Work

Atrial natriuretic factor type amyloid leading to restrictive cardiomyopathy

Year: 2014

Type: article

Abstract: To the editor: In August 2013, a 72-year-old Caucasian female was admitted with history of recurrent bilateral pleural effusions and signs and symptoms suggestive of right sided heart failure. This started three months back, after she underwent catheter ablation of atrioventricular node and pacemaker placement for permanent atrial fibrillation in an outside hospital. Her transthoracic echocardiogram showed mild systolic dysfunction, grade 3–4 diastolic dysfunction, moderate mitral regurgitation, and severe tricuspid regurgitation. Right and left heart catheterization showed equalization of right and left sided end diastolic pressure at 15 millimeter of mercury without any evidence of ventricular discordance during respiration, consistent with restrictive physiology. Catheterization also showed triple vessel disease. The patient underwent coronary artery bypass grafting, mitral, tricuspid valve annuloplasty, and stapler excision of left atrial appendage (LAA). Congo red staining was requested on LAA specimen which showed green birefringence under polarized light, consistent with amyloidosis (Figure 1A, B). The block was sent to Mayo clinic for amyloid subtyping which revealed; LAA specimen was involved by amyloidosis, alpha atrial natriuretic factor (AANF) type. Bone marrow biopsy was negative for plasma cell dyscrasia or amyloid deposits. Therefore, diagnosis of restrictive cardiomyopathy due to AANF type of amyloidosis was established.Figure 1. A:: Extracellular matrix material suggestive of amyloidosis (HE, original magnification ×400). B: A congo red staining (original magnification ×400).Since incidence of AANF type of amyloid is quite high in surgically resected or aged left atrium, its clinical significance is neglected. It should be considered in patients with unexplained heart failure, especially those with history of atrial fibrillation.

Source: Chinese Medical Journal

DOI: 10.3760/cma.j.issn.0366-6999.20133025

Authors: Haider Imran, Yang Hong

Institution: St. Luke's Hospital

Language: English

Topic: Amyloidosis: Diagnosis, Treatment, Outcomes

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,000