Pediatric Giant Right Atrial Aneurysm: A Case Series and Review of the Literature

Abstract

Giant right atrial aneurysm is a rare form of congenital heart disease with a wide spectrum of clinical presentation varying from asymptomatic patients to those with refractory atrial arrhythmias or severe airway obstruction. Diagnosis is often confused with other causes of right atrial dilation such as Ebstein disease. Because of its rare occurrence and variable clinical presentation, inconsistencies in medical and surgical management strategies exist between centers. We present five cases of giant right atrial aneurysm managed at our institution and discuss the clinical presentation, diagnostic challenges, and medical and surgical management.

Prenatal diagnosis of right atrial diverticulum and outcomes: a case report

“…Right atrial diverticulum also known as by the other names such as the idiopathic dilatation of right atrium(IDRA) (1,2), right atrial aneurysm(RAA)(3), or even aneurysm of the right atrial appendage (4,5), is a rare a condition which most often diagnosed by chance in adults (2,4), but the prenatal and antenatal diagnosis of this condition has been reported even more rarely. (1,3)while IDRA or RAA de ned as the isolated enlargement of the right atrium without the presence of any other cardiac lesions which can cause right atrium dilatation, a diverticulum is a bromuscular strand entry of the right atrium and it is important from this aspect that can be misdiagnosed with right atrial aneurysm which involves all layers(epicardium, myocardium, and endocardium) of the atrial wall, while it is better this two conditions to be differentiated from each other (1,6)in the literature, this differential diagnostic border has not been clearly established and that's why we see these two different terms are used frequently instead of each other as we can see in Morrow et al report (7). Other misdiagnoses such as Ebstein's anomaly should be ruled out using fetal heart echocardiography (3,8).…”

“…(1,3)while IDRA or RAA de ned as the isolated enlargement of the right atrium without the presence of any other cardiac lesions which can cause right atrium dilatation, a diverticulum is a bromuscular strand entry of the right atrium and it is important from this aspect that can be misdiagnosed with right atrial aneurysm which involves all layers(epicardium, myocardium, and endocardium) of the atrial wall, while it is better this two conditions to be differentiated from each other (1,6)in the literature, this differential diagnostic border has not been clearly established and that's why we see these two different terms are used frequently instead of each other as we can see in Morrow et al report (7). Other misdiagnoses such as Ebstein's anomaly should be ruled out using fetal heart echocardiography (3,8). Many cases presenting with right atrial enlargement are asymptomatic(4), however, long-term outcomes and prognosis should not be overlooked, hence it can lead to cardiac arrhythmia, tachycardia, atrial brillation, thromboembolism, and respiratory distress due to high pressure of the massive right atrium on the left bronchi and causing airway compression (3).…”

“…Other misdiagnoses such as Ebstein's anomaly should be ruled out using fetal heart echocardiography (3,8). Many cases presenting with right atrial enlargement are asymptomatic(4), however, long-term outcomes and prognosis should not be overlooked, hence it can lead to cardiac arrhythmia, tachycardia, atrial brillation, thromboembolism, and respiratory distress due to high pressure of the massive right atrium on the left bronchi and causing airway compression (3).…”

“…Management of the right atrial enlargement is still controversial and varies widely from routine clinical follow up to anti-arrhythmic prophylaxis and surgical resection depended on the clinical presentation (3,4).…”

Right Atrial Aneurysm with Supraventricular Tachycardia and Frequent Atrial Premature: A Case Report

“…At present, there is no uni ed recommended treatment standard in the world. In general, conservative treatment is recommended for asymptomatic patients 8 . Patients with congenital heart defects, or large and symptomatic tumors were recommended surgery.…”