Cardiac involvement affects 70% to 80% of patients with AL amyloidosis and represents the leading cause of mortality, making early diagnosis and multidisciplinary management critical.
Early diagnosis and intervention with chemo-immunotherapy or autologous stem cell transplantation are critical for improving outcomes in cardiac AL amyloidosis.
Cardiac amyloid light-chain (AL) amyloidosis is characterized by the deposition of misfolded light chain–derived amyloid fibrils within the myocardium, leading to restrictive cardiomyopathy. Direct fibril cytotoxicity is believed to be the main mechanism of AL amyloidosis. Cardiac involvement affects 70% to 80% of patients with AL amyloidosis and represents the leading cause of mortality. Diagnosis remains challenging due to nonspecific clinical manifestations and overlap with other cardiomyopathies. In patients with biopsy-proven AL amyloidosis, cardiac imaging, including echocardiography, cardiac magnetic resonance imaging (CMR), and positron emission tomography (PET), is essential for diagnosing cardiac involvement, assessing amyloid burden, and monitoring treatment response. Biomarkers, including N-terminal pro-B-type natriuretic peptide (NT-ProBNP) and troponin, are valuable for risk stratification and prognosis. Early diagnosis and intervention have become increasingly critical for improving patient outcomes, as delays in diagnosis are linked to poor survival. Treatment aimed at rapid suppression of light chain production with chemo-immunotherapy or autologous stem cell transplantation represents the current standard of care. Cardiac response is primarily monitored via changes in NT-proBNP and functional status, although emerging imaging modalities may enhance response assessment. Management of cardiac AL amyloidosis requires multidisciplinary collaboration and individualized care, especially given the impact of systemic disparities on diagnosis and treatment access.
Moaddab et al. (Thu,) conducted a review in Cardiac light-chain (AL) amyloidosis. Cardiac involvement affects 70% to 80% of patients with AL amyloidosis and represents the leading cause of mortality, making early diagnosis and multidisciplinary management critical.