Abstract

Introduction: Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease that has an incidence of sudden cardiac death of 0.2% per year, being arrhythmias the main cause of its occurrence. Objective: To compare characteristics of TOF patients referred for electrophysiological study (EPS) against those that were not (No-EPS). Method: Retrospective cohort with 215 patients (57.2% men; age = 29 ± 4) with corrected TOF (median of three years, ranging from 0.33 to 51) that underwent EPS between 2009-2020. The primary outcome was composed of death, implantable cardiac defibrillator (ICD) requirement and hospitalization. Results: Pre-syncope (EPS = 4.7%, No-EPS = 0%; p = 0.004), syncope (EPS = 7.1%, No-EPS = 1.7%; p = 0.056) and palpitations (EPS = 31%, No-EPS = 5.8%; p < 0.001) were symptoms that justified electrophysiological investigation. ICD was implanted in 24% of EPS and 0.6% of No-EPS (p=0.001). Twenty-six percent of the EPS group presented non-sustained ventricular tachycardia, while 0% in No-EPS (p = 0.012). The EPS group had more atrial fibrillation or atrial Flutter (35.7% vs. 6.9%; p < 0.001). The EPS patients had a wider QRS duration than the no-EPS group (171.12 ± 29.52 ms vs. 147 ± 29.77 ms; p < 0.001). Also, 26.2% of EPS performed ablation to correct macroreentrant atrial tachycardias. The incidence of primary outcome (death + ICD requirement + hospitalization) was higher in patients in the EPS group compared to the No-EPS group (p = 0.001). However, the total of seven deaths occurred during the clinical follow-up, but without differences between the groups (EPS = 4.7% vs. No-EPS = 2.8%; p = 0.480). Conclusion: EPS group had a profile of greater risk, more complex heart disease, and a greater occurrence of the primary outcome when compared to the No-EPS group.

Highlights

  • Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease that has an incidence of sudden cardiac death of 0.2% per year, being arrhythmias the main cause of its occurrence

  • Based on our multivariate and univariate analyses, we were able to identify that the presence of AF/Flutter was the characteristic that most significantly affected the primary outcome (Tables 4 and 5)

  • This is due to the risks that involve arrhythmias, such as sudden cardiac death

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Summary

Introduction

Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease that has an incidence of sudden cardiac death of 0.2% per year, being arrhythmias the main cause of its occurrence. The incidence of primary outcome (death + ICD requirement + hospitalization) was higher in patients in the EPS group compared to the No-EPS group (p = 0.001). Tetralogy of Fallot (TOF) is a cyanotic congenital heart disease with an incidence of one in 3,500 births, accounting for up to one-tenth of all congenital cardiac injuries[1,2] This pathology comprises four fundamental anatomical abnormalities: ventricular septal defect, pulmonary artery stenosis, hypertrophy of the right ventricle, and extraposition of the aorta over the septum[2]. Patients undergoing a surgical correction in the future may have complications such as pulmonary regurgitation, recurrence of pulmonary stenosis, and ventricular arrhythmias[2]

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