A rare case of giant congenital left atrial appendage aneurysm in a 4-month-old child

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A 4-month-old boy was referred to our hospital for cardiac murmur. Echocardiography showed a large left para-cardiac chamber communicating with a normal-sized left atrial cavity via a wide neck, with evidence of spontaneous echo contrast (Fig. 1a). Thus, a giant left atrial appendage aneurysm (LAAA) was diagnosed. The pericardium was intact. To better define the anatomical relationship of the aneurysm a computed tomography (CT) three-dimensional reconstruction was performed (Fig. 1b and c). The patient underwent surgical correction with LAAA resection (Fig. 2a, b and c).
Congenital LAAA, is a very rare entity, with only a few cases reported in the world literature during infancy.1 The condition generally became clinically manifest from the second to fourth decades of life.
Echocardiography has a pivotal role in LAAA diagnosis, demonstrating a direct connection between the aneurysm and a normal left atrial chamber, and the absence of pericardial defect.1
Differential diagnosis includes congenital deficit of pericardium, solid or cystic paracardiac tumors, and appendage juxtaposition.
CT or cardiac magnetic resonance may have an important role in better definition of the anatomical relationship of the LAAA with the surrounding structures.
In our case, in agreement with previously reported ones,1 the neck of the LAAA is wide. This can be explained by the laxity of the inner and outer muscle bands constricting the base of the appendage.2 Indeed, LAAA is attributed to congenital dysplasia of the pectinate muscles in the left atrial appendage and of the related muscle bands in the left atrium.2
The recommended treatment for congenital LAAA is surgical resection, effectively preventing both arrhythmias and systemic embolisms.

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