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CASE REPORT
Year : 2022  |  Volume : 32  |  Issue : 4  |  Page : 222-224

Very Mobile Left Ventricular Outflow Tract Papillary Fibroelastoma Presenting with Multiple Ischemic Strokes: A Case Report and Brief Review of the Literature


Cardiology and Arrhythmology Clinic, Marche Polytechnic University, University Hospital “Ospedali Riuniti,” Ancona, Italy

Date of Submission07-Jul-2022
Date of Decision31-Jul-2022
Date of Acceptance25-Aug-2022
Date of Web Publication23-Jan-2023

Correspondence Address:
Alessandro Barbarossa
Via Conca, 71, 60126 Ancona AN
Italy
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcecho.jcecho_40_22

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  Abstract 


Papillary fibroelastomas (PFs) are small and pedunculated left side valves associated mass, that frequently causing cerebral embolization. We present the case of a 69-year-old male with a history of multiple ischemic strokes and a small pedunculated mass in the left ventricle outflow tract, highly suggestive of a rare case of PF in an atypical localization. Due to the clinical history and the echocardiographic aspect of the mass, he underwent surgical excision and Bentall intervention for concomitant aortic root and ascending aorta aneurysm. The pathological analysis of the surgical specimen confirmed the diagnosis of PF.

Keywords: Cardiac masses, ischemic stroke, papillary fibroelastoma, transesophageal echocardiography


How to cite this article:
Barbarossa A, Coraducci F, Cipolletta L, Guerra F, Russo AD. Very Mobile Left Ventricular Outflow Tract Papillary Fibroelastoma Presenting with Multiple Ischemic Strokes: A Case Report and Brief Review of the Literature. J Cardiovasc Echography 2022;32:222-4

How to cite this URL:
Barbarossa A, Coraducci F, Cipolletta L, Guerra F, Russo AD. Very Mobile Left Ventricular Outflow Tract Papillary Fibroelastoma Presenting with Multiple Ischemic Strokes: A Case Report and Brief Review of the Literature. J Cardiovasc Echography [serial online] 2022 [cited 2023 Feb 2];32:222-4. Available from: https://www.jcecho.org/text.asp?2022/32/4/222/368432




  Introduction Top


Papillary fibroelastomas (PFs) are composed of collagen and elastic fibers; they are usually connected with a pedicle to the endocardial layer.[1] Found attached to the left-sided valve usually PFs do not cause valve dysfunction because of the small dimensions. Differential diagnosis with endocarditis can rely on the side of attachement: PFs are more frequently found downstream the valves, instead, endocarditis are mostly found upstream the valves.[2] Although usually asymptomatic, the most important complication is systemic embolization, particularly cerebral.[3] Surgical excision is reserved for large left-sided and symptomatic tumors.


  Case Report Top


A 69-year-old male presented at our echo lab for routine screening due to an anamnesis of prior ischemic strokes (three episodes 5 years ago). Cardiovascular risk factors were systemic hypertension, type 2 diabetes mellitus, prior smoking, and a familiar history of cardiovascular disease. He also referred in the last 3-year brief episodes of chest pain and dyspnea not related to exertion. He was on medical therapy with losartan 100 mg/die, nifedipine 30 mg/die, and bisoprolol 5 mg/die.

Transthoracic echocardiography showed hypertrophic left ventricle with normal systolic function, tricuspid aortic valve with moderate regurgitation, and an aneurysm of the aortic root (47 mm) and of the ascending aorta (49 mm). The most relevant finding was the presence of a small round-shaped, isoechogenic mass with a marked mobility in the left ventricular outflow tract (LVOT) [Figure 1]a.
Figure 1: (a) TTE apical three chambers; (b and c) TEE long-axis view; (d) surgical sample; (e) histopathological analysis of the specimen. TTE = Transthoracic echocardiography, TEE = Transesophageal echocardiography

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Due to these findings, the patient underwent transesophageal echocardiography (TEE).

With the TEE examination, we were able to define the origin of the mass, arising from the left ventricular basal septum with a very small pedunculus and a systolic protruding movement in LVOT [Figure 1]b and [Figure 1]c, and to correctly assess its dimensions (13 mm × 9 mm). Collaterally, the TEE examination confirmed the aortic root aneurysm (49 cm) with moderate aortic regurgitation.

The differential diagnosis of the mass was PF or cardiac myxoma (CM).

Morphological characteristics (small round shape dimension and homogeneous aspect of the mass) and clinical history were more suggestive of a PF in an atypical location.

Therefore, because of the history of ischemic strokes and the high likelihood of embolic complication of masses in that location, we scheduled a hospital admission for surgical removal.

During the inhospital stay, we performed a coronary angiography demonstrating mild nonobstructive coronary atherosclerosis of the proximal left anterior descending artery and angio-computed tomography scan to better evaluate the aortic vessel.

Finally, the patient underwent surgical mass resection [Figure 1]d and replacement of aortic valve, root, and ascending tract sec. Bentall with a mechanical valve conduit. The postoperative period had no complications.

Samples were taken from the mass and underwent histopathological analysis.

The pathology of the specimen confirmed the mass to be a PF [Figure 1]e.


  Discussion Top


PFs are rare cardiac tumors composed of collagen and elastic fibers, layered with endothelium.[4] They are mostly located (>80%) on valvular surfaces,[5] more commonly on left side cardiac valves (44% on the aortic valve and 35% on the mitral valve);[6] therefore, in the present knowledge, PFs arising from LVOT are rare and mostly anecdotally descripted.[6]

In the literature, they are usually found as incidentalomas in asymptomatic patients[7] or by routine echocardiographic examination for different clinical reasons. Some cases presented with symptomatic cerebral embolization with transient ischemic attack or stroke[8],[9] or with cardiac embolization manifesting with unstable angina/non-ST segment elevation myocardial infarction.[10]

The most frequent differential diagnosis is CM. Although definitive confirmation is only achieved by pathological examination of the specimen, echocardiography plays a relevant role in the first diagnostic suspicion. It allows to evaluate the location of the mass (usually CMs are located in the left atrium with a stalk attached to the fossa ovalis) and its morphological characteristics (CMs usually have an appearance of an heterogeneous mass, with calcifications seen in 14% of patients).[4]

In the majority of cases, the treatment of choice is surgical excision of the mass regardless of the symptoms[7],[8],[9],[10] due to its high embolic risk. Particular attention should be given to the timing of cardiac surgery in patients with ischemic stroke because of the risk of cerebral hemorrhagic complications while on systemic heparinization needed for cardiopulmonary bypass.

No data of these patients' follow-up are available in the literature, although the risk of relapse in these tumors is minimal.

In conclusion, cardiac fibroelastomas in LVOT are extremely rare but are at high risk for serious complications.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD, Tazelaar HD, et al. Surgical treatment of cardiac papillary fibroelastoma: A single center experience with eighty-eight patients. Ann Thorac Surg 2005;80:1712-8.  Back to cited text no. 1
    
2.
Habertheuer A, Laufer G, Wiedemann D, Andreas M, Ehrlich M, Rath C, et al. Primary cardiac tumors on the verge of oblivion: A European experience over 15 years. J Cardiothorac Surg 2015;10:56.  Back to cited text no. 2
    
3.
Cianciulli TF, Soumoulou JB, Lax JA, Saccheri MC, Cozzarin A, Beck MA, et al. Papillary fibroelastoma: Clinical and echocardiographic features and initial approach in 54 cases. Echocardiography 2016;33:1811-7.  Back to cited text no. 3
    
4.
Tyebally S, Chen D, Bhattacharyya S, Mughrabi A, Hussain Z, Manisty C, et al. Cardiac tumors: JACC CardioOncology state-of-the-art review. JACC CardioOncol 2020;2:293-311.  Back to cited text no. 4
    
5.
Siddiqui A, Rehman S. Cardiac papillary fibroelastoma: A rare cause of cardioembolic stroke. Neurol India 2022;70:380-3.  Back to cited text no. 5
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6.
Stiru O, Geana RC, Dragulescu PR, Tulin A, Raducu L, Bacalbasa N, et al. Transapical left ventricular approach for cardiac papillary fibroelastomas: A case report. In Vivo 2020;34:3681-5.  Back to cited text no. 6
    
7.
Coats CJ, Reid J, Wright S, Sonecki P, Al-Attar N. Papillary fibroelastoma arising from left ventricular outflow tract. Eur Heart J Cardiovasc Imaging 2018;19:826.  Back to cited text no. 7
    
8.
Outomuro D, del Riego H, Grana DR, Milei J. Unusual location in the left ventricular outflow tract and atypical symptoms of cardiac papillary fibroelastoma. J Cardiovasc Med (Hagerstown) 2006;7:768-70.  Back to cited text no. 8
    
9.
Sekar B, Vohra H, Nishtar S, Ehtisham J. Non-valvular left ventricular papillary fibroelastoma in a patient with recurrent stroke. Echo Res Pract 2015;2:I7-8.  Back to cited text no. 9
    
10.
Vivacqua A, Shafii A, Kalahasti V, Tan C, Gonzalez-Stawinski G. Images in cardiology. Ventricular outflow tract papillary fibroelastoma presenting with non-ST-segment elevation myocardial infarction. J Am Coll Cardiol 2010;55:2607.  Back to cited text no. 10
    


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