Fibroelastoma, an Incidentaloma Disease? – Image Cases of Fibroelastomas as Incidental Findings in Four Patients, Four Different Valves

Abstract

Fibroelastomas are the second most common benign cardiac tumor¹. They are small avascular structures with a mean size of 9mm, ranging up to 70mm, usually attached to the heart valves’ surface (aortic and mitral are the most affected, followed by tricuspid and pulmonary valves). Their etiology is unclear, but the hypothesis of coalescence of microthrombus at the coaptation margins of valves is the most widely accepted theory. On echocardiography, they are pedicled, mobile, with a filamentous surface, and usually have a speckled appearance with echolucencies and a stippled pattern near the edges. Clinically, they may be associated with embolic phenomena; however, in most cases, the diagnosis is incidental. We present a series of four clinical cases with an incidental diagnosis of fibroelastomas across the four cardiac valves as assessed by transthoracic echocardiography (Video 1; Figure 1).

Video 1
From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient. Link: http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4

Figure 1
From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient.

Keywords:
Fibroelastoma; Incidental Findings; Heart Neoplasias/surgery; Heart Valves; Echocardiography, Transesophageal/methods; Embolization

Resumo

Os fibroelastomas são o segundo tumor cardíaco benigno mais comum. São estruturas pequenas, avasculares, com uma dimensão média de 9mm, podendo atingir até 70mm, habitualmente aderentes à superfície das válvulas cardíacas (válvulas aórtica e mitral são as mais comumente afetadas, seguidas das válvulas tricúspide e pulmonar). A etiologia não é clara, sendo a hipótese de formação de microtrombos nas margens de coaptação das válvulas a mais aceite. Na ecocardiografia apresentam aspeto pediculado, móvel, com superfície filamentosa, tipicamente com uma aparência pontilhada nas margens e ecolucente. Do ponto de vista clínico, podem estar associados a fenómenos embólicos, no entanto, na maioria dos casos o diagnóstico é incidental. Apresentamos de seguida quatro casos de diagnóstico incidental de fibroelastomas nas quatro válvulas cardíacas, diagnosticados por ecocardiograma transtorácico (ETT) (Vídeo 1; Figura 1).

Vídeo 1
Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da válvula tricúspide, folheto anterior da válvula mitral, cúspide esquerda da válvula pulmonar e cúspide esquerda da válvula aórtica, cada um correspondendo a um doente diferente. Em: http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4

Figura 1
Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da válvula tricúspide, folheto anterior da válvula mitral, cúspide esquerda da válvula pulmonar e cúspide esquerda da válvula aórtica, cada um correspondendo a um doente diferente.

Palavras-chave
Fibroelastoma; Achados Incidentais; Neoplasias Cardíacas/cirurgia; Valvas Cardíacas; Ecocardiografia Transesofagiana/métodos; Embolização

Description and Discussion

We present four fibroelastomas that were first diagnosed by transthoracic echocardiography, each located in a different cardiac valve.¹,² In all cases, the echocardiographic finding was incidental as opposed to a diagnosis that is typically ascertained following an investigation of an embolic phenomenon.³,⁴ Further characterization of the masses with transesophageal echocardiography (TEE) was obtained, confirming their shape along with a pedicle attaching them to a heart valve, high mobility, and speckled appearance, the latter corresponding to the papillary projections found on the surface. At this stage, differential diagnosis is mandatory, namely with vegetation (new or calcified/ancient) or thrombus. However, crucial echocardiographic clues that pointed towards fibroelastoma were (1) the round or oval shape of fibroelastomas, (2) the well-demarcated and homogenous appearance (vs. heterogenous appearance of vegetation or thrombus), and (3) the speckled appearance with stippling around the perimeter (which does not exist in vegetations or thrombi). Additionally, from a clinical point of view, the absence of fever, inflammatory parameters, or any sign or symptom suggestive of an infectious condition makes the diagnostic hypothesis of infectious endocarditis/vegetation on the echocardiogram unlikely.

In the four cases presented, the location, shape (in all cases oval/round, relatively regular and homogenous appearance), size (3 to 8mm), and further detailed characterization by TEE allowed for a presumptive diagnosis of fibroelastoma.¹ No relevant differences were found between right heart vs left heart fibroelastomas. According to recent literature, such differences were never documented. Cardiac computed tomography and cardiac magnetic resonance could also be used, particularly for right heart valves where TTE or TEE can be rather difficult; however, owing to temporal resolution limitations, small fibroelastomas can be missed.

There are no current guidelines for the treatment of fibroelastomas. According to current literature, symptomatic fibroelastomas should be surgically removed.⁵ As for asymptomatic fibroelastomas, opinions diverge and are based upon the mass mobility/embolic risk, position, concomitant cardiac surgery for other reasons, and comorbidities of the patient.⁶ The size and mobility of the tumors are the most important independent predictors of embolic events, regardless of left or right heart location. All four cases were discussed in the Heart Team, and surgical removal was considered the best course of action. Histological analysis confirmed the diagnosis of fibroelastoma in all cases.

In all cases, cardiac surgery was performed without complications. No embolic events were ever registered after a two-year follow-up. On echocardiographic follow-up, the valves morphology remains normal, no valve insufficiency or stenosis were noted, and there was no recurrence of the cardiac mass.

Fibroelastomas are (relatively) benign masses that can affect different parts of the heart and, crucially, all four cardiac valves. They can affect valvular function and serve as embolization material. There are no current guidelines for the treatment of fibroelastomas. In symptomatic patients, surgical removal is advised. In asymptomatic patients, a shared decision should take place based on the characteristics of the mass, embolic risk, and surgical risk of the patient.

• Sources of funding
  There were no external funding sources for this study.
• Study association
  This study is not associated with any thesis or dissertation work.
• Ethics approval and consent to participate
  This article does not contain any studies with human participants or animals performed by any of the authors.

Referências

• ¹
  Zoltowska MD. Cardiac papillary fibroelastoma. J Geriatr Cardiol. 2021;18(5):346-51. doi: 10.11909/j.issn.1671-5411.2021.05.009
  » https://doi.org/10.11909/j.issn.1671-5411.2021.05.009
• ²
  Devanabanda AR, Lee LS. Papillary Fibroelastoma. Stat Pearls [Preprint]. PMID: 31751019
• ³
  LAM KM, Dickens P, Chang AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993;117(10):1027-31. PMID: 8215825
• ⁴
  Tamin SS, Maleszewski JJ, Scott CG, Khan SK, Edwards WD, Bruce CJ, et al. Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas. J Am Coll Cardiol. 2015;65(22):2420-9. doi: 10.1016/j.jacc.2015.03.569
  » https://doi.org/10.1016/j.jacc.2015.03.569
• ⁵
  Boodhwani M, Veinot JP, Hendry PJ. Surgical approach to cardiac papillary fibroelastomas. Can J Cardiol. 2007;23(4):301–2. doi: 10.1016/s0828-282x(07)70759-6
  » https://doi.org/10.1016/s0828-282x(07)70759-6
• ⁶
  Masiello P, Catalano A, Mastrogiovanni G, Eusebio G, De Roberto AM, Chivasso P, et al. Surgical removal of an exceedingly rare papillary fibroelastoma of the aortic wall causing unstable angina. Clin Case Rep. 2021;9(9):e04688. doi: 10.1002/ccr3.4688
  » https://doi.org/10.1002/ccr3.4688

Publication Dates

• Publication in this collection
  23 Feb 2024
• Date of issue
  2024

History

• Received
  30 Mar 2023
• Reviewed
  14 Oct 2023
• Accepted
  14 Nov 2023