Temporary pacemaker insertion leads to diagnosis of a rare s
This case has been published in IHJ cardiovascular case reports.

A 65-year-old female patient was admitted to the hospital with a history of recurrent syncope of one year duration. On enquiry, patient gave history of three episodes of syncope over past one year.

On examination, she was having pulse rate of 50/min with regular rhythm. Her blood pressure (BP) was 140/90 mmHg. Cardiovascular system examination was normal. Her routine blood investigations and electrolytes were normal. Electrocardiography (ECG) showed complete heart block with a junctional escape rhythm at 44 per minute.

Patient was taken up for temporary pacemaker insertion (TPI). IVC angiogram showed interruption of right IVC. Ultrasonography (USG) showed liver in midline and more towards left side, hilum leftward, but inferior vena cava (IVC) and hepatic veins on right side and were normal. No spleen on left side was identified consistent with situs ambiguous abdominis. Contrast-enhanced computed tomography (CECT) abdomen was performed on a 128 slice dual source dual energy scanner and it showed evidence of liver, gallbladder, IVC and common bile duct on left side of abdomen. Multiple accessory spleens (at least 7) were seen in the abdomen. A final impression of situs ambigus with polysplenia syndrome was made on CECT abdomen.

Some key points
1. Polysplenia is a complex syndrome with a high variability of anatomical findings. It was first described by Abernethy in 17931 and a full description was given by Moller et al in 1967
2. In the present case, the diagnosis of this syndrome was made retrospectively due to unusual encounter with the interrupted IVC during TPI insertion. This is quite unusual.
3. Patients of polysplenia syndrome with structurally normal heart presenting with isolated conduction disorder is unusual and rare

Source: https://www.sciencedirect.com/science/article/pii/S2468600X1930101X
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