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.