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\begin{document}

\title{Mitral degenerative valve leaflets suspected as primary valvular tumor:
a case report}



\author[1]{Shiqiang Wang}%
\author[1]{Huaidong Chen}%
\author[1]{Ximing Qian}%
\author[1]{Fan He}%
\affil[1]{Department of Cardiac Surgery Sir Run Run Shaw Hospital School of Medicine Zhejiang University Hangzhou China}%


\vspace{-1em}



  
  \date{May 15, 2024}


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\begin{abstract}
\textbf{Introduction:} Echocardiography plays an important role in the
diagnosis of heart disease. However, sometimes misdiagnosis information
is also provided. \textbf{Methods and Results:} We report a rare case of
preoperative echocardiography misdiagnosed as a primary mitral valvular
tumor with severe regurgitation. During the surgery procedure, the true
lesion was found to be mitral valve leaflet prolapse due to degenerative
mitral valve disease. \textbf{Conclusions:} For individual patient, it
may need to combine clinical symptoms and wide use of echocardiography
and CT or MRI to make the optimal clinical decision.%
\end{abstract}%



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\textbf{Introduction}

Primary valvular tumors and valve degeneration are generally easy to
distinguish, but in some special cases, the two have a certain
similarity in echocardiography, especially for patients with atypical
symptoms, it is often misdiagnosed one for another. Here, a case of
mitral valve degenerative disease suspected to be diagnosed as primary
valvular tumor was revealed.

\textbf{Case Report}

Due to recurrent chest tightness and shortness of breath, the patient
came to the emergency department of our hospital. Transthoracic
echocardiography revealed mitral leaflet cystic nodules, suspected
benign tumor or infective endocarditis with vegetations, and mitral
valve with severe regurgitation. The patient had no other comorbidities
except hypertension. Systolic murmur in apical region was heard.
Transesophageal echocardiography revealed the presence of multiple
cystic structures with significant separation in the A1, C1, and P1
leaflets of the mitral Carpentier nomenclature\textsuperscript{1}. The
larger one was located in the P1 area, about 18.3*14.2mm in size, and
smaller in the A1 area about 9.9*5.4mm (Figure 1). The conclusion of
ultrasonography showed that primary valve tumor should be considered
first after excluding infective endocarditis. We had done a lot of
examinations related to infective endocarditis, such as cranial Computed
Tomography (CT), brain magnetic resonance imaging (MRI) and blood
culture, etc. But our case did not fulfill the modified Duke criteria
for the clinical diagnosis of infective endocarditis. Then we planned to
perform valve tumor resection and mitral valve repair. However, no
tumors were occupied and no infectious vegetations were found on the
valve leaflets during the operation. The cystic structure described by
ultrasound was caused by prolapse due to leaflet degeneration (Figure
2). In order to maintain the integrity of the leaflet structure, we
performed folding repair for the mitral leaflet. The patient was
recovered well after operation, and the result of echocardiography
evaluation was satisfactory.

\textbf{Discussion}

Echocardiography has evolved rapidly since its advent in the 1950s and
is now the modality of choice for imaging occupying cardiac lesions. It
provides high-quality, real-time images, which are invaluable in the
evaluation of cardiac masses. Although transthoracic echocardiography is
an excellent initial diagnostic technique for the evaluation and
diagnosis of cardiac masses, transesophageal echocardiography provides
superior image resolution and better visualization of cardiac masses in
patients with poorly studied transthoracic
echocardiography\textsuperscript{2}. For some diseases with quite
similar echocardiographic presentation need to differential diagnosis
carefully, especially for cases where the symptomatic presentation
cannot be clearly identified\textsuperscript{3}. The treatment options
differ greatly between the two groups of potentially confusing diseases,
such as the primary valve tumors and valve degeneration in this case.

Primary cardiac tumors are fairly uncommon, with an average incidence
rate about 0.02\%, while primary valvular tumors account for only 10\%
among them. Among the more common types are myxoma, papillary
fibroelastoma, and lipoma\textsuperscript{4,5}. The features of
echocardiogram are often pedunculate, often a solitary mass, usually at
the mid-portion of valve leaflets, and with a frond-like characteristic
surface\textsuperscript{6}. The primary valvular tumors need to be
surgically removed if there is mobility, even for asymptomatic patients,
because of the potential cerebral and cardiac
embolization\textsuperscript{7,8}.Valvular degenerative disease due to
the absence of fibrin in the valve leads to lengthy chordae tendineae
and leaflet prolapse, which often causes different degrees of valvular
regurgitation\textsuperscript{9,10}. In this case, the apex systolic
murmur was consistent with the degree of echocardiographic mitral valve
regurgitation.

Generally, only patients with severe mitral regurgitation combined with
symptoms of cardiac insufficiency require surgery. This patient had
severe mitral regurgitation before operation, and the wrong judgment
before operation did not cause any adverse effect. However, for
suspected valve masses without valve regurgitation, more accurate
evaluation and identification of primary valvular tumors and valve
degenerative lesions is clearly warranted. For individual patient, it
may need to combine clinical symptoms and wide use of echocardiography
and CT or MRI to make the optimal clinical decision.

\textbf{Consent for publication}

Informed written consent was obtained from the patient for publication
of this case and accompanying images.

\textbf{The authors have no conflicts of interests to disclose.}

\textbf{Author contributions:}

Writing-original draft: Shiqiang Wang;

Writing-review \& editing: Huaidong Chen; Ximing Qian;

Conceptualization and Supervision: Fan He;

\textbf{Reference}

1. Carpentier A, Relland J, Deloche A, et al. Conservative management of
the prolapsed mitral valve. \emph{Ann Thorac Surg.} 1978;26(4):294-302.

2. Ragland MM, Tak T. The role of echocardiography in diagnosing
space-occupying lesions of the heart.\emph{Clin Med Res.}
2006;4(1):22-32.

3. Auriti A, Chieffi M, Cianfrocca C, et al. Giant vegetation of the
mitral valve simulating primary cardiac tumor. \emph{Echocardiography.}
2004;21(2):183-185.

4. Georghiou GP, Vidne BA, Sahar G, Sharoni E, Fuks A, Porat E. Primary
cardiac valve tumors. \emph{Asian cardiovascular \& thoracic annals.}
2010;18(3):226-228.

5. Issa OM, Reyna J, Santana O, DeBeer S, LaPietra A, Lamelas J. Cardiac
angiofibroma: a rare primary cardiac tumor. \emph{J Card Surg.}
2013;28(4):404-405.

6. Prifti E, Ikonomi M, Veshti A, Demiraj A, Xhaxho R. Papillary
fibroelastoma of the anterior leaflet of the mitral valve mimicking
vegetation. \emph{Int J Surg Case Rep.}2015;14:19-22.

7. Pacini D, Farneti PA, Leone O, Galli R. Cardiac papillary
fibroelastoma of the mitral valve chordae.\emph{Eur J Cardiothorac
Surg.} 1998;13(3):322-324.

8. Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac
papillary fibroelastoma: a comprehensive analysis of 725 cases. \emph{Am
Heart J.}2003;146(3):404-410.

9. Del Forno B, Ascione G, De Bonis M. Advances in Mitral Valve Repair
for Degenerative Mitral Regurgitation: Philosophy, Technical Details,
and Long-Term Results. \emph{Cardiology clinics.} 2021;39(2):175-184.

10. Michelena HI, Topilsky Y, Suri R, Enriquez-Sarano M. Degenerative
mitral valve regurgitation: understanding basic concepts and new
developments. \emph{Postgraduate medicine.} 2011;123(2):56-69.

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