Concurrent cor triatriatum sinister and levoatriocardinal vein in an 11-year-old boy presenting with foudroyant pulmonary edema after appendectomy: A living tribute to the mal-incorporation theory

Özet

Cor triatriatum sinister (CTS) is a rare cardiovascular malformation, in which the left atrium (LA) is separated by a membraneinto the proximal and distal LA chambers (1). Levoatriocardinal vein(LACV) is an even rarer vascular anomaly that connects the LA (ortributaries of pulmonary vein) with the left innominate vein (an embryologic derivative from the cardinal system) (2). Development ofthe pulmonary vein and systemic venous sinus are spatiotemporally correlated during embryogenesis (3). Here, we report a case ofan 11-year-old boy unexpectedly presenting with acute pulmonaryedema after appendectomy. Echocardiography and chest computed tomography (CT) revealed a combination of CTS and LACV,which were then surgically corrected. We briefly reviewed theEnglish literature reporting the pediatric patients with concurrentCTS and LACV, compared their clinical profiles with ours, highlighted the pathomechanism of a delay onset of clinical manifestationsin our patient, and provided an anatomical feature indicating themal-incorporation theory for the embryogenesis of CTS.