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\begin{document}

\title{Partial atrioventricular septal defect with left atrioventricular valve
aneurysm mimicking valve perforation}



\author[1]{Marien Lenoir}%
\author[2]{Holy Ranaivoson}%
\author[3]{Anne Claire Casalta}%
\author[1]{Loïc Macé}%
\author[3]{Philippe Aldebert}%
\author[3]{Alexis THERON}%
\affil[1]{Assistance Publique Hopitaux de Marseille}%
\affil[2]{Universite de Fianarantsoa}%
\affil[3]{Hopital de la Timone - Deparment de Cardiologie}%


\vspace{-1em}



  
  \date{May 05, 2024}


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\selectlanguage{english}
\begin{abstract}
Left atrioventricular valve aneurysm is rare. We present a rare case of
partial atrioventricular septal defect with an extremely thin left
atrioventricular valve aneurysm mimicking valve perforation. The
preoperative echocardiography demonstrated severe left sided
atrioventricular valve regurgitation on the ``cleft'' and leaflet
perforation. But in per-operative, we discovered a left sided
atrioventricular valve aneurysm instead of valve perforation. The
``cleft'' edge and a aneurysm were closed.%
\end{abstract}%



\sloppy


\textbf{Partial atrioventricular septal defect with left
atrioventricular valve aneurysm mimicking valve perforation}

Marien Lenoir \textsuperscript{1}, Holy Ranaivoson \textsuperscript{2},
Anne Claire Casalta \textsuperscript{3}, Lo\selectlanguage{ngerman}ïc Macé \textsuperscript{1},
Philippe Aldebert \textsuperscript{3}, Alexis Theron \textsuperscript{3}

1 Department of Cardiac Surgery, Hôpital Timone, AP-HM, Marseille,
France

2 Department of Cardiology, CHU Tambohobe Fianarantsoa, Antananarivo,
Madagascar

3 Department of Cardiology, Hôpital Timone, AP-HM, Marseille, France

Corresponding author:

Marien Lenoir

Department of Pediatric Cardiac Surgery,

264 Rue Saint-Pierre

13385 Marseille Cedex 5 France

number: 04 91 38 00 00

marien.lenoir@ap-hm.fr

Total word count: 996

\textbf{Keywords:}

partial atrioventricular septal, mitral valve aneurysm, aneurysm, mitral
valve, mitral valve cleft

Abstract

Left atrioventricular valve aneurysm is rare. We present a rare case of
partial atrioventricular septal defect with an extremely thin left
atrioventricular valve aneurysm mimicking valve perforation. The
preoperative echocardiography demonstrated severe left sided
atrioventricular valve regurgitation on the ``cleft'' and leaflet
perforation. But in per-operative, we discovered a left sided
atrioventricular valve aneurysm instead of valve perforation. The
``cleft'' edge and a aneurysm were closed.

Introduction

Left atrioventricular valve aneurysm is rare (1). Left atrioventricular
valve aneurysm is most common in-patient with infective endocarditis
(2). Left atrioventricular valve aneurysm is usually reported as large
and well-defined aneurysm by transthoracic echocardiography (2). We
present a rare case of partial atrioventricular septal defect with an
extremely thin left atrioventricular valve aneurysm mimicking valve
perforation. A aneurysm closed by a autologous pericardial patch and
``cleft'' was closed.

Case report

A 48 years-old female with partial atrioventricular septal defect
diagnosed in childhood but not followed. The patient had no previous
history of endocarditis or inflammation. She complained dyspnea
associated with chest pain in exercise. On physical examination, we find
an apical systolic murmur, but she had no signs of heart failure. The
rest of the examination was normal. She had cardiomegaly on the chest X
ray. The trans-esophageal echocardiography (TEE) found (with 3
dimensional):

\begin{itemize}
\tightlist
\item
  Ostium primum defect 15 * 35 mm
\item
  Atrial septal defects~with ostium secundum defect 8mm with exclusive
  left to right shunt,
\item
  Right and left atrial dilatation
\item
  Right ventricular dilatation with right ventricular systolic
  pressure~at 35mmHg
\item
  Left atrioventricular valve (Figure 1, A) with a significant leak: a
  predominant central component on the ``cleft''.
\item
  On left atrioventricular valve, perforation of 3 mm (Figure 1B) in the
  inferior bridging leaflet.
\item
  Left atrioventricular valve ring: 35 * 39 mm.
\item
  Satisfactory subvalvular apparatus with 2 papillary muscles.
\end{itemize}

Coronarography was normal.

She underwent a median full sternotomy. Cardiac arrest was induced with
anterograde cardioplegia, under cardiopulmonary bypass. The left
atrioventricular valve was explored through (ostium primum) and an
aneurysm was discovered on the inferior bridging leaflet (Figure 2 C)
(Figure 2 A). The aneurysm was 5mm x7mm, and the subvalvular apparatus
was normal.

Edge to edge closure of the ``cleft'' was performed (Figure 2 B). The
aneurysm had a narrow neck arising from the 3 mm ostium, and it was
possible to gather all of the emptied aneurysm and simply cover it with
the patch. An autologous pericardium patch sutured over the aneurysm
with running suture (Figure 2 B). The final saline test showed no
leakage. The ostium primum and ostium secondum was closed with an
autologous patch with running suture. TTE demonstrated no left
atrioventricular valve leakage with no aneurvrism. The patient's
postoperative course was uneventful, with a normal sinus rhythm. TTE
showed no left atrioventricular valve regurgitation and no valve
aneurysm at discharge and 1 year.

Comment

In the literature, there only one case report who associated a partial
atrioventricular septal defect with left atrioventricular valve
aneurysm. Imamura and colleagues (7) reported this association, but
valve aneurysm was a tissue aneurysm closing a ventricular septal defect
underneath the atrioventricular valve. The present patient had no
previous history of endocarditis or inflammation. She did not have a
ventricular septal defect. So, the etiology of this patient is different
from those previously reported. In this clinical case, the left
atrioventricular valve regurgitation jet was mainly from this ``cleft''
that was wide open.

Our lesion anatomically resembled a mitral valve aneurysm. Mitral valve
aneurysm is a saccular and bulging structure of the mitral leaflet that
expands on systole and collapses during diastole. Thus a aneurysm of the
mitral valve is rare (1).

They are usually reported as a sequelae of infective endocarditis(3,4).
However, the underlying mechanism for its development is not known.
Probably, they are the result of valvulitis with consequent formation of
granulation tissue and scar tissue that succumbed to intraventricular
pressure with the formation of sac-like outpouchings (5). Mitral valve
aneurysm may be induced by connective tissue diseases like Marfan
syndrome osteogenesis imperfect and pseudoxanthoma elasticum (6).

Left atrioventricular valve aneurysm is a difficult diagnosis to make by
echocardiography and often mimic a valve perforation due to infectious
endocarditis. This mistake could be explained by the thinness of the
aneurysmal membrane and also a lack of resolution of echocardiography.

We recommend surgical management of left atrioventricular valve
aneurysm. This is because left atrioventricular valve aneurysm might be
complicated by rupture, by thromboembolism or endocarditis. When the
left atrioventricular valve repair was not possible due to severely
distorted leaflets or too small healthy part of the valve, we suggest
left atrioventricular valve replacement (5). But, the surgical
management consists at close the left atrioventricular valve aneurysm,
if the ostium of the valve aneurysm is small (\textless{}3mm) direct
suture (8), if it is large (\textgreater{} 3mm) interposition of an
autologous pericardial patch.

In conclusion, left atrioventricular valve aneurysm with partial
atrioventricular septal defect is an unusual case of a valve aneurysm,
with a aneurysm mimicking valve regurgitation by endocarditis. The
autologous pericardium patch can be used to close the large left
atrioventricular valve aneurysm.

Financial Support: We did not need financial support for this study.

Conflict of Interest: Author Marien Lenoir has no conflict of interest.
Author Holy Ranaivoson has no conflict of interest. Loïc Macé has no
conflict of interest. Philippe Aldebert has no conflict of interest.
Anne Claire Casalta has no conflict of interest. Alexis Theron declares
that he has no conflict of interest.

Authors' Statement: The adult patient signed a consent for the
publication of her case report.

Reference

1. Vilacosta I, San Román JA, Sarriá C, Iturralde E, Graupner C, Batlle
E, et al. Clinical, anatomic, and echocardiographic characteristics of
aneurysms of the mitral valve. Am J Cardiol. 1999 Jul 1;84(1):110--3,
A9.

2. Reid CL, Chandraratna AN, Harrison E, Kawanishi DT, Chandrasoma P,
Nimalasuriya A, et al. Mitral valve aneurysm: clinical features,
echocardiographic-pathologic correlations. J Am Coll Cardiol. 1983
Sep;2(3):460--4.

3. Halkos ME, Symbas JD, Felner JM, Symbas PN. Aneurysm of the mitral
valve: a rare complication of aortic valve endocarditis. Ann Thorac
Surg. 2004 Oct;78(4):e65-66.

4. Saphir O, Leroy EP. True aneurysms of the mitral valve in subacute
bacterial endocarditis. Am J Pathol. 1948 Jan;24(1):83--95.

5. Gajjar TP, Desai NB. True aneurysm of anterior mitral leaflet--a rare
entity. J Thorac Cardiovasc Surg. 2012 Sep;144(3):e93-95.

6. Mollod M, Felner KJ, Felner JM. Mitral and Tricuspid Valve Aneurysms
Evaluated by Transesophageal Echocardiography. Am J Cardiol. 1997 May
1;79(9):1269--72.

7. Imura H, Sakamoto S, Maruyama Y, Ochi M, Shimizu K. Two-patch repair
for atrioventricular septal defect with mitral aneurysm. Ann Thorac
Surg. 2009 Oct;88(4):1341--3.

8. Ohira S, Doi K, Yamano T, Yaku H. Successful repair of a mitral valve
aneurysm with cleft of anterior mitral leaflet in an adult. Ann Thorac
Surg. 2013 Dec;96(6):2238--40.

Figure 1: A trans esophageal 3D echocardiogram revealing a ``cleft'' (A)
and left atrioventricular valve perforation (B) mimicking perforation by
endocarditis.

Figure 2: Schema of atrioventricular valve with aneurysm before repair
(A) and after repair (``Cleft'' closure and close aneurysm with
autologous pericardium patch) (B). (C) Intraoperativephotograph of the
left atrioventricular leaflets with aneurysm. The arrow indicate valve
aneurysm.\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure1CAV-f/Figure1CAV-f}

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\end{figure}\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure2-pCAVnew/Figure2-pCAVnew}

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\end{figure}

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