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\begin{document}

\title{Imaging of a Case of Atrioventricular Septal Defect: The added value of
using the Third Dimension}



\author[1]{Hoda Shehata}%
\author[2]{Mahmoud Abdelnabi}%
\author[1]{Abdallah Almaghraby}%
\author[1]{Mohamed Abdel-Hay}%
\affil[1]{Alexandria University Faculty of Medicine}%
\affil[2]{Alexandria University Medical Research Institute}%


\vspace{-1em}



  
  \date{May 22, 2024}


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\begin{abstract}
Atrioventricular septal defects (AVSD) entails a wide range of
morphological anomalies including the interatrial and interventricular
communications and the atrioventricular (AV) valve(s). However, ``En
face ''visualization of these anomalies could not be done using standard
two-dimensional echocardiography. Therefore, we report a case that
highlights the role of three-dimensional echocardiography in imaging
AVSDs.%
\end{abstract}%



\sloppy


\textbf{Case presentation:}

24-years old male patient presented to our medical facility complaining
of exertional dyspnea and easy fatigability for 4 years. On clinical
examination, a loud systolic murmur is heard over the left parasternal
area. 2D Transthoracic Echocardiography (2D TTE) revealed a large ostium
primum atrial septal defect (ASD), a cleft in the left and right
atrioventricular valves with mild regurgitation and a small restrictive
inlet ventricular septal defect (VSD). Marked hypertrophy of the right
ventricular free wall with increased systolic gradient across the right
ventricular outflow tract (RVOT) to 50 mmHg. 3D Transesophageal
echocardiography (TOE) with zoomed and full volume mode acquisition
revealed a common AV valve with common annulus with clear visualization
of the five leaflets. Moreover, the cleft in the left AV valve is
directed towards the interventricular septum (IVS) with small coaptation
gap seen at the central orifice. An oval-shaped large ostium primum ASD
is seen from both the right and left atrial perspectives. Multiple small
fenestrated VSDs are seen in the inlet septum. Associated infundibular
hypertrophy is noted with normal pulmonary valve orifice (Arrow) (Figure
1, Video 1,2). So, we report a case of complete AVSD associated with
infundibular stenosis. The patient was referred for surgical repair of
the AV valve, closure of the ASD and VSDs and reconstruction of the
RVOT.

Although being one of the common defects encountered by pediatric
cardiologists and echocardiographers, imaging of the AVSD associated
defects remains challenging.\textsuperscript{(1)} Residual cleft and
valve dysplasia are one of the common causes for further
operations.\textsuperscript{(2,3)} With the help of 3D echocardiography
, fine anatomical details of the AV valve(s) and associated defects of
AVSDs can be clarified .\textsuperscript{(4)} Accordingly, 3D
echocardiography is highly recommended to provide a clear picture to the
surgeon to avoid future reinterventions.

\textbf{Disclosure: All authors declare no conflict of interest}

\textbf{References:}

\begin{enumerate}
\tightlist
\item
  Smallhorn J.F. Cross-sectional echocardiographic assessment of
  atrioventricular septal defect: basic morphology and preoperative risk
  factors. Echocardiography. 2001; 18: 415-32
\item
  Barrea C. , Levasseur S. , Roman MK , Nii M. Three-dimensional
  echocardiography improves the understanding of left atrioventricular
  valve morphology and function in atrioventricular septal defects
  undergoing patch augmentation J Thorac Cardiovasc Surg 2005;129:746-53
\item
  Lange A, Mankad P, Walayat M, Palka P, Burns JE, Godman MJ.
  Transthoracic three-dimensional echocardiography in the preoperative
  assessment of atrioventricular septal defect morphology .Am J
  Cardiol.2000;85:630-5
\item
  Kutty S. , Smallhorn J.F. Evaluation of atrioventricular septal
  defects by three-dimensional echocardiography: benefits of navigating
  the third dimension J Am Soc Echocardiogr . 2012 ;25:932-44
\end{enumerate}

Figure 1: (Panel A): 2D TOE (RV inflow-outflow view) shows marked RVOT
hypertrophy. (Panel B, C): 2D TOE and TTE (Four chamber views) shows a
large ostium primum ASD. (Panel D): Zoomed mode of the AV valve from the
ventricular perspective confirms the presence of a common AV annulus
with five leaflets (superior and inferior bridging leaflets, left mural
leaflet and anteroseptal and posterior leaflets). (Panel E, F): Zoomed
mode of the IAS from both left and right atrial perspectives shows an
oval-shaped ostium primum ASD.

Video 1: 3D TOE, Zoomed mood of the common AV valve from ventricular
perspective

Video 2: 3D TOE (Full volume, 4 beat acquisition) shows multiple
fenestrated inlet VSDs and marked RVOT hypertrophy.\selectlanguage{english}
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