Cor triatriatum sinister--three case reports

Cor triatriatum is a rare congenital heart disease (0.1% of all congenital cardiac defects), but a higher incidence, up to 0.4% has been reported in autopsies of pts with CHD (1, 2, 7). There are two types: left and right. Cor triatriatum sinister is more common that dexter. Cor triatriatum dextrum is extremely rare. Fewer than 300 cases of cor triatriatum have been reported. It can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical). It's a surgically correctable CHD and can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical).

A retrospective review of three patients with Cor triatriatum, diagnosed at University Children's Hospital, during a eight year period (2000-2007). Among 1671 patients with CHD, the diagnosis of cor triatriatum has been established in three patients (0.18%). There were two boys and one girl, aged two years, 6 months and nine years, respectively. All of them had cor triatriatum sinister, with a communication between the right atrium and either the proximal or distal chamber. The first patient had a classic form of cor triatriatum, with a small hole in the diaphragm between atria, which imitated mitral stenosis, while the third patient had also mitral valve prolapse, but the hole between atria was unrestrictive. The second patient manifested atypical form, with many, additional defects: except large, unrestrictive ASD with a very small hole in the diaphragm between two atria, he had also total anomalous pulmonary venous return, draining in coronary sinus, large perimembranous VSD, hypoplastic aorta with coarctation, and high pulmonary vascular resistance. In the second patient, the diagnosis has been performed at 6 months of age, but due to lack of cardio-surgery and poor possibilities for going abroad for operation--finally he had been operated seven months later, but he died a week after surgery. The first patient has been successfully operated, immediately after the diagnosis was performed, while the last patient was diagnosed incidentally at the age of nine. She was symptoms free up to now, but recently she was developing symptoms and was successfully operated.

Cor triatriatum is more prevalent than is thought before echocardiography era. Echocardiography was method of choice in the diagnosis of typical forms, while in a atypical form cardiac catheterization was also performed. Two patients with classic form of CT were successfully operated, while the patient with atypical form and many additional cardiac anomalies died after cardio surgery. The main predictors for prognosis are: the size of the hole in the diaphragm between two chambers of atrium, additional cardiac malformations, and time of surgery.