[Diagnosis and evaluation of a patient with cor triatriatum using perioperative transesophageal echocardiography].

Cor triatriatum is a rare congenital cardiac anomaly accounting for only 0.1-0.4% of all congenital heart diseases usually diagnosed in infancy or childhood and rarely found in adults. It is characterized by fibromuscular membrane dividing the left atrium into two chambers. This congenital heart disease is reported to be frequently associated with variety of cardiac anomalies such as an atrial septal defect, anomalous pulmonary venous drainage, and persistent left superior vena cava. A woman with no cardiac history was admitted to the hospital due to acute heart failure and diagnosed as severe mitral regurgitation and cor triatriatum by pre-orerative transthoracic echocardiography. Emergency mitral valve plasty was undertaken because of the severity of mitral regurgitation without determining the detailed type of cor triatriatum. Thus, diagnosis of the type of cor triatriatum with perioperative transesophageal echocardiography (TEE) was required to establish correct cardiopulmonary bypass and determine the operative procedure. Perioperative TEE revealed that the type was Lucas-Schmidt- I A, and cardiopulmonary bypass was established safely. Operation was performed without any problems. The TEE skill of our anesthesiologists could contribute to the safe management of the cardiac surgery.

Cor triatriatum: presentation, diagnosis and long-term surgical results.

BACKGROUND: Cor triatriatum sinister is a rare, surgically correctable, congenital cardiac anomaly. Fewer than 250 cases have been reported. It can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical). METHODS: A retrospective review of 28 consecutive patients diagnosed at the Royal Children's Hospital in Melbourne, Australia during a 22-year span from 1981 to 2003. RESULTS: There were 13 men (46%) and 15 women (54%). Fifteen patients were less than 1 year of age (neonates, [n = 7], 25%; infants, [n = 8], 29%). 17 patients (61%) had a communication between the right atrium and either the proximal or distal chamber. Fifteen patients (54%) had atypical cor triatriatum. Median age at presentation was 6 months (range, 0.6 to 240). Twenty-four patients (86%) had presented by 5 years of age. Five patients (18%) underwent emergency surgery. Median age at operation was 7 months (range, 1 to 243). Twenty-seven patients (96%) underwent preoperative transthoracic echocardiography. Nine patients (32%), all with atypical cor triatriatum, required cardiac catheterization for diagnosis. Twenty-seven patients (96%) were correctly diagnosed before treatment. Twenty-seven patients (96%) were treated surgically. The defect was approached through the right atrium in 26 patients (93%). There was 1 early death and 1 patient died 10 years after repair. Follow-up was 86% complete. At a median follow-up of 98 months (range, 0.2 to 284), all patients including those with atypical cor triatriatum were in New York Heart Association's function class 1. Post-repair survival was 96% and 88% at 5 and 15 years, respectively. CONCLUSIONS: Surgery offers good early and long-term results for both classic and atypical cor triatriatum.

Cor triatriatum sinistrum: a rare congenital cardiac anomaly presenting in an adult with chronic atrial fibrillation.

Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium is divided into proximal (dorsal or upper) and distal (ventral or lower) chambers by a fibromuscular septum. The upper chamber receives the pulmonary veins and the lower chamber contains the atrial appendage and the mitral valve. The 2 chambers communicate through a defect in the membrane. Cor triatriatum is often associated with other congenital cardiac anomalies. Most frequently, the upper chamber communicates with the right atrium through a patent foramen ovale or atrial septal defect, and the clinical symptoms simulate anomalous pulmonary venous return. Less commonly, the foramen ovale communicates with the distal chamber and the clinical features mimic mitral stenosis. When cor triatriatum is the only abnormality, the clinical findings are also similar to mitral stenosis with development of pulmonary hypertension and subsequent right ventricular hypertrophy and atrial enlargement. The diagnosis is usually made in infancy or childhood, and the lack of treatment results in death in 75% of patients. We report the case of a woman who presented much later in life. The patient was a 57-year-old female with a clinical history of chronic atrial fibrillation who presented to the emergency department because of a "funny sensation" in her chest, though she denied chest pain, nausea, vomiting, or diaphoresis. EKG revealed atrial fibrillation with a rapid ventricular response and a tachycardic rate of 157. She had a therapeutic level of digoxin, and cardiac enzymes were normal. The patient was admitted and placed on Cardizem drip. Serial EKGs remained normal and heart rate control was achieved. On hospital day 2, the patient became dyspneic and cyanotic. She went into cardiac arrest and died.Autopsy revealed cardiomegaly (610 g) with 4-chamber dilatation. A septum divided the left atrium into 2 chambers. The defect in the dividing membrane measured 1 cm in diameter. No other congenital defects were noted. The large size of the defect in the membrane likely accounted for the late onset of symptoms that allowed this patient to survive into adulthood without previous diagnosis or surgical intervention (which is usually required in childhood).

[A case report of Lucas-Schmidt IIA type Cor triatriatum in neonate].

Lucas-Schmidt IIA type of Cor triatriatum has been rarely reported. Since it shows the same hemodynamics as Darling IIb type TAPVC, it is important as a emergent surgical case in early infancy. We reported a male neonate with IIA type Cor triatriatum. He was admitted to our hospital because of cyanosis and dyspnea since birth. The echocardiographic examinations revealed the accessory chamber behind the left atrium and atrial septal defect. The view from the right atrium revealed that there was no direct connection between the accessory chamber and the true left atrium, and we diagnosed it Lucas IIA type Cor triatriatum. The abnormal diaphragma was resected, and the atrial septum was corrected with a Xenomedica patch. The patient had an uneventful postoperative course, and was discharged on the 25th postoperative day.

[Clinical classification and surgical treatment of cor triatriatum].

Twenty-one patients (age 35 days to 39 years) with cor triatriatum were treated surgically over a 17 year period at our institute. We define cor triatriatum as a congenital heart anomaly with the presence of an abnormal diaphragm that subdivides the left atrium into an accessory and a true left atrial chambers and that has "direct" hemodynamic communication between an accessory and a true left atrial chambers through one or more fenestration irrespective of accompaniment of an atrial septal defect or a partial anomalous pulmonary venous connection. By contrast with our findings of the hearts with cor triatriatum, every representative classification, such as Lucas-Schmidt's one, had some lack or duplication of the addressed types respectively. So we presented a clinical classification of cor triatriatum as follows: type I is the classical form with intact atrial septum, type IIa has an ASD between an accessory and a right atrial chambers, type IIb has an ASD between a right and a true left atrial chambers, and type IIc is a complexed form that has both communications of IIa's and IIb's. Of 14 typical cor triatriatum without isomerism nor other major complexed anomalies, 7 cases belonged to type I, and IIa 4, IIb 2, IIc 1 respectively. Of 7 so-called atypical cases, 5 cases belonged to type IIc and the rest two were unclassified complexed forms. Characteristically all of atypical cases had persistent left superior vena cava in common. 17 patients underwent total corrections and the rest 4 cases with single right ventricles underwent palliative operations for Fontan procedure in the near future. Transaccessory chamber approach for type I and IIb as the first choice provided ample exposure for complete excision of the obstructing membrane. Trans-right atrial approach were employed for closure of ASDs or for the other intraatrial partitions. Incision of the atrial septum should be avoided to prevent postoperative supraventricular arrhythmias if possible. There were no surgical death nor late death over 6 years follow-up period on the average. There existed some atypical cases that seemed very difficult to make differential diagnoses preoperatively, so careful surgical investigations into intraatrial anatomy should be made to perform proper corrections especially in the atypical cases.