Tachyarrhythmia in Patients after the Staged Fontan Palliation: Prevalence, Therapy, and Risk Factors

Background: Although outcomes after total cavopulmonary connection (TCPC) have improved continuously, there remain Fontan-specific late complications. Tachyarrhythmia is one of these complications that require medical and interventional therapy. The purpose of this study is to evaluate the outcomes after TCPC regarding the appearance of tachyarrhythmia.

Method: We performed a retrospective analysis of 620 consecutive patients with univentricular heart who underwent TCPC between January 1994 and December 2021 at our institution. Patients who underwent TCPC conversion from classic Fontan procedure were excluded from this study. Most of the patients had non-fenestrated extracardiac TCPC. Development of tachyarrhythmia requiring medical treatment after TCPC was retrospectively depicted using internal and external medical records. Freedom from development of tachyarrhythmia was estimated using Kaplan–Meier analysis, and pre- and perioperative variables associated with the onset of tachyarrhythmia were identified.

Results: A total of 52 patients presented with tachyarrhythmia that required medical therapy. Onset during hospital stay was observed in 27 patients and onset after hospital discharge was observed in 32 patients. Freedom from tachyarrhythmia following TCPC at 5, 10, 15, and 20 years was 97, 95, 91, and 80%, respectively. Factors associated with tachyarrhythmia during hospital stay after TCPC were double-outlet right ventricle (hazard ratio [HR: 3.1; confidence interval [CI], 1.3–7.3, p = 0.010), heterotaxy (HR, 3.0; CI, 1.1–8.3, p = 0.035) and dominant right ventricle (HR, 3.20; CI, 1.3–8.0, p = 0.014). Factors associated with tachyarrhythmia after hospital discharge were double outlet right ventricle (hazard ratio [HR], 3.0; confidence interval [CI], 1.4–6.4, p = 0.006), dominant right ventricle (HR, 3.20; CI, 1.3–8.0, p = 0.014), age at TCPC (HR, 1.0; CI, 1.0–1.1, p = 0.024), and weight at TCPC (HR, 1.0; CI, 1.0–1.0, p = 0.009).

Conclusion: In our large cohort of 620 patients following TCPC, freedom from tachyarrhythmia at 10, 15, and 20 years was 95, 91, and 80%, respectively. Patients with double outlet right ventricle, heterotaxy syndrome, and dominant right ventricle were at increased risk for tachyarrhythmia after TCPC.

Publikationsverlauf

Artikel online veröffentlicht:
28. Januar 2023