Giant Right Atrial Aneurysm: Observation Versus Intervention

Idiopathic right atrial aneurysm (IRAA) is rarely reported in pediatric literature. Clinical presentations range from incidental findings to serious complications including arrhythmias, thrombosis, and cardiac arrest.

A 4-year-old girl with DCHS1 mutation, autism spectrum disorder, and congenital IRAA managed conservatively presented to the emergency department with 3-day history of viral symptoms. Shortly after arrival, the patient developed unstable atrial flutter requiring electric cardioversion. Later, imaging demonstrated a 1.2 × 1.2-cm right atrial thrombus in a massively dilated right atrium. The patient underwent successful surgical atrial reduction and thrombectomy.

IRAA has been rarely reported in pediatric literature. Half of the reported cases were diagnosed incidentally. Our case is unique in its rapid progression to serious complications despite recently initiated aspirin therapy.

This case proposes a possible association between DCHS1 variants and IRAA and highlights the importance of considering prophylactic surgical intervention in select pediatric patients to prevent serious complications.