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Cor triatriatum presenting in adulthood is extremely rare. This paper reports a case of cor triatriatum initially masquerading as mitral stenosis, which was later complicated by myxomatous mitral valve degeneration with severe regurgitation, which necessitated mitral valvular replacement. To the authors' knowledge, such a combination has not been reported in the literature.
Cor triatriatum is a congenital heart defect resulting in abnormal septation of the left atrium by a fibromuscular membrane. Echocardiography has improved the preoperative diagnosis of this rare congenital heart defect. We report a case where transesophageal echocardiography proved useful in the intraoperative diagnosis of a previously undetected cor triatriatum.
Cor triatriatum, although rare in adults, is often associated with anomalous pulmonary venous return. Accurate diagnosis of the pulmonary venous return and its anatomic connections is essential in planning proper surgical correction. This case illustrates the advantage of selective pulmonary artery angiography in obtaining accurate detail of the pulmonary venous anatomy from digital subtraction levophase images. Selective right and left pulmonary angiography was performed using a balloon catheter to simultaneously occlude the opposite pulmonary artery. This technique was well tolerated and greatly enhanced visualization of the anomalous pulmonary venous connections, allowing proper planning for corrective surgery.
Cor triatratum is a rare congenital malformation. Problems in diagnosis and surgery arise due to associated anomalies such as atrial septal defects and anomalous pulmonary venous drainage. A 21-year-old man proved to have a rare variant of cor triatiatum in which both the upper and lower compartments of the left atrium communicated with the right atrium through two separate atrial septal defects. In addition, he had anomalous drainage of the right superior pulmonary vein into the superior vena cava. Intracardiac repair was undertaken successfully.
Cor triatriatum dextrum was an incidental echocardiographic finding in a 5 year old boy and a 9 year old girl, who were evaluated for the presence of a heart murmur. Apart from slightly enlarged right atria, they had otherwise normal hearts and were symptomless. Most of the patients reported until then had been symptomatic due either to the persistent right sinus venosus valve or to commonly associated structural heart anomalies. Therefore, the clinical significance of asymptomatic cor triatriatum dextrum remains coniectural, but might lie in the possibility of development of arrhythmia, progressively worsening interference with the systemic venous return and thrombus formation.
Cor triatriatum dextrum is a rare malformation of the right atrium usually without typical signs or symptoms. Today the intra vitam diagnosis of C.t.d. is done by echocardiography and angiocardiography. Once the diagnosis is established, surgical correction of this anomaly is possible in symptomatic patients. When the presence of an abnormal membrane in the right atrium is not recognized before operation, the outcome may be unsatisfactory.
Cor triatriatum was diagnosed in a 32-year-old woman (Case 1) and a 36-year-old man (Case 2). The definitive diagnosis in Case 1 was made by transthoracic 2-D echocardiography, in Case 2 (after a chance finding) only after additional transoesophageal echocardiography. Colour Doppler echo in Case 1 provided information on the number and localization of membrane openings, while in Case 2 simultaneous measurement of maximal flow velocity and normal right-sided pressures indicated that the anomaly was haemodynamically insignificant owing to the size of the central opening in the membrane (maximal diameter 2.1 cm). In Case 1, abnormal haemodynamic findings on right-heart catheterization provided the indication for surgery and the membrane was successfully removed. Postoperatively the patient was much improved and cardiac catheterization demonstrated normal values.
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.
A case of cor triatriatum is described in which transseptal left heart catheterization was useful in making the diagnosis by allowing easy access to the accessory chamber for pressure measurements and angiography.
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.
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.
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.
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.
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.
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.
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.
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.
A case of cor triatriatum in a 51-year-old man was demonstrated by rapid acquisition cine-CT (CCT). The CCT findings are compared with cardiac catheterization and two-dimensional echocardiography.