Tan Ernestine Faye S, Gharti Sakar B, Tan Danielle Grace S
Internal Medicine, Interfaith Medical Center, New York, USA.
General Practice, University of Santo Tomas Faculty of Medicine and Surgery, Manila, PHL.
Cureus. 2025 Aug 17;17(8):e90281. doi: 10.7759/cureus.90281. eCollection 2025 Aug.
Atrial septal defects (ASDs) are among the most common congenital heart anomalies and often remain undetected until adulthood. While small ASDs may close spontaneously, larger unrepaired defects can lead to serious complications such as right heart failure, pulmonary arterial hypertension, Eisenmenger syndrome (ES), and thromboembolic events. We present a case of a 66-year-old Filipino female with a longstanding unrepaired secundum-type ASD, who was admitted with progressive dyspnea and hypoxemia. The patient developed atrial flutter, and further workup revealed severe pulmonary hypertension, right ventricular failure, and pulmonary embolism. Imaging confirmed chronic thromboembolic pulmonary hypertension (CTEPH), a rare but life-threatening complication in patients with ES. Despite aggressive medical management, including anticoagulation, antibiotics for pneumonia, and antiarrhythmic therapy, the patient's condition deteriorated, and she eventually succumbed to her illness. This case highlights the critical importance of early detection and surgical repair of large ASDs to prevent irreversible pulmonary vascular disease and associated complications such as ES and CTEPH, particularly in resource-limited settings where definitive interventions are often inaccessible.
房间隔缺损（ASD）是最常见的先天性心脏异常之一，通常在成年期之前未被发现。虽然小型ASD可能会自行闭合，但较大的未修复缺损可导致严重并发症，如右心衰竭、肺动脉高压、艾森曼格综合征（ES）和血栓栓塞事件。我们报告一例66岁菲律宾女性，患有长期未修复的继发孔型ASD，因进行性呼吸困难和低氧血症入院。患者出现心房扑动，进一步检查发现严重肺动脉高压、右心室衰竭和肺栓塞。影像学检查证实为慢性血栓栓塞性肺动脉高压（CTEPH），这是ES患者中一种罕见但危及生命的并发症。尽管采取了积极的药物治疗，包括抗凝、治疗肺炎的抗生素和抗心律失常治疗，但患者病情恶化，最终因病死亡。该病例凸显了早期发现和手术修复大型ASD以预防不可逆肺血管疾病以及ES和CTEPH等相关并发症的至关重要性，特别是在资源有限的环境中，确定性干预措施往往难以获得。
