Clinical manifestations and cardiac abnormalities in systemic Amyloid A Amyloidosis

Scritto il 08/10/2026
da Alexander Fardman

Int J Cardiol. 2026 Oct 8:134973. doi: 10.1016/j.ijcard.2026.134973. Online ahead of print.

ABSTRACT

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.

PMID:42849794 | DOI:10.1016/j.ijcard.2026.134973