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PubMed2006

Cor triatriatum: operative results in 20 patients.

Cor triatriatum is an uncommon cardiac anomaly occurring in 0.1% of children with congenital heart disease. We reviewed our results from 20 patients presenting with cor triatriatum in the last 20 years (from March 1983 to August 2002). Mean age at repair was 20 months (1-132 months). During a mean follow-up period of 31 months (2-156 months) there were no deaths. All patients were in sinus rhythm except one patient who needed pacemaker insertion one week after repair. A total of 14 patients were asymptomatic and 6 were in New York Heart Association Class I. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early, and when not associated with other severe cardiac anomalies.

Cardiac Catheterization↗
PubMed1993

Cor triatriatum dexter in two dogs.

Cor triatriatum dexter is a congenital heart defect in which the embryologic right sinus venosus valve persists as a septum within the right atrium. Cor triatriatum dexter was diagnosed in 2 dogs on the basis of clinical signs, two-dimensional echocardiography, and cardiac catheterization. In 1 of the dogs, the condition was successfully treated by surgical resection of the intra-atrial septum. In the second dog, the defect was associated with an incomplete persistent cranial left vena cava and Ebstein's anomaly; surgery was declined.

Animals↗
PubMed1979

Successful surgical correction of symptomatic cor triatriatum dexter.

An unusual case of cor triatriatum dexter is presented. Abnormal embryologic development of the eustachian valve caused nearly complete septation of the right atrium in a 67-year-old woman. Multiple supraventricular arrhythmias prompted clinical evaluation and diagnosis. Surgical excision of the anomalous membrane resulted in complete relief from arrhythmias and a clinical cure. Cor triatriatum dexter is rare and is infrequently diagnosed before postmortem study; however, once the diagnosis is extablished, the condition is amenable to a relatively simple surgical correction. Supraventricular tachycardia has not previously been reported to be associated with cor triatriatum dexter. We believe this is the first case in which such arrhythmias led to early diagnosis and successful surgical correction.

Aged↗
PubMed1991

[Transesophageal echocardiography in the diagnosis of cor triatriatum in the adult].

Cor triatriatum (CT) is a rare congenital defect, surgically correctable, and sometimes difficult to diagnose by cardiac catheterization. This report describes three young patients with this particular defect, one of whom was sent to us because of signs of right ventricular failure. The diagnosis of CT was made by transesophageal echocardiography and confirmed by cardiac catheterization and surgical data. The other two cases underwent cardiac catheterization and cardiac surgery during infancy for other congenital defects. The diagnosis of CT was made only during post-operative controls by transthoracic echocardiography. In these two cases transesophageal echocardiography provided the most valuable information about the morphological features of the membrane and the mitral valve, and about the flow between the two left atrial chambers.

Adolescent↗
PubMed1995

Transesophageal echocardiographic evaluation of cor triatriatum in children.

Four children with cor triatriatum underwent intraoperative transesophageal echocardiography. Two patients had cor triatriatum alone and two had associated complex congenital heart disease. Transesophageal echocardiography provided optimal imaging of these defects and provided unique information that facilitated surgical management in these children.

Child, Preschool↗
PubMed1991

Cor triatriatum dexter: recognition and percutaneous transluminal correction.

Cor triatriatum dexter is a rare congenital anomaly in which an obstructive membrane is located in the right atrium. The detection usually occurs after the sequelae of systemic congestion, coagulopathy, and hepatic dysfunction have set in, leading up to a high surgical risk. A percutaneous balloon correction of cor triatriatum dexter in a patient with advanced right-sided congestive symptoms and hepatic dysfunction is presented. This efficacious method is an alternative to surgical correction and could be extended to the more common cor triatriatum sinistra.

Adult↗
PubMed1975

Cor triatriatum: study of 20 cases.

Twenty cases of cor triatriatum are reported. The diagnosis was confirmed by necropsy in 16 cases and at the time of operation in 4. The lesion occurred as an isolated anomaly in 7 cases; in 13, other associated cardiac anomalies were present. Three anatomic types of cor triatriatum were identified in the cases studied at necropsy: diaphragmatic (10 cases), hourglass (3) and tubular (3). The diaphragmatic type was also present in all four cases in which the diagnosis was confirmed at operation. Associated anomalies were found in five cases of the diaphragmatic type and in each case of the hourglass of tubular types. In isolated cor triatriatum the clinical findings were characteristic of pulmonary venous and arterial hypertension. In two cases, one with a communication between the right atrium and the accessory left atrial chamber and one with partial anomalous pulmonary venous connection associated with cor triatriatum, the clinical findings suggested a large left to right shunt with pulmonary arterial hypertension. The clinical findings varied in the cases with associated anomalies, and it was difficult to determine the cause of disturbance of the circulation.

Adolescent↗
PubMed2005

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.

Child, Preschool↗
PubMed1993

[Cor triatriatum with atrial fibrillation as initial symptom in adults].

Cor triatriatum is a rare congenital heart disease. In its classic form its consists of a fibromuscular perforated membrane which divides the left atrium and obstructs blood flow from pulmonary veins to mitral orifice. Clinical symptoms and signs resemble those of mitral stenosis. Traditionally cor triatriatum was encountered most often in infancy and childhood. We describe the case of a young man with cor triatriatum and atrial fibrillation, with syncope. He was operated on with total resection of the obstructing membrane. Modern echocardiography has made diagnosis more easy. Thus, an increasing number of cases of cor triatriatum are diagnosed in adults with few or no symptoms. Symptoms, diagnosis and treatment are discussed, with emphasis on these patients.

Adult↗
PubMed1989

Cor triatriatum masked by coexisting COPD in an adult.

Cor triatriatum presenting in adulthood is extremely rare. We describe a case of adult cor triatriatum in which the diagnosis was initially masked by the concomitant existence of COPD. Cardiac catheterization revealed only slightly elevated pulmonary wedge pressure despite severe pulmonary arterial hypertension. Both the primary lung disease and cor triatriatum greatly accentuated the pulmonary vascular disease which led to a reduction of pulmonary blood flow. Consequently, pulmonary venous obstruction was masked and was not reflected by measuring pulmonary wedge pressure. The diagnosis was made by two-dimensional echocardiography and left ventriculography.

Cardiac Catheterization↗
PubMed1992

[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.

Adult↗
PubMed2005

Cor triatriatum dexter in two adult patients.

Cor triatriatum dexter, in which the right atrium (RA) is divided into two chambers by a membrane, is a very rare congenital abnormality. In this abnormality, there is a high incidence of associated congenital abnormalities, particularly of the right heart. We presented two men with cor triatriatum dexter. Echocardiography is discussed as a non-invasive diagnostic tool.

Adult↗
PubMed1993

[A case report of cor triatriatum benefit of MRI for preoperative diagnosis and surgical method].

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.

Child, Preschool↗
PubMedDate not supplied

Two-dimensional, spectral Doppler, and color flow imaging in adults with acquired and congenital cor triatriatum.

Four adult patients ranging in age from 37 to 72 years with cor triatriatum diagnosed by two-dimensional, spectral Doppler, and color flow imaging are presented, including a previously undescribed "acquired" form after orthotopic heart transplantation and the oldest documented patient with cor triatriatum. The echocardiographic features of cor triatriatum with use of surface and transesophageal imaging are reviewed, with particular emphasis on the color flow findings. Color flow imaging has simplified the diagnosis and assessment of cor triatriatum, including the demonstration of other often-associated congenital cardiac anomalies.

Adult↗
PubMed1992

Cor triatriatum sinistrum: one institution's 28-year experience.

Twelve patients with cor triatriatum sinistrum were treated over a 28-year period. Their ages ranged from 1 month to 7.5 years. Congestive heart failure was the most common presentation. Cardiac catheterization was performed on six of the 12 patients and a correct diagnosis of cor triatriatum was made on angiography in only four of the six. Of the remaining six patients, three were diagnosed as having cor triatriatum by echocardiography and three by autopsy. Echocardiography is now considered to be the diagnostic modality of choice in our institution. Seven patients were operated on and five died prior to diagnosis or treatment Associated cardiac anomalies included persistent left superior vena cava, atrial septal defects, coarctation of the aorta, and total anomalous pulmonary venous drainage. A right atrial, transseptal approach to the common pulmonary chamber and excision of the left atrial membrane was found to be the treatment of choice and was used in six of the seven patients operated on. One patient died in the postoperative period. Thus, cor triatriatum sinistrum, a rare and potentially lethal congenital cardiac anomaly, can be diagnosed by echocardiography and successfully treated surgically with a low operative mortality.

Child↗
PubMed2001

Unusual finding of cor triatriatum in a newborn with hypoplastic left heart syndrome.

Cor triatriatum is a rare anomaly in which the left atrium is partitioned into 2 distinct chambers with a variable degree of obstruction to left atrial inflow. This anomaly often occurs in isolation but can occur in combination with other cardiac lesions. The association of cor triatriatum with hypoplastic left heart syndrome has not been reported. We describe a newborn with hypoplastic left heart syndrome who also had the unusual finding of cor triatriatum sinistrum.

Cor Triatriatum↗
PubMed1992

[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.

Cor Triatriatum↗