Echocardiographic findings focused on the inferior vena cava partly aid the diagnosis of cardiac amyloidosis

Abstract

Cardiac amyloidosis is typically suspected based on various echocardiographic signs termed ‘red flags.’ 1 In this case, we present clinical and pathological findings focused on the inferior vena cava (IVC) in a patient with cardiac amyloidosis. A 95-year-old female was admitted at our hospital for shortness of breath. Upon admission, echocardiography revealed unexplained left ventricular thickness (interventricular septum thickness 14 mm, posterior wall thickness 13 mm) and brightness that resembled a granular sparkling sign with preserved ejection fraction (left ventricular ejection fraction 53%, Panel A). Because of her advanced age, the patient denied invasive clinical examinations and subsequently died of heart failure after 5 days. Pathological examination conducted at her family’s request revealed amyloid deposition in the left ventricular wall through direct fast scarlet (DFS) staining (Panel B). Retrospectively, echocardiography detected high-contrast and hypertrophy of the IVC wall (IVC wall thickness 3.0 mm, Panel C) compared with the normal range. 2 Macroscopic findings also showed venous hypertrophy and sclerosis (Panel D). Light microscopy specifically revealed extracellular deposition of misfolded proteins in the IVC wall (Panel E). DFS staining revealed diffuse amyloid deposition in the IVC wall (Panel F). Although a previous pathological report clarified that amyloid deposition can occur in the whole body including the veins, 3,4 this is the first clinical report focused on cardiac amyloidosis in the IVC. IVC findings on echocardiography, such as those observed in this case, may provide useful additional information in the diagnosis of cardiac amyloidosis. No new data were generated or analysed in support of this research. Taiki Tojo (Conceptualization: Supporting; Data curation: Supporting; Investigation: Supporting; Project administration: Lead; Supervision: Supporting; Visualization: Equal; Writing – review & editing: Lead), Ichiro Maeda (Data curation: Lead; Formal analysis: Lead; Investigation: Equal; Supervision: Lead; Writing—review & editing: Equal), and Kazuhiro Fujiyoshi (Conceptualization: Lead; Data curation: Equal; Formal analysis: Equal; Investigation: Lead; Project administration: Equal; Visualization: Lead; Writing—original draft: Lead) All authors declare no conflict of interest for this contribution. Figure 1 (Panel A) Echocardiographic image depicting a four-chamber view of the heart. (Panel B) The image of DFS staining revealed that the deposits were DFS positive at the myocardium. (Panel C) Echocardiographic image of the IVC wall (arrowheads). (Panel D) Macroscopic image of the IVC. Panel E) the image of the IVC stained by hematoxylin and eosin. (Panel F) The image of DFS staining revealed that the deposits were DFS positive at the media of IVC. IVC, inferior vena cava, DFS, direct fast scarlet.