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\begin{document}

\title{Double Inlet Left Ventricle with common AV valve, discordant
ventriculo-arterial connection and pulmonary atresia: A rare anomaly on
fetal echocardiogram with three dimensional spatio-temporal imaging
correlation (STIC)}



\author[1]{Usha Nandhini Sennaiyan}%
\author[1]{Mani Ram Krishna}%
\affil[1]{Dr R.K. Hospital for Women and Children}%


\vspace{-1em}



  
  \date{June 17, 2024}


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\selectlanguage{english}
\begin{abstract}
Double Inlet Left Ventricle through a common AV valve is a rare type of
functionally univentricular heart. We report a fetus with double inlet
left ventricle with discordant ventriculo-arterial connection and
pulmonary atresia. The anatomy was readily apparent on three dimensional
rendering by spatio-temporal imaging correlation technique(STIC)%
\end{abstract}%



\sloppy


\textbf{Double Inlet Left Ventricle with common AV valve, discordant
ventriculo-arterial connection and pulmonary atresia: A rare anomaly on
fetal echocardiogram with three dimensional spatio-temporal imaging
correlation (STIC)}

Usha Nandhini Sennaiyan and Mani Ram Krishna

Dr R.K. Hospital for Women and Children

Thanjavur, Tamil Nadu, India

Correspondence to

Dr Mani Ram Krishna

Dr R.K. Hospital for Women and Children,

No 7, V.O.C.Nagar, Thanjavur -- 613007.

Tamil Nadu, India.

E-mail: mann\_comp@hotmail.com

\textbf{Conflict of Interest:} The authors report no conflict of
interest with regard to this work.

\textbf{Funding:} There was no external funding involved in the
submitted work

Abstract:

Double Inlet Left Ventricle through a common AV valve is a rare type of
functionally univentricular heart. We report a fetus with double inlet
left ventricle with discordant ventriculo-arterial connection and
pulmonary atresia. The anatomy was readily apparent on three dimensional
rendering by spatio-temporal imaging correlation technique (STIC)

A pregnant woman was referred to us at 21 weeks gestational age for
evaluation of a suspected congenital heart disease. On fetal
echocardiogram, there was usual arrangement of the abdominal organs and
the atria. The heart was in the left side of the chest with the apex
pointing to the left. The systemic and pulmonary veins drained normally.
There was a large primary type of atrial septal defect (Fig 1A). There
was univentricular atrio-ventricular (AV) connection to a dominant left
ventricle through a common AV valve (Fig 1A and Video 1). A small
incomplete ventricle was imaged anterior and to the left of the dominant
ventricle (Fig 1 B and Video 2). The aorta arose from the incomplete
ventricle (Fig 1D) and was unobstructed. There was pulmonary atresia
with retrograde flow in the arterial duct to the pulmonary trunk (Fig 1D
and Video 3). The anatomy was readily apparent on three dimensional
rendering by spatio-temporal imaging correlation (STIC) technique ( Fig
1B and Videos 1 to 3)

Double Inlet Left Ventricle is the commonest type of functionally
univentricular heart with univentricular AV connection (1). The most
common types of DILV have discordant ventriculo-arterial connections
(2). A very small proportion of DILV have a primary type of atrial
septal defect with the AV junction guided by a common AV valve (3). To
the best of our knowledge, this anatomy has not been reported previously
during a fetal echocardiogram.

Figure Legends:

Figure 1A: A fetal echocardiogram image in the 4-chamber view
demonstrating the inter-atrial septum and a large primary type atrial
septal defect (green star). The atria are connected to a single dominant
ventricle through a common AV valve

Figure 1B: An image from a three-dimensional rendering of the fetal
heart by spatio-temporal imaging correlation (STIC) demonstrating the
incomplete ventricle which is present anterior and to the left of the
dominant ventricle

Figure 1C: A fetal echocardiogram image demonstrating an unobstructed
aortic outflow arising from the incomplete ventricle (RV)

Figure 1D: A fetal echocardiogram image from the three-vessel tracheal
view demonstrating reversal of flow (red color) in a vertically aligned
arterial duct

Video 1: A three-dimensional fetal echocardiogram loop rendered by
spatio-temporal imaging correlation (STIC) demonstrating the common AV
valve when viewed from the ventricular aspect

Video 2: A three-dimensional fetal echocardiogram loop rendered by
spatio-temporal imaging correlation (STIC) demonstrating the dominant
left ventricle and the incomplete ventricle from which the aorta arises

Video 3: A three-dimensional fetal echocardiogram loop with color
Doppler rendered by spatio-temporal imaging correlation (STIC)
demonstrating forward flow in the aorta (blue) and reversal of flow in
the arterial duct(red)

References

\begin{enumerate}
\tightlist
\item
  Van Praagh R, Ongley PA, Swan HJ. Anatomic types of single or common
  ventricle in man: morphologic and geometric aspects of 60 necropsied
  cases.~\emph{Am J Cardiol.~} 1964;~~\emph{13} : 367--386
\item
  Khairy P, Poirier N and Mercier L. Univentricular
  Heart.\emph{Circulation.} 2007; 115: 800-812
\item
  Al-Ethawi AESD and Al-Kaaby BA. The univentricular heart:
  Revisited.\emph{Cir Cardiov.} 2018; 25(3): 141-147.
\end{enumerate}\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Fig-1A/Fig-1A}

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\includegraphics[width=0.70\columnwidth]{figures/Fig-1C/Fig-1C}

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\end{figure}\selectlanguage{english}
\begin{figure}[H]
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\includegraphics[width=0.70\columnwidth]{figures/Fig-1D/Fig-1D}

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