„ Clinical vignette

Papillary fibroelastoma of the aortic valve

Virginija Rudienė1Sigita Glaveckaitė1Mindaugas Matačiūnas2Edvardas Žurauskas3Kęstutis Ručinskas1
1Clinic of Cardiac and Vascular Diseases, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania
2Department of Radiology, Nuclear Medicine and Medical Physics, Institute of Biomedical Sciences, Vilnius University Faculty of Medicine, Vilnius, Lithuania
3National Center of Pathology, Vilnius University Hospital Santaros Klinikos, Vilnius, Lithuania

Correspondence to:

Virginija Rudienė, MD,

Faculty of Medicine Vilnius University,

M.K. Ciurlionio 21, Vilnius 03101, Lithuania

phone: +37 065 554 952,

e-mail: vr.rudiene@gmail.com

Copyright by the Author(s), 2022

DOI: 10.33963/KP.a2022.0102

Received: March 6, 2022

Accepted: April 13, 2022

Early publication date: April 15, 2022

Cardiac papillary fibroelastomas (CPFs) are benign primary cardiac tumors and are most often found on the downstream side of cardiac valves [1–3]. The most often affected valve is aortic, but these tumors could implicate all valves. The clinical presentation varies from asymptomatic to embolic complications, which can lead to cerebral stroke, myocardial infarction, and sudden cardiac death [1, 2, 4, 5]. In approximately 30% of cases, they are found incidentally, during autopsies, echocardiography, or cardiac surgery [2, 5].

A 34-year-old male was referred to the cardiology department for further evaluation due to dizziness and blinking in the eyes. Neurological examinations (carotid ultrasound imaging, transcranial color-coded duplex ultrasonography, and head computed tomography) were without abnormalities. Transthoracic echocardiography revealed a 0.9 × 1.2 cm oval-shaped mobile mass attached to the left coronary cusp of the tricuspid aortic valve (Figure 1AB, the arrows).

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Figure 1. Papillary fibroelastoma of the aortic valve seen on transthoracic echocardiography (A, B) and contrast-enhanced computed tomography images (C) (the arrows). The microscopic image of the excised mass (D) and histo-anatomopathological analysis of the mass (EF, the red stroma, the black arrow endocardium)

The mass did not cause any outflow tract obstruction or aortic regurgitation. A contrast-enhanced computed tomography scan of the chest confirmed the presence of a hypodense nodular lesion of approximate size of 1.1 × 1.4 × 1.2 cm in the aortic root, adjacent to the origin of the right and left coronary arteries (Figure 1C, the arrow). Based upon the above findings, a differential diagnosis was made which included: papillary fibroelastoma, myxoma, thrombus, and inflammatory mass. A coronary computed tomography angiography was normal. The decision was made to perform an excision of the tumor under cardio-pulmonary bypass. The valve-sparing surgery was done. At the time of surgery, a gel-like tumor of 1.2 × 1.0 × 1.0 cm was found (Figure 1D). The tumor with a pedicle was attached at the base of the commissure between the right and left coronary cusps of the aortic valve.

The pathological analysis confirmed the nature of the mass and revealed multiple, branching fronds of paucicellular, avascular fibroelastic tissue lined by a single layer of the endocardium (Figure 1EF). The postoperative period was complicated by hemorrhagic anemia and bacterial infection. The patient was discharged after 13 days. There was no recurrence seen on an echocardiogram 6 months after surgery.

The pathogenesis and risk factors of CPFs are unclear. Cardiac papillary fibroelastomas could be diagnosed at any age but most commonly occurs in middle-aged and older adults. Echocardiography is the principal diagnostic examination. Transesophageal echocardiography is more sensitive compared with transthoracic due to typically small sizes of these tumors and their attachment to the endocardial surface. Multimodality imaging (computed tomography and magnetic resonance angiography) could be helpful for differential diagnosis. A biopsy of the tumor is not usually needed. The treatment of CPFs is not clearly defined by guidelines. The surgery is recommended for larger than 1 cm left-sided papillary fibroelastomas, and it reduces the risk of thromboembolic complications [5]. CPFs can be safely excised with preservation of the native valve in experienced surgical centers [4]. Tamin et al. [4] demonstrated that the risk-to-benefit ratio of cardiac surgery is influenced by older age, comorbidities, and perhaps the uncertainty of embolic risk.

Usually, surgical resection is safe and has low perioperative mortality, and it is associated with perfect long-term outcomes [4, 5].

Article information

Conflict of interest: None declared.

Funding: None.

Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.

REFERENCES

  1. Tyebally S, Chen D, Bhattacharyya S, et al. Cardiac tumors. JACC Cardio­Oncol. 2020; 2(2): 293311, doi: 10.1016/j.jaccao.2020.05.009 indexed in Pubmed: 34396236..
  2. Gowda RM, Khan IA, Nair CK, et al. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J. 2003; 146(3): 404410, doi: 10.1016/s0002-8703(03)00249-7, indexed in Pubmed: 12947356.
  3. Koźma M, Stokłosa P, Kuśmierczyk M, et al. Cardiac papillary fibroelastoma mimicking infective endocarditis. Kardiol Pol. 2019; 77(1): 61, doi: 10.5603/KP.2019.0006, indexed in Pubmed: 30672580.
  4. Tamin SS, Maleszewski JJ, Scott CG, et al. Prognostic and bioepidemiologic implications of papillary fibroelastomas. J Am Coll Cardiol. 2015; 65(22): 24202429, doi: 10.1016/j.jacc.2015.03.569, indexed in Pubmed: 26046736.
  5. Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation. 2001; 103(22): 26872693, doi: 10.1161/01.cir.103.22.2687, indexed in Pubmed: 11390338.

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