AL Cardiac Amyloidosis in Resource-Limited Settings
- 1Department of Cardiology, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru
- 2Department of Hematology, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru
- 3Vicerrectorado de Investigación, Universidad San Ignacio de Loyola, Lima, Peru
Abstract
Amyloid light chain (AL) cardiac amyloidosis remains a diagnostic challenge in resource-limited settings. We present 3 cases with varying heart failure presentations, all demonstrating concentric left ventricular hypertrophy with characteristic apical sparing strain pattern on echocardiography. Serum free light chains were consistently abnormal, though immunofixation was negative in 2 patients. Traditional screening biopsies were negative in 2 cases, requiring endomyocardial biopsy for diagnosis. Mass spectrometry for amyloid typing was unavailable; immunofluorescence performed in 1 patient confirmed lambda light chain deposition. All patients received CyBorD-based therapy; 1 patient additionally accessed daratumumab abroad and showed marked improvement, while another died within 6 months on CyBorD alone. These cases highlight the heterogeneous presentation of presumed AL amyloidosis and underscore how diagnostic barriers contribute to delayed diagnosis and more advanced disease at presentation, emphasizing the urgent need for improved diagnostic capabilities and therapeutic accessibility in resource-constrained settings.
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BibTeX
@article{torresvalencia2026cardiac,
title = {{AL Cardiac Amyloidosis in Resource-Limited Settings}},
author = {Javier Torres-Valencia and Gabriela Zavaleta-Camacho and Luisser Saavedra-Cordova and Fernando Untiveros-Mayorga and Bryan Angulo-Garcia and Lucia Ronceros-Salas and Carlos Diaz-Arocutipa},
journal = {JACC Case Reports},
year = {2026},
month = aug,
doi = {10.1016/j.jaccas.2026.109794},
url = {https://doi.org/10.1016/j.jaccas.2026.109794},
note = {CC BY-NC-ND 4.0},
}
RIS
TY - JOUR
TI - AL Cardiac Amyloidosis in Resource-Limited Settings
AU - Javier Torres-Valencia
AU - Gabriela Zavaleta-Camacho
AU - Luisser Saavedra-Cordova
AU - Fernando Untiveros-Mayorga
AU - Bryan Angulo-Garcia
AU - Lucia Ronceros-Salas
AU - Carlos Diaz-Arocutipa
PY - 2026
DA - 2026/08/08
JO - JACC Case Reports
DO - 10.1016/j.jaccas.2026.109794
UR - https://doi.org/10.1016/j.jaccas.2026.109794
AB - Amyloid light chain (AL) cardiac amyloidosis remains a diagnostic challenge in resource-limited settings. We present 3 cases with varying heart failure presentations, all demonstrating concentric left ventricular hypertrophy with characteristic apical sparing strain pattern on echocardiography. Serum free light chains were consistently abnormal, though immunofixation was negative in 2 patients. Traditional screening biopsies were negative in 2 cases, requiring endomyocardial biopsy for diagnosis. Mass spectrometry for amyloid typing was unavailable; immunofluorescence performed in 1 patient confirmed lambda light chain deposition. All patients received CyBorD-based therapy; 1 patient additionally accessed daratumumab abroad and showed marked improvement, while another died within 6 months on CyBorD alone. These cases highlight the heterogeneous presentation of presumed AL amyloidosis and underscore how diagnostic barriers contribute to delayed diagnosis and more advanced disease at presentation, emphasizing the urgent need for improved diagnostic capabilities and therapeutic accessibility in resource-constrained settings.
SN - 2666-0849
ER -