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A 64-years-old man had cor triatriatum (Lucas-Schmidt type I A) with severe mitral regurgitation and atrial fibrillation. We perfomed resection of the anomalous septum between the accessory chamber and left atrium, and conducted mitral annuloplasty and maze procedure. Arrhythmia were not encountered after surgery. The maze procedure and resection of the anomalous septum with mitral surgery proved to be effective for atrial fibrillation with cor triatriatum.

Cor triatriatum is a rare congenital cardiac anomaly especially in adulthood. A 68-year-old female was diagnosed as a cor triatriatum classified to Lucas-Schmidt IA, severe degree of mitral regurgitation and atrial fibrillation. Resection of the abnormal diaphragm in the left atrium and miral valve replacement were performed. Although the reason of sudden death of this patient after discharge is unknown, surgical intervention for atrial fibrillation should have performed to prevent a thromboembolism in such cases.

A 37-day-old girl having cor triatriatum with partial anomalous pulmonary venous return was found on emergent admission. Echocardiography and angiography revealed severe pulmonary hypertension with anomalous pulmonary venous return to inominate vein. She still was in respiratory failure after emergent surgical repair because of severe pulmonary hypertension following pulmonary venous obstruction. Prostacyclin is the first Food and Drug Administration (FDA)-approved treatment for advanced primary pulmonary hypertension, which has antithrombotic properties related to its effect on platelets and is a potent vasodilator of both the systemic and pulmonary arteries, and has positive inotropic properties. We started prostacyclin oral internal therapy for her and she recovered from respiratory problems very quickly. Prostacyclin might be effective for pulmonary hypertension before and after surgical repair as primary pulmonary hypertension.

A surgical treatment of a 54 year-old female with cor triatriatum was reported. Two dimensional echocardiography had demonstrated an abnormal septum in the left atrium. The abnormal septum had a 2 x 1 cm fenestration and existed between the left and right pulmonary veins. Preoperatively, we had misjudged the septum as ASD and misdiagnosed this case as PAPVC. During surgery we found that there was the accessory atrial chamber posterior to the true ASD. The accessory chamber received right pulmonary veins and connected to the left atrium. The left pulmonary veins connected to the left atrium normally behind the abnormal septum. We diagnosed this case as Lucas-Shmidt IIIA1 type cor triatriatum with ASD. The abnormal septum was resected, and the ASD was closed with a bovine pericardium. The postoperative course was uneventful and she was discharged 19 days after the operation.

A case was 6-year-old female diagnosed as the cor triatriatum. The magnetic resonance imaging synchronized at R wave on the electrocardiogram was performed in order to see the abnormal septum in the left atrium. In the horizontal view, the abnormal septum that divides the left atrium into the accessory left atrial chamber and the true left atrium, was well visualized. This septum lied on the vertical portion. Operation was carried out with the approach through the accessory chamber and the fibromuscular membrane were excised. Preoperative imaging of the MRI was useful and helpful for us to reach to a decision of the surgical approach in this patient.

A case report of a variant type of cor triatriatum was presented. The patient was critically ill when she was admitted to our hospital. An emergent operation was performed under the cardio-pulmonary bypass. The right atrium was incised, then the interatrial septum was incised. Inspection revealed an abnormal membrane in the left atrium. All the pulmonary veins were drained into the accessory chamber. The left atrial appendage was also connected with the accessory chamber. The membrane was not including the mitral valve tissue. It was resected completely. Postoperative course was uneventful.

Cor triatriatum is uncommon in all congenital heart diseases. It is a malformation resulting in a separation of the left atrium or right atrium into two chambers due to a congenitally abnormal diaphragm. We wish to present a case of cor triatriatum in which MRI was found most useful for preoperative diagnosis and surgical procedure. A 2-year-old girl was transferred to us for severe pulmonary congestion as shown on chest X-ray. Echocardiography showed abnormal diaphragm in the left atrium. MRI demonstrated clearly the relationship between left pulmonary vein and the abnormal diaphragm. Therefore we should preoperatively determine type I A according to the Lucas and Schmidt's classification. Cardiac catheterization showed moderate pulmonary hypertension and confirmed cor triatriatum. The resection of the abnormal diaphragm was performed under extracorporeal circulation with moderate hypothermia. The postoperative course was uneventful. MRI is a very useful non-invasive technique in making a diagnosis and in choosing the appropriate surgical procedure for cor triatriatum.

Cor triatriatum associated with situs inversus is rare. A 6-month-old male who had cor triatriatum with atrial septal defect (ASD) and situs inversus totalis was successfully operated. Urgent surgical intervention was performed because of congestive cardiac failure despite drug therapy. The abnormal septum in the left atrium was resected and ASD was closed. His postoperative course has been uneventful.

A successful repair of cor triatriatum with VSD, PDA and CoA is reported. A two-month-old boy was admitted for evaluation of growth failure and cyanosis. Physical examination on admission revealed systolic murmurs at left sternal border. Chest X-ray showed increased pulmonary vascularities without cardiomegaly. Echocardiography showed abnormal wall in left atrium with VSD, PDA and CoA. An emergency operation consisting of patch closure of VSD, ligation of PDA and resection of the anomalous left atrial membrane were performed. At postoperative course, an episode of pulmonary hypertension crisis happened. But the patient tolerated this episode and survived.

A case of cor triatriatum associated with coarctation of the aorta (CoA) and VSD is reported. A 4-month-old boy underwent subclavian flap aortoplasty and ligation of PDA with the diagnosis of CoA and VSD. At the age of 1 year and 2 months, echocardiography revealed an abnormal subdividing membrane in the left atrium. Under the definite diagnosis of cor triatriatum (Lucas & Schmidt type I B1) and VSD, complete removal of the abnormal septum, patch closure of ASD and VSD was performed with the aid of cardiopulmonary bypass, blood cardioplegia and topical cardiac cooling. Postoperative course was uneventful and the patient was discharged from the hospital on the 38th postoperative day. An association of cor triatriatum, VSD, and coarctation of the aorta is very rare, and to our knowledge, the case presented here seems to be the first report of successful total correction by a staged approach in the literature.

Four cases of cor triatriatum are reported. Cases 1 and 2 had IIA, case 3 had IA, and case 4 had IIIB 1 type of cor triatriatum according to Lucas-Schmidt classification. Cases 1 and 2 were diagnosed by echocardiography preoperatively. They were not performed cineangiocardiography because of pulmonary congestion. Case 3 had type IA so called classical type, which is hemodynamically identical to mitral stenosis and supravalvular stenosing ring. The clinical features are depend on the size of the communication between accessory chamber and true left atrium. As case 4, he had been diagnosed of simple atrial septal defect preoperatively, careful echocardiographic examination was essential for accurate diagnosis. In case 2 with hypoplastic left heart, secondary surgical repair is considered to be desirable followed by balloon dilatation of two atrial septal defects communicated to accessory chamber and to true left atrium. Thus, we expect development of hypoplastic left ventricle.

Three operative cases with Cor triatriatum, age ranged from 30 days to 16 months, were presented. All types were IB1 of Lucas-Schmidt's classification and one patient was associated with right upper PAPVC. One patient died of low output syndrome due to preoperative shock. Communication of abnormal diaphragm in left atrium varied from small and multiple to 5 mm in diameter. Their preoperative diagnoses were established by two-dimensional echocardiogram prior to angiocardiogram and the intracardiac communications were well evaluated by color doppler echocardiogram which was superior to angiocardiogram in this evaluation. Postoperatively, no abnormal diaphragm were detected in two survivors. The diagnosis and operative procedure for this anomaly were discussed on.