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.