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\begin{document}

\title{Left atrial mitral valve chordae; an important congenital abnormality
mimicking endocarditis on transthoracic echocardiography}



\author[1]{Anita Shirwaiker}%
\author[1]{Ian Hong Siang Goh}%
\author[1]{Luke Kristofer Cieslik}%
\author[1]{Alan Appelbe}%
\affil[1]{University Hospital Geelong}%


\vspace{-1em}



  
  \date{May 27, 2024}


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\selectlanguage{english}
\begin{abstract}
A 59-year-old male was incidentally diagnosed with a left atrial mitral
valve chordae involving the junction of the A1 and A2 mitral valve
leaflets and resulting in moderate mitral regurgitation. The recognition
of this extremely rare congenital malformation prevented over diagnosis
and overtreatment.%
\end{abstract}%



\sloppy


\emph{Left atrial mitral valve chordae; an important congenital
abnormality mimicking endocarditis on transthoracic echocardiography}

\textbf{Authors:} Anita Shirwaiker B.Med\textsuperscript{1}, Ian Hong
Siang Goh MBBS\textsuperscript{1}, Luke Kristofer Cieslik MBChB
Bsc\textsuperscript{1}, Alan Appelbe MBBS FRACP\textsuperscript{1}

1 -- University Hospital Geelong, Barwon Health, Geelong, Victoria,
Australia

\textbf{Data statement}

The data that support the findings of this study are available from the
corresponding author, Dr Alan Appelbe, upon reasonable request.

\textbf{Funding}

This research did not receive any specific grant from funding agencies
in the public, commercial, or not-for-profit sectors.

\textbf{Conflict of interest}

There is no conflict of interest to disclose.

\textbf{Ethics approval}

Local ethics committee approval at Barwon Health, Victoria was obtained
prior to data collection.

\textbf{Patient consent}

Patient information has been deidentified so consent was not obtained.

\textbf{Reproduction of material from other sources}

All material is original therefore no permission was sought from other
sources.

\textbf{Corresponding Author/Address for Correspondence:}

Dr Alan Appelbe

Barwon Health

Bellerine street, Geelong, VIC, 3220

Email: alan.appelbe@barwonhealth.org.au

Phone: (03) 4215 0000

\textbf{Abstract}

A 59-year-old male was incidentally diagnosed with a left atrial mitral
valve chordae involving the junction of the A1 and A2 mitral valve
leaflets and resulting in moderate mitral regurgitation. The recognition
of this extremely rare congenital malformation prevented over diagnosis
and overtreatment.

\textbf{Case report}

An otherwise well 59-year-old male was admitted to hospital with
undifferentiated migratory polyarthralgia, fevers, raised inflammatory
markers and a new pansystolic murmur. Transthoracic echocardiogram was
performed to investigate for infective endocarditis and showed localised
thickening of the anterior mitral valve leaflet with mild prolapse with
moderate mitral regurgitation (see Figure 1). The findings were
suspicious for endocarditis. Left ventricular cavity size and systolic
function were normal. A transoesophageal echocardiogram revealed a left
atrial mitral valve chordae. This was attached from the left atrial
wall, near the ostium of the left atrial appendage, to the anterior
leaflet near the junction of A1 and A2 (see Figures 2 and 3).
Mild-moderate mitral regurgitation was present with multiple jets. The
patient was discharged home with a plan for repeat echocardiography in 2
years to reassess the severity of his mitral regurgitation and left
ventricular function.

Left atrial mitral valve chordae is a rare congenital abnormality of
which is the prevalence is largely unknown. There have been few case
reports worldwide and the clinical significance remains uncertain,
ranging from incidental findings with mild-moderate mitral valve
pathology managed conservatively(1-4) to severe mitral regurgitation
requiring surgical intervention(5-13). There has been an isolated report
of left atrial mitral valve chordae causing complex endocarditis(14). In
our patient, recognition of this congenital abnormality was imperative
to avoid over diagnosis and unnecessary surgical intervention,
particularly given the differential diagnosis of infective endocarditis.

The origin of left atrial mitral valve chordae is proposed to be a
result of a developmental defect during embryogenesis between the
14\textsuperscript{th} and 17\textsuperscript{th} weeks of gestation as
the papillary muscles and chordae develop(4). Left atrial mitral valve
chordae have been identified in children as young as 8-years-old(12),
while one case was only diagnosed at the age of 85-years-old(6). At
59-years-old, our patient was asymptomatic from his valvular pathology
and his finding was incidental. It has been hypothesised that symptoms
may be delayed by progressive growth and dilatation of the left atrium,
resulting in gradual traction of the aberrant chord eventually resulting
in leaflet prolapse(6). Other suggested mechanisms include the chord
holding the free edge of the leaflet in a scallop during diastole with
the atrial kicking motion resulting in the development of mitral
regurgitation(7) and traumatic injury to the free edge of the leaflet
resulting in tethering and restricted mobility(10). Most cases in the
literature describe involvement of the A2 leaflet of the mitral valve(3,
4, 8, 10-13), with one case involving the A3 leaflet(7) and one case
involving P2 leaflet(6). We describe the first case which involves the
junction of A1 and A2.

\textbf{Conclusion}

Left atrial mitral valve chordae is a rare congenital abnormality which
has a broad range of clinical presentations. In our patient it mimicked
a vegetation on transthoracic echocardiography. Recognition of this
pathology is essential to avoid over diagnosis and treatment. In
patients with mild-moderate mitral regurgitation, close follow-up with
serial echocardiography is recommended given the risk of progression to
severe mitral regurgitation.

\textbf{Figure 1 --} Transthoracic echocardiogram showing thickening on
anterior mitral valve leaflet, suspicious for infective endocarditis\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 2 --} Transoesophageal echocardiogram showing left atrial
mitral valve chordae attached from the left atrial wall, near the ostium
of the left atrial appendage, to the anterior leaflet of the mitral
valve.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image2/image2}

\end{center}
\end{figure}

\textbf{Figure 3} -- 3D transoesophageal echocardiogram showing left
atrial mitral valve chordae attached to the anterior leaflet of the
mitral valve at the junction of A1 and A2\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}

\emph{}

\emph{References}

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9. Taglieri C, Botta L, Roghi A, Alberti A. Unusual insertion of a
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10. Dawson D, Mankad P, Bloomfield P, Boon NA. An unusual cause of
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11. Ivanova V, Anreddy S, Bailey S, Schuett A, Hughes-Doichev R. A case
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12. Taqatqa AS, Bokowski JW, Polimenakos AC. Congenital anomalous
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insufficiency in children. J Card Surg. 2010;25(5):584-5.

13. Khan H, Chaubey S, Kenny C, MacCarthy P, Wendler O. A rare case of
an aberrant anterior mitral valve chord resulting in severe mitral
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14. Chhabra SK, Bogar LJ, DeCaro MV, Cohen IS. Complex mitral valve
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2012;60(22):2330.

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