Anomalía de ebstein en paciente pediátrico. Reporte de caso

Abstract

Ebstein's Anomaly is a congenital heart disease with a prevalence of less than 1%, which is characterized by anatomical alterations of the tricuspid valve, right atrium, and right ventricle. This anomaly produces tricuspid insufficiency and, consequently, symptoms resulting from it, being the most frequent cyanosis, dyspnea, palpitations, and less frequently, arrhythmias. Transthoracic echocardiography is the most effective method to confirm the diagnosis and define the therapeutic approach, with the final treatment being tricuspid valve reconstruction surgery with the Da Silva cone technique. OBJECTIVE: Description of a case of Ebstein's Anomaly in a pediatric patient. METHODOLOGY: The case of an 11-year-old male patient with severe Ebstein's Anomaly is presented, who presents symptoms of tricuspid insufficiency, for which pharmacological treatment is instituted from the age of two, which is abandoned on several occasions, with progression of symptoms and need for corrective surgery, which cannot be performed due to respiratory infection. For several years, he has abandoned treatment and does not attend regular check-ups, which is why there is a severe progression of the pathology, with necessary complementary examinations and risk-benefit assessment for corrective surgery at present. CONCLUSIONS: Ebstein's Anomaly is a rare congenital heart disease; its clinical features and early diagnosis are of great interest to establish appropriate treatment and prevent its progression. Compliance with pharmacological treatment is essential since, if not obeyed, corrective surgery cannot be performed, compromising the patient's quality of life and prognosis