An adult case of polysplenia syndrome associated with sinus node dysfunction

Title : An adult case of polysplenia syndrome associated with sinus node dysfunction

Abstract:

Background: Polysplenia syndrome, a rare form of left isomerism, usually presents in childhood with severe cardiac anomalies. Adult presentation with a structurally normal heart is uncommon.

Case Presentation: A 26-year-old male presented with recurrent syncope over one year. Examination revealed bradycardia (40 bpm). ECG showed sinus node arrest with junctional escape, confirmed by Holter monitoring. Blood tests were normal.

Ultrasonography revealed a midline liver; CT scan showed multiple accessory spleens, bilateral trilobed lungs, and midline stomach, consistent with situs ambiguous with polysplenia syndrome. Echocardiography demonstrated a structurally normal heart without defects.The patient underwent DDDR pacemaker implantation, resulting in symptom resolution.

Discussion: Polysplenia syndrome can be associated with sinus node dysfunction, atrioventricular block, or dual AV nodal pathways. Recognition is crucial, as anatomical variations (e.g., interrupted IVC, left-sided SVC) may impact pacemaker implantation strategy.

Conclusion: In young adults with unexplained sinus node dysfunction, congenital anomalies like left isomerism should be considered. Early diagnosis informs both management and procedural planning.

Keywords: Polysplenia Syndrome, Left Isomerism, Sick Sinus Syndrome, Pacemaker
 

Biography:

Apoorva Tripathi is an FY2 doctor working in Urology at Churchill Hospital, Oxford University Hospitals since 2024. She graduated from B.J. Medical College, Ahmedabad. Passionate about internal medicine, she aims to pursue specialty training in this field. She has actively engaged in clinical case reports, audits, and teaching, gaining experience in patient care, research, and multidisciplinary collaboration. This case report reflects her interest in rare cardiac conditions and advancing clinical knowledge.