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\begin{document}

\title{Heterotaxy pattern associated with Sinus Node Dysfunction in an adult: A
case report.}



\author[1]{naman shah}%
\author[2]{sankalp acharya}%
\author[2]{apporva tripathi}%
\author[2]{himanshi bisht}%
\author[1]{maitri shah}%
\author[3]{aayushi pareek}%
\author[3]{asmita gera}%
\author[4]{Abhigan  Shrestha}%
\author[5]{Vikash Jaiswal}%
\affil[1]{GMERS Medical College and Hospital}%
\affil[2]{BJ Medical College}%
\affil[3]{RUHS College of Dental Sciences}%
\affil[4]{M Abdur Rahim Medical Medical College Hospital}%
\affil[5]{AMA School of Medicine}%


\vspace{-1em}



  
  \date{May 19, 2024}


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\textbf{Heterotaxy pattern associated with Sinus Node Dysfunction in an
adult: A case report.}

\textbf{Authors} : Naman Shah MBBS\textsuperscript{1}, Sankalp
Acharya\textsuperscript{2}, Apoorva Tripathi MBBS\textsuperscript{2},
Himanshi Bisht MBBS\textsuperscript{2},

Maitri Shah MBBS\textsuperscript{1}, Aayushi Pareek
MBBS\textsuperscript{3}, Asmit Gera MBBS\textsuperscript{4}, Abhigan
Babu Shrestha, MBBS\textsuperscript{5}, Vikash Jaiswal
MD\textsuperscript{6}

1. GMERS Medical college Sola, Ahmedabad, India

2. B. J. Medical College, Ahmedabad, India

3. RHUS College of Medical Science, Jaipur, Rajasthan

4. JCCR Cardiology Research, Varanasi, India

5. M Abdur Rahim Medical College, Dinajpur, Bangladesh

6. AMA School of Medicine, Makati, Philippines

\textbf{Corresponding Author}

Abhigan Babu Shrestha

M Abdur Rahim Medical College, Dinajpur, Bangladesh

Abigan17@gmail.com

\textbf{Acknowledgment:} None

\textbf{Source of funding:} None

\textbf{Patient Consent:} Written informed consent was obtained from the
patient to publish this case report

and accompanying images. A copy of the written consent is available for
review by the Editor-in-Chief

of this journal on request.

\textbf{Conflict of Interest:} None

\textbf{Authors contribution:} Conceptualization: N.S, S.A; Writing -
original draft preparation: A.T, N.S,

S.A, H.B, A,P; Writing - review and editing: V.J, A.B.S., A.G; Funding
acquisition: None

\textbf{Abstract:}

This case report presents a 26-year old male patient brought to the
medical ward with an experience of repeating blackout for a year. The
patient was diagnosed with sick sinus syndrome, and further
investigations revealed left isomerism, polysplenia, and no congenital
heart defects. Holter monitoring, ultrasonography, electrocardiography,
and computed tomography were used to confirm the diagnosis. The patient
underwent DDDR pacemaker implantation for the treatment of SA node
dysfunction. The report highlights the variability of anatomical
findings associated with polysplenia pattern and the various types of
heartbeat disruptions that may occur in the atrial appendages of the
left side isomerism.

\textbf{Key Clinical Message:}

\begin{itemize}
\tightlist
\item
  A 26-year old male patient admitted to the hospital ward with
  experience of repetitive syncopes for a year.
\item
  The patient was diagnosed with sick sinus syndrome.
\item
  The aim of this clinical report is to highlight the variability of
  anatomical findings associated with polysplenia pattern .
\end{itemize}

\textbf{Keywords:} Polysplenia Syndrome, SA node dysfunction, Pathology

\textbf{INTRODUCTION}

Heterotaxy pattern, also called Situs ambiguous, is a medical condition
characterised by an atypical arrangement of the internal organs in the
Thorax and abdomen. This disease can lead to complicated congenital
deformities that affect various organ systems, such as the circulatory,
pulmonary, digestive, reproductive and the urinary system systems. Situs
inversus totalis is a rare congenital condition in which the internal
organs of the body are asymmetrically placed, with the heart located on
the opposite side of the body from its normal position. However, other
congenital defects are also known to cause serious health problems.
These include abnormal connections between major blood vessels and the
heart, which can lead to altered blood flow. Pulmonary system is also
affected, lungs have different numbers of lobes than normal anatomical
lungs, and the size of bronchi is also affected. Furthermore, it is also
possible for this medical condition to lead to absence of spleen or the
condition may result in the formation of numerous small and inefficient
spleens (polysplenia) in the abdomen. The liver is located in the center
of the body rather than its usual spot on the right side of the abdomen.
The condition also varies in severity. Some individuals may experience
mild health problems while others may face potentially life-threatening
risks even with treatment during infancy or childhood (1).

The mortality rate for most patients diagnosed with polysplenia syndrome
is alarmingly high, with many not surviving beyond the age of five. This
is largely attributed to the presence of severe cardiac abnormalities.
Yet, a miniscule fraction (5.0-10.0 \%) of people with this syndrome
have normal hearts or minor cardiac abnormalities and can mature without
exhibiting any significant signs. This case presentation is an example
of such an occurrence (2).

Heterotaxy syndrome is classified into two main categories: right
isomerism and left isomerism. Left Isomerism is a condition in which
paired organs are located on the left side of the body, whereas in Right
Isomerism, paired organs are positioned along the right side, and there
may be an absence of organs on the left. Both versions are correlated
with complicated heart malfunctions. In left isomerism, one can expect
to find left atrial isomerism with bilateral morphologic left atrial
appendages, viscerocardiac heterotaxy, and multiple cardiac
malformations such as congenital heart block, atrioventricular septal
defects and pulmonary stenosis, multiple polysplenia, bilobed lungs on
the left side with hyparterial bronchi, intestinal malrotation,
non-random genitourinary malformations, and an interruption of the
inferior vena cava (IVC) accompanied by azygos continuation. (3).
Hereby, we report Sick sinus syndrome in a 26-year-old man who was
admitted to our clinic with complaints of syncope (sick sinus syndrome)
and found to have left isomerism on the investigation.

\textbf{Case presentation}

A 26-year- old age male patient presented to our medical facility with a
history of multiple episodes of fainting scattered throughout the
previous year. Upon further inquiry, the patient mentioned that they had
experienced three episodes of fainting within a period of one year. On
examination, her pulse was at 40/min with an irregular rhythm. Her blood
pressure (BP) reading registered as 140/90 mmHg. The cardiovascular
system examination showed no abnormalities. Further investigations
showed that the full blood count and sugar levels were in the normal
range. Additionally, the kidney function tests, serum electrolytes and
liver tests all yielded normal results. The electrocardiography (ECG)
results showed the presence of sinus node arrest accompanied by a
junctional escape that was recorded at a rate of 35 to 40 beats per
minute \textbf{(Figure 1).} Holter monitoring was performed to verify
the diagnosis and identify any presence of AV blockage or alternate
rhythm disturbances that might result in syncope. The test results
confirmed Sick Sinus Syndrome, which showed pauses and arrest in sinus
rhythm along with junctional escape beats/rhythm \textbf{(Figure 2).} An
ultrasonography revealed a midline liver that was more towards the left
side, with a leftward hilum and normal hepatic veins and inferior vena
cava on the right side. Additionally, no spleen was identified on the
left side, in accordance with situs ambiguous abdominis.
Echocardiography revealed normal left ventricular ejection fraction and
no congenital heart defects. An HRCT chest with upper abdomen was
conducted on a 128-slice dual source dual energy scanner. The scan
results revealed the presence of liver on the left side of the abdomen
and in the midline stomach. (Figure 3A). The abdomen contained numerous
accessory spleens, at least 7. A bilateral trilobed lung with
hyparterial bronchus is seen (Figure 3B). Situs ambiguous with
polysplenia syndrome was diagnosed on CT. (Figure \textbf{3C).} The
patient received a DDDR (dual-pacing dual-sensing dual-response
rate-adaptive) pacemaker for sinus node dysfunction. The patient
responded well, with improvement in symptoms and findings at follow-up.

\textbf{Discussion}

Polysplenia is a syndrome that is characterized by various anatomical
findings, and it can be quite complex. In cases where there is isomerism
of the left atrial appendages, individuals may experience one of three
types of heart rhythm disturbances. These include atrioventricular block
(4,5), dual atrioventricular nodal pathways and sinus node dysfunction
(6). Patients with Polysplenia syndrome have been found to exhibit
different mutations, such as LEFTYA3, Z1C3 etc. (6) Our patient, on the
other hand, did not exhibit or undergo any study for such mutations.

The reported patient was an adult who had been diagnosed with
polysplenia syndrome, had a structurally normal heart, and suffered from
sinus node dysfunction. It is worth noting that the occurrence of
isolated conduction disorder in patients with polysplenia syndrome and a
structurally normal heart is quite rare and unusual (5).

When it comes to patients who have sinus node dysfunction, using AAI C/R
and DDD C/R pacemakers for electrical stimulation yields a better
outcome due to the reduced incidence of atrial fibrillation,
thromboembolic events, and heart failure. This is supported by research
findings mentioned in the source (3).

IVC interruption with azygous continuation is seen in left isomerism and
affects the course of catheter or TPI (thrombolytic predictive
instrument) lead while performing temporary pacemaker implantation from
femoral vein access. Alternatively, one can choose for internal jugular
vein access for TPI.

While doing TPI, one must be aware of the presence of left sided SVC and
pacemaker lead course accordingly.

\textbf{Conclusion}

Left isomerism is an important etiology of Sinus node dysfunction in
young patients and should be evaluated thoroughly. Diagnosis of left
isomerism in sick sinus syndrome will affect the management of patients
with regards to the mode and access of pacemaker implantation.

\textbf{Acknowledgment:} None

\textbf{Source of Funding:} None

\textbf{Patient Consent:} The patient provided written consent for the
publication of this case report and its accompanying images, and a copy
of this consent is available upon request for review by the
Editor-in-Chief of this journal, to ensure compliance with ethical
standards.

\textbf{Conflict of Interest:} None

\textbf{REFERENCE}

1. Heterotaxy syndrome: MedlinePlus Genetics {[}Internet{]}. {[}cited
2023 Apr 8{]}. Available from:
https://medlineplus.gov/genetics/condition/heterotaxy-syndrome/

2. Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al.
Polysplenia syndrome detected in adulthood: report of eight cases and
review of the literature. Abdom Imaging. 1999;24(2):178--84.

3. Berg C, Geipel A, Kamil D, Kn\selectlanguage{ngerman}üppel M, Breuer J, Krapp M, et al. The
Syndrome of Left Isomerism. Journal of Ultrasound in Medicine
{[}Internet{]}. 2005 {[}cited 2023 Apr 8{]};24(7):921--31. Available
from: https://onlinelibrary.wiley.com/doi/abs/10.7863/jum.2005.24.7.921

4. Fukuda K, Onda T, Kimura Y, Miura S, Matsumori R, Masaki Y, et al. An
adult case of polysplenia syndrome associated with sinus node
dysfunction, dextrocardia, and systemic venous anomalies. Intern Med.
2015;54(9):1071--4.

5. Momma K, Takao A, Shibata T. Characteristics and natural history of
abnormal atrial rhythms in left isomerism. Am J Cardiol. 1990 Jan
15;65(3):231--6.

6. Zhu L, Belmont JW, Ware SM. Genetics of human heterotaxias. Eur J Hum
Genet. 2006 Jan;14(1):17--25.

\textbf{Figure Legend:}

\textbf{Figure 1:} Electrocardiography shows sinus node arrest with a
junctional escape at 35 -- 40 bpm.

\textbf{Figure 2} : Holter shows sinus pause and sinus arrest with
junctional escape beats/rhythm, confirming the diagnosis of sick sinus
syndrome).

\textbf{Figure 3A:} CT scan (HRCT) of the chest with upper Abdomen shows
evidence of liver on the left side of the abdomen and midline stomach.

\textbf{Figure 3B:} CT scan of the chest (HRCT) with upper Abdomen shows
Bilateral trilobed lung with hyparterial bronchus.\textbf{Figure 3C:} CT
scan on axial view shows evidence of liver on the left side of the
abdomen and midline stomach. Multiple accessory spleens (at least 7) are
seen in the abdomen.

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