Ebstein's Anomaly And Sudden Death In Adolescence: A Case Report In Barranquilla, Colombia

Abstract

Introduction: Congenital heart defects, including Ebstein anomaly (EA), are a major cause of morbidity and mortality worldwide. AE manifestations depend on anatomy and may include arrhythmias and right atrial enlargement. AE is diagnosed through echocardiography, and treatment ranges from symptomatic and antiarrhythmic management to surgical repair. Case Presentation: A previously healthy 16-year-old adolescent who experienced sudden cardiac arrest recovered after 20 minutes of CPR. He was admitted to the pediatric intensive care unit and placed on a ventilator and vasopressors. His electrocardiogram showed a preexcitation pattern, and an electrophysiological study identified two accessory pathways. He was diagnosed with Ebstein's anomaly, and propafenone was administered to modulate the slow nodal pathway. In a second procedure, the right posterior accessory pathway was ablated, which allowed the antiarrhythmic medication to be discontinued while the antiplatelet therapy continued. The patient had a satisfactory outcome. Discussion: Early diagnosis and treatment impact the survival of patients with EA, even when the patient's medical history is unknown. EA is a rare congenital heart disease that affects one in 20,000 live births. EA is associated with arrhythmias in 22-42% of cases, 5-10% of which are associated with Wolff-Parkinson-White syndrome. Up to 25% of patients have accessory pathways. Conclusion: Similar cases with a fatal outcome are rare, which makes this clinical case exceptional. Given the appropriate clinical response, it is important to understand that EC can go unnoticed throughout life.