Key result
Transvenous implantation of a passive fixation pacing lead at the right ventricular apex successfully resolved recurrent syncopal episodes in a patient with a transitional atrioventricular septal defect.
Why the study?
Most existing literature focuses on pacemaker implantation in patients with isolated atrial septal defects, with limited documentation on challenges in transitional atrioventricular septal defect.
Case Report (n=1)
No
Transvenous pacemaker implantation in adults with uncorrected transitional AVSD and severe pulmonary hypertension is challenging but can be successfully achieved using a passive fixation lead and multidisciplinary planning.
May offer pacing option for syncope in transitional AVSD; hypothesis-generating and should not change practice without further evidence.
A 56-year-old woman, known in her past medical history with uncorrected atrioventricular septal defect, secondary pulmonary hypertension, and permanent atrial fibrillation, with repeated hospitalizations for congestive heart failure over the years, presented to the Emergency Department for repeated syncopal episodes. Holter monitoring detected atrial fibrillation with periods of asystole. While most cases in the literature mainly focus on patients with isolated atrial septal defects (ASD)(1), we present a case of a challenging pacemaker implant in a patient with transitional atrioventricular septal defect (AVSD). The case illustrates the challenges of differential diagnosis of syncope and of implanting a pacemaker in adults with congenital heart disease, underscoring the importance of an excellent understanding of electrophysiology, defect anatomy, and postoperative alterations. In addition, this case emphasizes that structural heart disease can be a potential cause when assessing recurrent syncope. When selecting the implantation and pacing modes, operators must consider the possibility of multiple reimplantation throughout the patient’s lifespan.
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Stoiculescu et al. (2025) conducted a case report in Transitional atrioventricular septal defect with recurrent syncope (n=1). Permanent pacemaker implantation was evaluated on Recurrence of syncopal episodes. Transvenous implantation of a passive fixation pacing lead at the right ventricular apex successfully resolved recurrent syncopal episodes in a patient with a transitional atrioventricular septal defect.
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