CHEST Guidelines-An-8-Year-Old-Boy-With-a-Giant-Left-Atrium

An-8-Year-Old-Boy-With-a-Giant-Left-Atrium_chest

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An 8-year-old boy with a history of a heart murmur and biatrial enlargement was admitted to a medical facility due to nausea and vomiting, alongside a year-long history of shortness of breath and exercise intolerance. Initially evaluated years ago, a follow-up was not pursued until this admission. Diagnostic imaging showed extreme left atrium dilation and mitral and tricuspid regurgitation. The condition was diagnosed as Congenital Left Atrial Aneurysm (CLAA), a rare cardiac anomaly characterized by significant enlargement of the left atrium due to congenital wall weakness without prior known valvular disease.<br /><br />Upon examination, the boy presented with a holosystolic murmur, jugular venous distension, and hepatojugular reflux. His diagnostic profile included elevated B-type natriuretic peptide, significant cardiomegaly, and ECG changes indicative of atrioventricular and intraventricular blocks. CT and echocardiography revealed a significantly dilated left atrium exerting pressure on the lung structures.<br /><br />The boy underwent surgical intervention, where a left atrial aneurysm resection and mitral valve reconstruction with a "C-shaped" artificial ring were performed, alongside tricuspid annuloplasty. Pathological assessment supported the CLAA diagnosis, noting focal myocardial thinning and no inflammation, ruling out congenital mitral valve dysfunction. Post-operative recovery included substantial reduction in atrial size and valve regurgitation, with plans for ongoing follow-up.<br /><br />CLAA poses diagnostic challenges, requiring exclusion of other conditions such as cardiomyopathy, rheumatic heart disease, and LAAA. Echocardiography, cardiac CT, and histopathological evaluation are pivotal in confirming CLAA. Surgery becomes crucial in cases with progressive atrial enlargement and significant valve regurgitation, with asymptomatic cases requiring regular monitoring.

Keywords

Congenital Left Atrial Aneurysm

biatrial enlargement

mitral regurgitation

tricuspid regurgitation

cardiac anomaly

surgical intervention

echocardiography

atrial enlargement

valve reconstruction

cardiomegaly