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Eisenmenger Syndrome After Delayed Atrial Septal Defect Closure at High Altitude: A Case Report

Angelica Guaman-Gavilema, Domenica Villacres-Alarcon, Andrés Arteaga-Arellano, Roberto Arregui-Costales, Jordan Llerena-Velastegui, R. Jimbo-Sotomayor, Xavier Sánchez

The American journal of case reports, 2026

Abstract

Patient: Female, 46-year-old Final Diagnosis: Eisenmenger syndrome following delayed surgical repair of atrial septal defect Symptoms: Chest pain • cyanosis • dyspnea • fatigue • orthopnea Clinical Procedure: — Specialty: Cardiology Objective: Congenital defects / diseases Background Eisenmenger syndrome develops from untreated or late-repaired cardiac shunts, causing irreversible pulmonary-vascular remodeling and systemic symptoms. Although timely repair has nearly eradicated Eisenmenger syndrome in high-income regions, delayed diagnosis in middle-income countries, especially when combined with chronic high-altitude hypobaric hypoxia, such as at approximately 2800 m (9186 ft) in the Ecuadorian Andes, can accelerate pulmonary hypertension through hypoxic vasoconstriction and erythrocytosis. We report a case of Eisenmenger syndrome 2 decades after surgical atrial septal defect closure. Case Report A 46-year-old woman living at high altitude presented with severe cyanosis and heart failure 22 years after atrial septal defect closure. She had marked hypoxemia and secondary erythrocytosis. Echocardiography showed suprasystemic pulmonary artery pressure, severe right-chamber dilation, and reduced right ventricular systolic function. Right-heart catheterization confirmed Eisenmenger physiology with severe pulmonary-vascular resistance and low cardiac index. Chest computed tomography showed marked pulmonary artery enlargement without thromboembolic disease. Multidisciplinary…

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