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 (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 (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 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.