Prevalence of Isolated Atrial Amyloidosis in Young Patients Affected by Congestive Heart Failure

Abstract

Atrial natriuretic peptide (ANP), whose amyloid is responsible of isolated atrial amyloidosis (IAA), is known to play an important role in the pathophysiology of congestive heart failure (CHF). We provide here the microscopic examination of atrial biopsies from 36 young (mean 40 years) CHF patients distinguished in idiopathic dilated cardiomyopathy (DC) affected and hypertrophic Cardiomyopathy (HC) affected, endorsing the presumptive association of early CHF with IAA. We utilized a multiple method, using Congo red (CR) staining, CR fluorescence (CRF), and immunohistochemistry to assess the presence of IAA in CHF. Immunostaining showed a moderate deposition of IAA in the atrium surrounding working myocardium with small intracellular deposits. Our findings suggest a monitoring of young CHF cases for the development of IAA. Our study also demonstrated how the concurrent use of immunohistochemistry, CR, and CRF may greatly enhance the detection of low-grade amyloid deposits.

Recent Advances in the Diagnosis and Management of Cardiac Amyloidosis

“…Unlike other cardiac amyloidosis, this is almost always localized to atria and can only be diagnosed on histologic examination of the atrial tissue, either at biopsy or autopsy examination. The incidence of IAA increases with age and appears to be more common in patients with atrial diseases such as rheumatic heart disease [24]. The exact underlying etiology of IAA remains to be fully elucidated and it has been postulated as a potential cause of common tachyarrhythmias, such as atrial fibrillation.…”

Amyloid and Amyloid-Like Aggregates: Diversity and the Term Crisis

“…[51]. An example of age related disease is isolated atrial amyloidosis caused by the presence of amyloid fibrils, the main component of which is an atrial natriuretic factor (ANF) [52]. Aggregation of semenogelin 1 (SEMG1) in epithelial cells of the seminal vesicles occurs during the senile local ized amyloidosis [53].…”

Cardiac amyloidosis: a hidden cause of cardiovascular complications in oncology practice

“…Nevertheless, there are studies that predict a role of isolated atrial amyloidosis in the development of disorders of the conduction system and atrial fibrillation in older population [1,18]. A recent study involving a group of middle-aged patients with mean age of 40 years with diagnosis of congestive heart failure showed the presence of amyloid deposits in 91.6% of cases, where 93.9% of them were positive for ANP [20]. This demonstrates the need to monitor these patients due to an increased risk of progressive heart amyloidosis.…”