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.