Background: Cor triatriatum is a rare cardiac malformation with clinical presentations that may vary from asymptomatic to severe respiratory and heart failure. The natural course of cor triatriatum is not well known. Purpose: To investigate the clinical presentation, diagnosis, and outcomes in children with cor triatriatum. Patients and Methods: From 1990 to 2000, 11 cases of cor triatriatum were diagnosed in our institution. Results: There was a male predominance, with eight male and three female patients. The age of diagnosis ranged from 1 day to 8 years (median 2 years). Eight of the patients had cor triatriatum sinister, and the remaining 3 had cor triatriatum dexter. Four (50%) of the patients with cor triatriatum sinister presented with early respiratory and heart failure during infancy which mimicked acute bronchiolitis, and all of them had obvious pulmonary venous obstruction. The other 4 cases without pulmonary venous obstruction were diagnosed incidentally due to innocent murmur in 3, and associated ventricular septal defect (VSD) in 1. Only 2 of the 8 cases had other cardiac abnormalities (1 partial anomalous pulmonary venous return, 1 VSD) in addition to atrial shunt. Six of the 8 patients received operation and all of them had good outcomes. All 3 of the patients with cor triatriatum dexter died of associated cardiac anomalies (trisomy 18 with tetralogy of Fallot in 1, Goldenhar syndrome with pulmonary atresia with VSD in 1, and Ebstein's anomaly in 1). Conclusions: Most children with cor triatriatum have no obvious heart murmur and present with non-specific symptoms. The true incidence of this disease might not be as rare as indicated from previous reports because of under-diagnosis. Cor triatriatum should be included in the differential diagnosis of young infants with unexplained respiratory or heart failure.
|頁（從 - 到）||137-142|
|期刊||Acta Cardiologica Sinica|
|出版狀態||Published - 2003 九月|
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