Key result
Systemic right ventricular septal pacing improves cardiac index and stabilizes heart failure over 2 years.
Case Report (n=1)
No
This case report describes the oldest known surviving patient (60 years old) with unrepaired d-TGA and demonstrates that transvenous systemic RV septal pacing with AV delay optimization and apixaban anticoagulation can safely stabilize severe heart failure and bradytachyarrhythmia over 2 years despite the presence of an intracardiac shunt.
Answers are grounded in this paper and cite their sources.
May support individualized pacing in select unrepaired d-TGA; leaves open prospective trials before broader adoption.
Background: Dextro-transposition of the great arteries (d-TGA) typically results in death within the first year of life without surgery. Shunts can allow sufficient blood mixing and survival into adulthood; however, survival of patients with unrepaired d-TGA beyond 60 years has not been reported. Case summary: A 60-year-old woman with unrepaired d-TGA, an unrestricted atrial septal defect, and partial anomalous pulmonary venous return presented with recurrent syncope due to atrial tachycardia (AT) following two sessions of radiofrequency catheter ablation (RFCA) and sinus node dysfunction. She had a history of recurrent heart failure (HF) because of severe systemic right ventricle (sRV) dysfunction and tricuspid regurgitation. Multiple inducible ATs persisted even after repeated RFCA, making pacemaker implantation (PMI) and anti-arrhythmic therapy the preferred options. Although transvenous lead implantation was relatively contraindicated due to the presence of an intracardiac shunt, transvenous DDD PMI was deemed the most viable option considering the patient's fragile haemodynamic state. Pacing studies indicated that sRV pacing was superior to left ventricular septal pacing, as it reduced the QRS duration and improved RV synchrony. Following successful sRV septal lead implantation and atrioventricular delay (AVD) optimization, HF symptoms improved, ATs were suppressed with amiodarone, and syncope resolved. The patient remained stable for 2 years with no thromboembolic events under apixaban therapy and no HF or arrhythmia recurrence. Discussion: Although continuous close monitoring is warranted, endocardial pacing, AVD optimization, and medication enabled effective stabilization over 2 years in this rare case of unrepaired d-TGA with bradytachyarrhythmia and sRV dysfunction.
No takes yet. Share an insight, caveat, or question.
Machino‐Ohtsuka et al. (2025) conducted a case report in Unrepaired dextro-transposition of the great arteries (d-TGA) with atrial septal defect, partial anomalous pulmonary venous return, heart failure, and atrial tachycardia (n=1). Transvenous DDD pacemaker implantation with systemic right ventricular septal lead placement, atrioventricular delay optimization, and medical therapy (amiodarone, apixaban, empagliflozin, spironolactone, bisoprolol, furosemide, tolvaptan, pimobendan) was evaluated on Resolution of syncope, suppression of atrial tachycardia, heart failure stabilization, and cardiac index improvement over 2 years. Systemic right ventricular septal pacing, AV delay optimization, and medical therapy increased cardiac index from 1.47 to 1.87 L/min/m² and stabilized heart failure and arrhythmias for 2 years.
Synapse has enriched 10 related papers. Consider them for comparative context: