Cor triatriatum sinistrum in children: A congenital cardiac anomaly which is difficult to diagnose

Background. Cor triatriatum (CT) is a rare congenital cardiac anomaly which is difficult to diagnose. Aim. To present a large cohort of paediatric patients with CT. Methods. The study group consisted of 15 patients (mean age 3.4 years, range 0.25-12 years) with CT in whom the results of clinical findings, ECG, chest radiography and echocardiography were analysed. Five patients underwent cardiac catheterisation. Results. Clinical symptoms and ECG as well as radiographic findings were non-specific. The final diagnosis was established based on echocardiographic examination. In all children CT was diagnosed; in addition, three patients had coexisting congenital anomaly such as ventricular septal defect, pulmonary stenosis or atrio-ventricular septal defect. The results of cardiac catheterisation did not change the diagnosis established by echocardiography. Fourteen patients underwent surgical correction. In three patients the intra-operative findings differed slightly from those obtained during echocardiography: in one patient the presence of total anomalous pulmonary venous connection with the right atrium was not confirmed, whereas in the other two patients an additional left superior vena cava connected with the left atrium was found. Two children died in the early post-operative period: one due to low cardiac output syndrome and the second due to central nervous system disorder. Conclusions. CT is a congenital cardiac anomaly which may require early surgical intervention. Echocardiography with the use of Doppler technique is the best diagnostic method. During echocardiographic examination, special attention should be paid to venous drainage to the atria.

Duke Scholars

Author Larry Bruce Goldstein Neurology, Stroke and Vascular Neurology