International journal of cardiology · 2026
Clinical manifestations and cardiac abnormalities in systemic Amyloid A Amyloidosis.
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Fardman Alexander, Negev Robert, Kukuy Olga Lesya, Wasserstrum Yishay, Brodov Yafim, Pras Elon, Livneh Avi, Kuperstein Rafael, Maor Elad, Arad Michael
Аннотация
BACKGROUND: Cardiac involvement in systemic amyloid A (AA) amyloidosis is traditionally considered rare. Respectively, contemporary data describing cardiac characteristics of patients with systemic AA amyloidosis are scarce.
METHODS: All patients with a diagnosis of systemic amyloidosis were identified from electronic medical records of a tertiary medical center. Patients with non-AA amyloidosis, concomitant multiple myeloma, lack of histological confirmation, or absence of cardiac evaluation were excluded. Probable cardiac involvement was diagnosed based on predefined criteria.
RESULTS: The final cohort included 43 patients with AA amyloidosis, diagnosed either by immunohistochemistry (n = 32,74%) or clinically (positive Congo Red staining + systemic inflammatory disease). The mean age was 56 ± 11 years, and 23 (54%) were women. Probable cardiac involvement was identified in14 (33%) patients. Patients with probable cardiac involvement had wider QRS duration (101 milliseconds, IQR (83,114) vs 86 milliseconds, IQR (76,98), p-value = 0.019), right ventricular hypertrophy (29% vs 0, p-value = 0.008) and dysfunction (21% vs 0, p-value = 0.029), larger left atrial diameter (41.6 ± 5.7 mm vs 38 ± 4.6, p-value = 0.04), and more frequent pericardial effusion (43% vs 14%, p-value = 0.009) compared with those without probable cardiac disease. Patients with probable cardiac involvement had higher median serum Amyloid A levels (24 mg/L IQR (15,56) vs 13 mg/L (6.6,36), p-value = 0.047) and more frequently had evidence of gastrointestinal manifestations (64% vs 28%, p-value = 0.021), then those without cardiac involvement.
CONCLUSIONS: Pathological cardiac findings are not uncommon in patients with AA amyloidosis warranting a routine cardiac evaluation and longitudinal follow up.
METHODS: All patients with a diagnosis of systemic amyloidosis were identified from electronic medical records of a tertiary medical center. Patients with non-AA amyloidosis, concomitant multiple myeloma, lack of histological confirmation, or absence of cardiac evaluation were excluded. Probable cardiac involvement was diagnosed based on predefined criteria.
RESULTS: The final cohort included 43 patients with AA amyloidosis, diagnosed either by immunohistochemistry (n = 32,74%) or clinically (positive Congo Red staining + systemic inflammatory disease). The mean age was 56 ± 11 years, and 23 (54%) were women. Probable cardiac involvement was identified in14 (33%) patients. Patients with probable cardiac involvement had wider QRS duration (101 milliseconds, IQR (83,114) vs 86 milliseconds, IQR (76,98), p-value = 0.019), right ventricular hypertrophy (29% vs 0, p-value = 0.008) and dysfunction (21% vs 0, p-value = 0.029), larger left atrial diameter (41.6 ± 5.7 mm vs 38 ± 4.6, p-value = 0.04), and more frequent pericardial effusion (43% vs 14%, p-value = 0.009) compared with those without probable cardiac disease. Patients with probable cardiac involvement had higher median serum Amyloid A levels (24 mg/L IQR (15,56) vs 13 mg/L (6.6,36), p-value = 0.047) and more frequently had evidence of gastrointestinal manifestations (64% vs 28%, p-value = 0.021), then those without cardiac involvement.
CONCLUSIONS: Pathological cardiac findings are not uncommon in patients with AA amyloidosis warranting a routine cardiac evaluation and longitudinal follow up.