Clinical Presentation, Diagnosis, and Management of Idiopathic Enlargement of the Right Atrium: An Analysis Based on Systematic Review of 153 Reported Cases

Abstract

Idiopathic enlargement of the right atrium (IERA) is a rare cardiac anomaly, and only sporadic cases have been reported. Little is known about its clinical relevance, and inconsistencies in medical and surgical management remain among different settings. In this paper, we systematically reviewed the published cases of the IERA in terms of clinical presentation, diagnosis, and management. A total of 153 cases of IERA were covered. Arrhythmia, dyspnea, and palpitation were found to be the most common clinical manifestations. It tends to be associated with life-threatening complications and sudden cardiac death. Diagnosis was mostly established by using echocardiography. Presenting symptoms, abnormal ECG findings, and therapeutic modalities were significantly related to the prognosis of IERA. Symptomatic patients were significantly more likely to have poor outcomes than asymptomatic patients (<i>p</i> = 0.044), and conservative treatment was more associated with adverse outcomes compared to surgical resection (<i>p</i> = 0.016). In conclusion, IERA, although rare, tends to be associated with potential life-threatening complications and sudden cardiac death. Echocardiography is the most common diagnostic modality. Surgical resection is indicated for symptomatic patients.