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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 3  |  Page : 220-223  

Surgical resection of very small multiple papillary fibroelastomas of the aortic valve


Department of Cardiothoracic Surgery, Ruhr-University Hospital Bergmannsheil, Bochum, Germany

Date of Submission27-Nov-2020
Date of Acceptance08-Aug-2021
Date of Web Publication11-Oct-2021

Correspondence Address:
Dr. Dritan Useini
Department of Cardiothoracic Surgery, Ruhr-University Hospital Bergmannsheil, Bürkle de la Camp Platz 1; 44789 Bochum
Germany
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/HEARTVIEWS.HEARTVIEWS_208_20

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   Abstract 


Papillary fibroelastomas (PFEs) in multiple presentations on the aortic valve surface are extremely rare tumors that can cause sudden major cardio-cerebral adverse events. The treatment of these tumors varies from conservative care strategy to curative surgical resection. The bigger the tumor size, the bigger seems the tendency toward surgical treatment concepts. Here, we present the case of a completely healthy young woman who suffered sudden myocardial infarction and occlusion of the circumflex artery caused by very small multiple PFEs of the aortic valve. The patient underwent cardiac surgery for resection at our institution. The surgical approach appears to be recommended as a solitary reasonable treatment option once multiple PFEs have been detected.

Keywords: Multiple papillary fibroelastoma, myocardial infarction, surgical resection, very small


How to cite this article:
Useini D, Schlömicher M, Bechtel M, Strauch J. Surgical resection of very small multiple papillary fibroelastomas of the aortic valve. Heart Views 2021;22:220-3

How to cite this URL:
Useini D, Schlömicher M, Bechtel M, Strauch J. Surgical resection of very small multiple papillary fibroelastomas of the aortic valve. Heart Views [serial online] 2021 [cited 2021 Nov 28];22:220-3. Available from: https://www.heartviews.org/text.asp?2021/22/3/220/328022




   Introduction Top


A cardiac papillary fibroelastoma (PFE) is a rare tumor arising from the endocardium and is typically presented as a solitary tumor. The aortic valve is the most affected valve.[1] The occurrence of PFEs in multiple settings is very rare. Only 37 cases of multiple PFEs between 1981 and 2018 have been described in the literature.[2] The clinical presentation ranges from asymptomatic course up to stroke, myocardial infarction, and sudden death, all of these due to the tumor or thromboembolism.[1]

Most of the multiple PFEs of the aortic valve embolize when the tumor size achieves dimensions between 6 and 15 mm,[2] whereas the surgical resection of PFEs is recommended when the tumor size exceeds 10 mm.[3],[4] Here, we report the case of a young healthy woman without coronary artery disease who experienced a sudden myocardial infarction. The diagnostic workup revealed three very small PFEs of the aortic valve. A surgical resection was performed.


   Case Presentation Top


A 42-year-old athlete woman without any previous diagnosis was referred to a peripheral healthy institution due to the sudden occurrence of very severe angina pectoris symptoms during a jogging session. Physical, laboratory, and electrocardiography examinations revealed an acute nonST-elevation myocardial infarction. Immediately, a left heart catheterization was performed. A coronary artery disease was excluded. However, occlusion of the circumflex artery was diagnosed [Figure 1]. The patient was hemodynamically stable.
Figure 1: Left heart catheterization with coronarography, showing the occluded circumflex artery (black arrow)

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Transesophageal echocardiography detected three small tumorous formations on the aortic valve [Figure 2]. The other heart valves, the right and left outflow tracts, and all heart chambers were free from suspicious tumor formations. The inflammatory values were normal. The blood cultures were negative for microorganisms, and the patient had no symptoms and signs of endocarditis.
Figure 2: Transesophageal echocardiogram: Long axis (a) and a 3D view (b) of the preoperative transesophageal echocardiogram, showing the multiple papillary fibroelastoma

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As an aortic valve tumor was suspected, a surgical resection was indicated. After being admitted to our institution, the patient was completely stable without any symptoms. After adequate preparations, the surgical resection of the tumors was performed through upper partial sternotomy.

Intraoperatively, we saw three small tumors on the aortic side of the left coronary cusp (2 mm), the noncoronary cusp (1 mm), and the right coronary cusp (1 mm). The anchoring area was minimal, and the surrounding tissue was unremarkable [Figure 3]. Other tumor structures could not be found. The resection of the tumors was not complicated and unspectacular. Next, the aortic valve remained structurally and functionally intact.
Figure 3: Intraoperative view of the multiple papillary fibroelastomas (a and b) of the aortic valve (black arrows)

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A subsequent histopathological assessment of all three tumors revealed a PFE. The highlight of the histopathological assessment was a thrombus deposition on the top of the tumor [Figure 4]. The postoperative course was completely uneventful. The patient was discharged on the 10th postoperative day.
Figure 4: Hematoxylin and eosin stain of the papillary fibroelastoma with areas comprising thrombus deposition; magnification, ×100

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   Discussion Top


Generally, the decision for surgical resection of a PFE is recommended after considering the tumor size, tumor mobility, embolization, symptoms, and localization.[1],[2],[3],[4],[5] The tumor size seems to be the leading benchmark for deciding in favor of surgical treatment of PFEs. While the PFE size varies from 2 mm to 70 mm,[1] many studies predetermined a cutoff value of 10 mm in consideration of surgical treatment.[3],[4] Multiple PFEs are very scarcely reported. One of the most recent and comprehensive reviews (included all published cases of multiple PFEs between 1981 and 2018)[2] reported that the most average size of embolizing multiple PFE tumors was between 6 and 10 mm. There was only one case reported which was embolized and where the tumor size was <6 mm (5 mm). We report here the smallest tumor size of embolized multiple PFEs. Owing to the rarity of these tumors, therapy recommendations are not based on the guidelines or randomized controlled trials. However, the therapy target should be to avoid the embolization that can be life threatening.

Besides the surgical resection, alternative therapy concepts have been recommended, for instance, a different anticoagulation regimen with multimodal imaging follow-ups are suggested, especially when the tumor is small, not pedunculated, and immobile.[1] These care concepts are partly justified because the embolization potential of the PFE originates not only from the fragmentation of the papillary spikelet but also from the thrombi organized on the surface of the tumors. The last pathomechanism of the embolization was obviously assumed in our case. However, we would not have recommended an anticoagulation regimen. First, there was no evidence of safety and the degree of protection from thrombus embolization. Moreover, no protection can be expected from tumor particles. Second, an anticoagulation regimen can be associated with bleeding complications. Third, anticoagulation therapy is not curative and its duration is not clear. Moreover, repeated hospital admissions for clinical and imaging follow-ups may be associated with increased costs. On the other side, the long-term prognosis after the surgical removal of the PFE is excellent.[1] Therefore, we recommend surgical resection of multiple PFEs independent of the tumor size and other concomitant pathoanatomical features of the tumor.


   Conclusion Top


Independent of the size and clinical presentation, multiple PFEs of the aortic valve should be surgically resected to avoid life-threatening complications such as myocardial infarction.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: A comprehensive analysis of 725 cases. Am Heart J 2003;146:404-10.  Back to cited text no. 1
    
2.
Tsugu T, Nagatomo Y, Endo J, Kawakami T, Murata M, Yamazaki M, et al. Multiple papillary fibroelastomas attached to left ventricular side and aortic side of the aortic valve: A report of new case and literature review. Echocardiography 2019;36:1194-9.  Back to cited text no. 2
    
3.
Nowacka A, Rancati V, Roumy A, Monney P, Marcucci CE, Kirsch M. Multiple Cardiac Papillary Fibroelastomas-Are They Really Rare or Underdiagnosed? Heart Lung Circ 2019;28:e83-5.  Back to cited text no. 3
    
4.
Miller A, Perez A, Pabba S, Shetty V. Aortic valve papillary fibroelastoma causing embolic strokes: A case report and review. Int Med Case Rep J 2017;10:109-12.  Back to cited text no. 4
    
5.
Maludum O, Ugoeke N, Mahida H, Ajam F, Alrefaee A, Calderon D, et al. Papillary fibroelastoma on the aortic valve presenting as multiple cardiac arrests from electrical storm due to ischemia in patient without previous cardiac history. HeartRhythm Case Rep 2019;5:134-7.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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