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PubMed1992

Cor triatriatum sinister, not mitral stenosis, in an adult with previous Sydenham's chorea: diagnosis and preoperative assessment by cross sectional echocardiography.

In cor triatriatum sinister, one of the rarest congenital cardiac anomalies, a membrane divides the left atrium into a pulmonary venous component above and the vestibule below. The importance of the anomaly lies in the effects of the resultant pulmonary venous obstruction that usually present in the first year of life and can mimic obstructed total anomalous venous drainage or congenital mitral stenosis. A case presented as mitral stenosis in the third decade of life, ten years after a well documented episode of Sydenham's chorea. The diagnosis was made rapidly by transthoracic echocardiography and transoesophageal echocardiography was used for complete assessment. Cardiac catheterisation added nothing to the non-invasive diagnosis or the preoperative assessment. Uncomplicated corrective surgery was undertaken.

Adult↗
PubMed1992

Cor triatriatum associated with total anomalous pulmonary venous drainage in the setting of mitral atresia and a restrictive interatrial communication.

A rare variant of cor triatriatum is described in which the proximal "accessory" chamber received all four pulmonary veins and drained into the levoatriocardinal vein and then into the innominate vein. The distal "true" chamber contained the left atrial appendage. The left atrioventricular valve was atretic and the interatrial septum was restrictive.

Angiography↗
PubMed1994

Biplane transesophageal echocardiographic diagnosis of cor triatriatum.

A transthoracic echocardiogram disclosed a linear structure in the left atrium of an adult presenting with atypical chest pain. Biplane transesophageal echocardiography revealed a cor triatriatum. The longitudinal plane was most useful for the assessment of the membrane orifice and the Doppler flow pattern.

Cor Triatriatum↗
PubMed1977

[Cor triatriatum in infants. Four cases with two surgical successes].

Four cases of cor triatriatum are reported in 6 weeks to 23 months old infants. Two were associated with partial anomalous pulmonary venous connection and one with a mild aortic stenosis. Two were successfully operated on and are now normal children: one of these underwent surgery at 3 months with an hemodynamic control one year later. The two others died: the youngest because of an inadequate overloading transfusion, the other soon after pulmonary angiography. Problems in diagnosis and treatment of this rare but very tractable condition are discussed.

Female↗
PubMed1983

[2-dimensional contrast echocardiographic diagnosis of associated atrioventricular canal and cor triatriatum].

2D echocardiography provided the diagnosis of partial atrioventricular canal associated with cor triatriatum in a 4 year old child. The apical four chamber view showed an ostium primum and the presence of an abnormal transverse echo in the left atrium, dividing it into ventral and dorsal chambers. Contrast echocardiography in the four chamber apical view showed an atrial right to left shunt and only opacification of the ventral chamber of the left atrium at the mitral orifice. On the other hand, the dorsal chamber which received the pulmonary veins could not be opacified by contrast. The contrast injection allowed the diagnosis to be confirmed by demonstrating the division of the left atrium into two chambers.

Child, Preschool↗
PubMed1982

[Cor triatriatum: results of surgical treatment. A propos of a series of cases].

Cor triatriatum is a rare heart defect that may be surgically repaired, with good results when preoperative diagnosis is correct. Ultrasonography, angiography and catheterism reduce preoperative diagnostic errors such as abnormal pulmonary venous return. The authors report their experience with 7 cases, and discuss diagnostic problems and causes of errors.

Angiography↗
PubMed1989

Cor triatriatum sinister: echocardiographic assessment.

A 16 year old white female presented with paroxysmal cough and hemoptysis of recent onset. Cardiac evaluation was consistent with pulmonary hypertension. Cor triatriatum sinister was diagnosed by echocardiography. The clinical presentation and the echocardiographic features of this anomaly are discussed.

Adolescent↗
PubMed1989

[Two-dimensional echocardiographic findings of cor triatriatum: differential diagnosis from total anomalous pulmonary venous connection to the coronary sinus].

Two-dimensional echocardiograms were reviewed in six patients with cor triatriatum (CTA) and six patients with total anomalous pulmonary venous connection to the coronary sinus (TAP) to characterize the echocardiographic features of distinguishing both diseases. Patients' ages ranged from nine days to 25 months in CTA, and from one to 11 months in TAP. The echocardiographic findings of CTA included three findings which were previously reported; the "double arch appearance" of the intra-atrial membrane, a "recess" which developed between the posterior wall of the aorta and the membrane, and the small size of the "recess", and two additional peculiar findings: the direction and echo density of the intra-atrial membrane. The "double arch appearance" of the intra-atrial membrane could not be detected in any of the CAT nor in TAP cases, except in one TAP patient who had a drainage vein obstruction. The "recess" was found in all six TAP patients, but it was recognized only in three of six patients with CTA. Among six patients with TAP, the size of a "recess" was small in two and large in one patient. Among three patients with CAT who had a "recess", the size was small in two and large in one patient: Thus, the size had little to do with differentiating CTA and TAP. The intra-atrial membrane ran parallel with the mitral valve ring in all patients with CTA except one in whom the membrane could not be identified because of the presence of endocardial cushion defect and a round membrane. On the contrary, the membrane ran parallel with the posterior wall of the common pulmonary venous chamber in all patients with TAP. The echo of the membrane was of low density in all patients with CAT and in three of six patients with TAP. The "double arch appearance" of intra-atrial membrane, the presence of a "recess", and the size of the "recess" were not characteristic echocardiographic features which could distinguish CAT from TAP. However, the direction of the membrane was useful as a means of distinguishing two anomalies. The intra-atrial membrane runs parallel with the posterior wall of the common pulmonary venous chamber in TAP, and with the mitral valve in CTA. The echo density of the membrane could not clearly differentiate CAT from TAP, but it was of low density in all patients with CTA.

Child, Preschool↗
PubMedDate not supplied

Cor-triatriatum with atrial septal defect and bicuspid aortic valve diagnosed by cross-sectional echocardiography.

We report a rare case with a combination of cor-triatriatum, large secundum atrial septal defect and bicuspid aortic valve. The diagnosis was made by cross-sectional echocardiography. A defect was present between proximal (accessory) left atrial chamber and right atrium decompressing the accessory left atrial chamber. The large left to right shunt resulted in severe pulmonary arterial hypertension.

Adult↗
PubMed2001

Congenital pulmonary vein stenosis associated with cor triatriatum.

We report an 11-year-old boy who underwent surgical correction for stenosis of the right and left lower pulmonary veins at their junction with the left atrium and associated cor triatriatum. The embryology and therapeutic approaches to congenital pulmonary veins stenosis are discussed.

Child↗
PubMed1993

A case of cor triatriatum with end-stage renal disease: successful management of open heart surgery under hemodialysis, subsequent renal transplantation and pregnancy.

A female patient who had open heart surgery for cor triatriatum under hemodialysis, subsequent kidney transplantation and pregnancy is reported. We performed hemodialysis on the patient before, during and after heart surgery to control renal failure. Two years after heart surgery, she received a kidney graft from her mother. The kidney graft showed good function. She was treated with azathioprine and prednisone. Three years after renal transplantation she delivered a healthy male infant by elective Caesarean section at 37 weeks' gestation. Mother and infant did well following delivery. There was lack of hypertension, proteinuria, signs of graft rejection, and recurrence of heart failure during pregnancy. She showed serum creatinine level < 2 mg/dl, a prednisone of < 2 mg/kg/day. Elective Caesarean section has improved hydronephrosis due to the compression of the fetus. The aforementioned good criteria contributed to the successful pregnancy of the renal transplant patient in our experience. We believe early surgical intervention overcomes complicated heart disease even with endstage renal disease, and it gives a chance to receive renal transplantation and have a healthy child. To our knowledge, this is the first report that has described the successful management of open heart surgery under hemodialysis, subsequent renal transplantation and pregnancy in a female patient with chronic renal failure.

Adult↗
PubMed1994

Giant congenital coronary artery fistula to left superior vena cava and right atrium with compression of left pulmonary vein simulating cor triatriatum--diagnostic value of magnetic resonance imaging.

A 3-year-old girl with a continuous murmur on auscultation was found to have left coronary artery to right atrial fistula simulating cor triatriatum sinister on echocardiography. Coronary angiography of the patient was also insufficient to clarify the exact pathologic anatomy. Magnetic resonance imaging showed that the main drainage of the aneurysmal coronary artery was to the left superior vena cava, resulting in a diffuse arteriovenous fistula in the lower lobe of the left lung by compressing its pulmonary vein.

Child, Preschool↗
PubMed1987

Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium.

Two-dimensional echocardiographic findings and surgical repair in three infants with atrioventricular (AV) canal and pulmonary venous obstruction are described. The AV canal was complicated by cor triatriatum in two patients and by double outlet right atrium in the other. In patients with AV canal, anatomic obstruction of pulmonary venous return should be excluded by detailed cardiac ultrasound examination.

Cor Triatriatum↗
PubMed2001

[Cor triatriatum dextrum resulting from the persistence of embryonic remnants of the right valve of the sinus venosus: prevalence and echocardiographic aspects in a large consecutive non-selected patient population].

BACKGROUND: Cor triatriatum destrum is a rare congenital malformation, usually associated with complex right heart abnormalities, characterized by a membrane that divides the right atrium into two chambers. It is considered the result of the incomplete and abnormal regression of the embryonic right valve of the sinus venosus. When the valve regresses the cephalic portion forms the crista terminalis and the caudal portion develops into the Eustachian and Thebesian valve. With an incomplete regression, a fenestrated or an unfenestrated membrane may persist in the right atrium. We describe the clinical and echocardiographic characteristics of this type of remnants in the right atrium. METHODS: In a 4-month period, 1728 transthoracic two-dimensional and Doppler echocardiographic studies were consecutively performed. RESULTS: Twenty-seven patients (16 males, 11 females, mean age 55 +/- 21 years, range 1-87 years) were occasionally recognized to be affected by a membrane inside the right atrial cavity. On the basis of its echocardiographic appearance (a membranous structure connecting the orifice of the inferior vena cava to the atrial septum with evident fenestration in 74% of cases), absence of associated congenital abnormalities (92.5% of cases), interatrial shunt or abnormal flow pattern of venous systemic return, a diagnosis of a normal benign anatomic variant of sinus venous remnants (similar to the so-called "Chiari's network") could be made in every case. CONCLUSIONS: The incomplete regression of the embryonic right valve of the sinus venosus may leave a fenestrated or an unfenestrated membrane in the right atrium that should be considered a normal benign variant of the so-called "Chiari's network".

Adolescent↗
PubMed1989

[The roentgenologic diagnosis of cor triatriatum sinistrum].

Varied in compliance with the presence and size of the septal foramen, existence and location of the atrial shunt and abnormal pulmonary venous connection, the haemodynamic status of the reported 10 cases of cor triatriatum sinistrum might be classified into four types: Pulmonary venous hypertensive, polyemic, cyanotic polyemic and normal. Accordingly, the roentgenologic features of this anomaly manifested differently and mimicked mitral stenosis or atrial septal defect.

Adolescent↗
PubMed2005

Association of a Cor Triatriatum Sinister and a right partial anomalous pulmonary venous return. A case report.

A 56-day old baby girl referred for recurrent respiratory tract infections and stridor, showed a rare association of a Cor Triatriatum Sinister (CTS) and a right Partial Anomalous Pulmonary Venous Return (PAPVR), in the form of a stenosed Scimitar vein, draining the right pulmonary veins to the subdiaphragmatic inferior vena cava (IVC), on echography and angiography. The baby underwent successful surgical repair of the CTS, by resection of the obstructive false membrane and reconnection of the Scimitar vein to the left atrium. At the age of 5 years, the child is doing well without any evidence of obstruction on the right or the left pulmonary venous drainage.

Abnormalities, Multiple↗
PubMed1998

Cor triatriatum in a 70-year-old woman: role of transesophageal echocardiography and dynamic three-dimensional echocardiography in diagnostic assessment.

In this report we describe a 70-year-old woman with unrecognized nonobstructive cor triatriatum (CT). She had concomitant mitral regurgitation and coronary artery disease. Diagnosis of CT and its hemodynamic assessment were accomplished by transesophageal echocardiography. Dynamic three-dimensional echocardiography demonstrated multiple fenestrations in the left atrial membrane. Intraoperative findings confirmed the diagnostic accuracy of three-dimensional echocardiography. To our knowledge, this is the first case of CT that has anatomic correlation with three-dimensional echocardiography.

Aged↗
PubMed1991

[Cor triatriatum].

A 16-year-old patient was evaluated for congestive heart failure and pulmonary hypertension, suggesting mitral valve stenosis. A left atrial membrane with a 4 mm orifice was seen in echocardiography and angiography, and a diagnosis of cor triatriatum was made. The membrane was successfully removed by surgery.

Adolescent↗