Editorial Type:
Article Category: Research Article
 | 
Online Publication Date: Jun 17, 2020

Giant Papillary Fibroelastoma Attached to the Left Atrial Septum, Near the Foramen Ovale

MD, PhD,
MD, PhD,
MD, PhD, and
MD, PhD
Page Range: 175 – 176
DOI: 10.14503/THIJ-17-6516
Save
Download PDF

An asymptomatic 70-year-old woman was referred to our hospital because of hypertension and diabetes mellitus. A grade 3/6 systolic murmur was heard at the 2nd left sternal border. The only abnormal laboratory finding was a high plasma brain natriuretic peptide level (84 pg/mL). Transthoracic echocardiograms revealed moderate aortic regurgitation caused by sclerotic aortic valve changes, and also a mobile, low-echoic, 40 × 10-mm restiform lesion on the left atrial septum near the foramen ovale (Fig. 1). We suspected trapped thrombus in the foramen ovale. Computed tomograms showed no cerebral infarction, thrombus in the pulmonary artery or deep veins, or malignancy. Because of embolic risk, we surgically resected the 34 × 8 × 3-mm lesion, which had a short pedicle and multiple papillary fronds (Fig. 2). The histologic findings of multiple papillary fronds consisting of an acellular matrix surrounded by a single layer of endothelial cells led to the diagnosis of papillary fibroelastoma (PFE) (Fig. 3). Six years later, no recurrence was observed.

Fig. 1.Fig. 1.Fig. 1.
Fig. 1. Transesophageal echocardiogram (midesophageal 4-chamber view, 0° rotation) shows a mobile, low-echoic restiform lesion at the left atrium (LA) (arrows) and a stalk attached to the left atrial septum near the foramen ovale. Ao = aorta; LV = left ventricle Supplemental motion image is available for Figure 1.

Citation: Texas Heart Institute Journal 47, 2; 10.14503/THIJ-17-6516

Fig. 2.Fig. 2.Fig. 2.
Fig. 2. Photograph of the resected 34 × 8 × 3-mm fibroelastoma shows multiple glistening, frond-like papillary structures.

Citation: Texas Heart Institute Journal 47, 2; 10.14503/THIJ-17-6516

Fig. 3.Fig. 3.Fig. 3.
Fig. 3. Photomicrographs of the papillary fibroelastoma show A) elongated and branching papillary fronds, composed of central avascular collagen, and variable elastic tissue ringed by flattened endothelial cells (H & E, orig. ×100); B) multiple papillary fronds with a dense core of connective tissue containing elastic fibers (Elastica van Gieson stain, orig. ×100); and C) surface endothelial lining cells are positive for CD31 (immunohistochemical stain, orig. ×100).

Citation: Texas Heart Institute Journal 47, 2; 10.14503/THIJ-17-6516

Comment

Papillary fibroelastoma typically originates from valvular endocardium; nonvalvular origin accounts for 16.4% of cases.1 Most PFEs are <10 mm in size, and only 1% are >20 mm.2 In our patient's case, the surface of the interatrial septum near the foramen ovale was an atypical location for a PFE; moreover, the lesion seems to have been larger than any PFE previously reported, which initially made us suspect thrombus trapped at the foramen ovale. However, histologic examination confirmed the lesion to be a PFE. Although PFEs are benign tumors, the risk of embolic complications is high,1 and surgical excision is recommended in all symptomatic patients.

References

  • 1.

    Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J2003;146(

    3
    ):404–10.

  • 2.

    Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation2001;103(

    22
    ):2687–93.

  • Download PDF
Copyright: © 2020 by the Texas Heart® Institute, Houston
video
Fig. 1.
Fig. 1.

Transesophageal echocardiogram (midesophageal 4-chamber view, 0° rotation) shows a mobile, low-echoic restiform lesion at the left atrium (LA) (arrows) and a stalk attached to the left atrial septum near the foramen ovale.

Ao = aorta; LV = left ventricle

Supplemental motion image is available for Figure 1.

Fig. 2.
Fig. 2.

Photograph of the resected 34 × 8 × 3-mm fibroelastoma shows multiple glistening, frond-like papillary structures.

Fig. 3.
Fig. 3.

Photomicrographs of the papillary fibroelastoma show A) elongated and branching papillary fronds, composed of central avascular collagen, and variable elastic tissue ringed by flattened endothelial cells (H & E, orig. ×100); B) multiple papillary fronds with a dense core of connective tissue containing elastic fibers (Elastica van Gieson stain, orig. ×100); and C) surface endothelial lining cells are positive for CD31 (immunohistochemical stain, orig. ×100).

Contributor Notes

Section Editor: Raymond F. Stainback, MD

Address for reprints: Toshimitsu Tsugu, MD, Department of Cardiology, Federation of National Public Service Personnel Mutual Aid Association Tachikawa Hospital, 4-2-22 Nishiki-cho, Tachikawa City, 190-8531 Tokyo, Japan; E-mail: tsugu917@gmail.com