Therapeutic Challenges in Total Situs Inversus Associated with Sick Sinus Syndrome, Ventricular Arrhythmias, and Heart Failure with Preserved Ejection Fraction: Narrative Review and Case Report.

BACKGROUND

Total situs inversus (TSI) is a rare genetic anomaly, and approximately half of the affected individuals also have other associated cardiovascular anomalies. Thus, the concomitance of conduction and rhythm disturbances is seldom described in the medical literature.

METHODS

We searched the medical literature for similar cases published as full text, in English, on Clarivate, PubMed, and Google Scholar between 2016 and 2025.

RESULTS

We found 9 reports on TSI patients also having sick sinus syndrome (SSS) associated with rhythm disturbances, mainly atrial fibrillation, raising diagnostic and procedural challenges due to the anatomical anomalies requiring a peculiar approach. We describe the case of a 43-year-old woman diagnosed with TSI associated with ventricular arrhythmias in 2015 who experienced SSS requiring the implantation of a pacemaker during 10 years of follow-up but continued to have frequent episodes of nonsustained ventricular tachycardia (NSVT), raising multiple diagnoses and therapeutic challenges. After developing heart failure with preserved ejection fraction, she received guideline-adjusted treatment and, surprisingly, her clinical status improved, and NSVT diminished in frequency and then disappeared.

CONCLUSIONS

Highlighting TSI's clinical implications, often associated with other cardiovascular abnormalities, is important for an accurate diagnosis and adapted therapeutic management, considering the procedural challenges and potential complications.