyloidosis: Comparison to Familial Transthyretin Cardiomyopathy. The Journal of Protein Folding Disorders, 18, 157-159. https://doi.org/10.3109/13506129.2011.574354059