A patient with pulmonary hypertension: a rare case of AL amyloidosis involving the heart and external auditory canal
https://doi.org/10.15829/1560-4071-2024-5805
EDN: IDSDDZ
Abstract
The article presents a case of immunoglobulin light chain amyloidosis with cardiac involvement without typical noncardiac manifestations. A complex diagnostic search for the cause of newly diagnosed pulmonary hypertension is described. Aspects of differential diagnosis in invasive hemodynamic assessment of pulmonary circulation and echocardiographic data are discussed. A special case feature is involvement of the hearing organ, which is very rare.
About the Authors
V. A. Ryabkov
Petrozavodsk State University, Petrozavodsk;
Baranov Republican Hospital
Russian Federation
Russian Federation
Petrozavodsk
M. A. Egorenkova
Baranov Republican Hospital
Russian Federation
Russian Federation
Petrozavodsk
A. E. Zdorov
Petrozavodsk State University, Petrozavodsk;
Baranov Republican Hospital
Russian Federation
Russian Federation
Petrozavodsk
E. V. Golovchanskaya
Baranov Republican Hospital
Russian Federation
Russian Federation
Petrozavodsk
N. L.. Ryabkova
Petrozavodsk State University, Petrozavodsk;
Baranov Republican Hospital
Russian Federation
Russian Federation
Petrozavodsk
References
1. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-731. doi:10.1093/eurheartj/ehac237.
2. Donnelly J, Hanna M.Cardiac amyloidosis: An update on diagnosis and treatment. Cleve Clin J Med. 2017;84(12 Suppl 3):12-26. doi:10.3949/ccjm.84.s3.02.
3. Muchtar E, Dispenzieri A, Magen H, et al. Systemic amyloidosis from A (AA) to T (ATTR): a review. J Intern Med. 2021;289(3):268-92. doi:10.1111/joim.13169.
4. Tereshchenko SN, Zhirov IV, Moiseeva OM, et al. Practical guidelines for the diagnosis and treatment of transthyretin amyloid cardiomyopathy (ATTR-CM or transthyretin cardiac amyloidosis). Terapevticheskii Arkhiv. 2022;94(4):584-95. (In Russ.) doi:10.26442/00403660.2022.04.201465.
5. Kittleson MM, Ruberg FL, Ambardekar AV, et al. 2023 ACC Expert Consensus Decision Pathway on Comprehensive Multidisciplinary Care for the Patient With Cardiac Amyloidosis: A Report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2023;81(11):1076-126. doi:10.1016/j.jacc.2022.11.022.
6. Cuddy SAM, Chetrit M, Jankowski M, et al. Practical Points for Echocardiography in Cardiac Amyloidosis. J Am Soc Echocardiogr. 2022;35(9):A31-A40. doi:10.1016/j.echo.2022.06.006.
7. Dzhioeva ON. Functional methods of amyloid cardiomyopathy diagnostic in practice and in expert centers: A review. Terapevticheskii Arkhiv. 2023;95(1):96-102. (In Russ.) doi:10.26442/00403660.2023.01.202081.
8. Garcia-Pavia P, Rapezzi C, Adler Y, et al. Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases. Eur Heart. 2021;42(16):1554-68. doi:10.1093/eurheartj/ehab072.
9. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503-626. doi:10.1093/eurheartj/ehad194.
10. Dima D, Mazzoni S, Anwer F, et al. Diagnostic and Treatment Strategies for AL Amyloidosis in an Era of Therapeutic Innovation. JCO Oncol Pract. 2023;19(5):265-75. doi:10.1200/OP.22.00396.
11. Kumar SK, Callander NS, Adekola K, et al. Systemic Light Chain Amyloidosis, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2023;21(1):67-81. doi:10.6004/jnccn.2023.0001.
12. Reznik EV, Nguyen TL, Stepanova EA, et al. Cardiac Amyloidosis: Internist and Cardiologist Insight. The Russian Archives of Internal Medicine. 2020;10(6):430-57. (In Russ.) doi:10.20514/2226-6704-2020-10-6-430-457.
13. Reznik EV, Nguyen TL, Ustyuzhanin DV, et al. Red flags to diagnose infiltrative cardiomyopathies. Russian Journal of Cardiology. 2023;28(1S):5259. (In Russ.) doi:10.15829/1560-4071-2023-5259.
14. Gudkova АYa, Polyakova АА, Amelin АV, et al. Non-Val30Met-transthyretin amyloid cardiomyopathy. Literature review and clinical case. Russian Journal of Cardiology. 2018;(2):121-8. (In Russ.) doi:10.15829/1560-4071-2018-2-121-128.
15. Kitaoka H, Izumi C, Izumiya Y, et al. JCS 2020 Guideline on Diagnosis and Treatment of Cardiac Amyloidosis. Circ J. 2020;84(9):1610-71. doi:10.1253/circj.CJ-20-0110.
16. Jacques T, Giddings G, Hawkins P. Head and neck manifestations of amyloidosis. The Otorhinolaryngologist. 2013;6(1):35-40.
17. Hosoi T, Dhaliwal G, Tokuda Y.Amyloidosis in bilateral external auditory canals. BMJ Case Rep. 2014;2014:bcr2013200753. doi:10.1136/bcr-2013-200753.
18. Sethi S, Paner A, Okwuosa T, et al. Light Chain Amyloidosis Presenting as Bilateral External Auditory Canal Obstructing Masses. Otol Neurotol. 2018;39(8):e749-e751. doi:10.1097/MAO.0000000000001890.
19. Chow K, Kaul VF, Iacob CE, et al. Amyloidosis Presenting as a Mass Abutting the Tympanic Membrane. Ear Nose Throat J. 2021;100(5):337-9. doi:10.1177/0145561319868453
Supplementary files
- Isolated cardiac involvement in amyloidosis with diastolic dysfunction and pulmonary hypertension can be primarily interpreted as pulmonary hypertension.
- Echocardiography can reveal characteristic signs of cardiac amyloidosis.
- Right heart catheterization is not a necessary method for diagnosing cardiac amyloidosis, but may be considered in difficulties of non-invasive studies' interpretation in heart failure.
- Immunoglobulin light chain amyloidosis may be accompanied by external auditory canal.
Review
For citations:
Ryabkov V.A., Egorenkova M.A., Zdorov A.E., Golovchanskaya E.V., Ryabkova N.L. A patient with pulmonary hypertension: a rare case of AL amyloidosis involving the heart and external auditory canal. Russian Journal of Cardiology. 2024;29(2S):5805. (In Russ.) https://doi.org/10.15829/1560-4071-2024-5805. EDN: IDSDDZ
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