Abstract

Primary ciliary dyskinesia patients often have situs ambiguous, a congenital condition with abnormal arrangement of visceral organs in the abdomen and chest. It is associated with an increased risk of congenital heart defects and anomalous venous connections. As a result, placement of the leadless pacemaker through the femoral approach may not be viable in these patients. Herein we describe a successful placement of a leadless pacemaker through the left subclavian approach in a patient with primary ciliary dyskinesia with associated interrupted IVC, right internal jugular (RIJ) thrombus, failed epicardial pacers, and extraction of infected transvenous pacer. N/A A 36-year-old female with primary ciliary dyskinesia, situs ambiguous, RIJ thrombus, and azygous venous connection presents with paroxysmal atrial fibrillation, sinus node dysfunction and syncope (A); History of multiple pacemakers, including failed epicardial pacer, substance abuse and recurrent bacteremia with multiple transvenous pacer infections requiring extractions. Ultrasound access was gained in the left subclavian vein via the modified Seldinger technique. Access was serially dilated until a 23Fr dilator. A manual curve was applied to the tip of the Micra-introducer sheath and the long 27Fr hydrophilic micra-introducer sheath was advanced over a Lunderquist wire, using a stiff rail, into middle of the right atrium (B). The 105 cm Micra delivery catheter was inserted into the sheath, and advanced with fluoroscopic guidance into the right atrium. The introducer sheath was pulled back into the SVC and the delivery catheter was counter-clocked, flexed downward and advanced across the tricuspid valve. The pacemaker was directed to the midseptal position. Contrast was injected to verify positioning against the myocardium (C). The device was deployed with satisfactory parameters noting R waves of 12mV and threshold less than 1V at 0.24 msec pulse with. The pacer was then released with tugging testing confirming adequate deployment. (D). The patient was discharged safely home 2 days later. The left subclavian vein is a viable and safe approach for placing the Micra leadless pacemaker.

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