Cases reported "Cor Triatriatum"

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1/7. Divided left atrium associated with supravalvar mitral ring.

    Reported is a case with a rare association of divided left atrium, supramitral stenosing ring of the left atrium, connection of the left superior caval vein to the roof of the left atrium, unroofed coronary sinus with an interatrial communication at the mouth of the unroofed sinus and ventricular septal defect. The need for a complete echocardiographic examination in the presence of pulmonary venous obstruction is emphasized. Surgery was successful in spite of significant preoperative pulmonary hypertension.
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2/7. Divided right atrium associated with extensive coronary vein abnormalities.

    This report describes a case of a divided right atrium associated with coronary vein abnormalities. An 18-year-old woman who had a past history of surgery for repair of a divided right atrium and atrial septal defect developed exertional dyspnea 8 years after the surgery. Selective coronary angiography showed a dilated right coronary artery with a fistulous communication to the right atrium, tortuous coronary veins draining directly into the cardiac chambers, and the absence of the coronary sinus. Embryologically, regression failure of the right venous valve is hypothesized to have created both the divided right atrium and extensive coronary vein abnormalities.
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3/7. Spontaneous echo contrast of unexpected etiology.

    cor triatriatum sinister is a rare congenital anomaly characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers: one entering the four pulmonary veins, the other connecting to the mitral valve. The extent of the communication between the two chambers and the presence of associated lesions determine the severity of symptoms and the complications. We report the case of a 20-year-old man firstly diagnosed with obstructive cor triatriatum sinister and severe pulmonary hypertension.
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4/7. 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.
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5/7. diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature.

    BACKGROUND: cor triatriatum is rarely found in adults and its diagnosis is mostly dependent on transesophageal echocardiogram. We present a case of an adult male with cor triatriatum and discuss our approach to diagnosis with a review of literature. CASE: A 49-year-old male presented with progressive exertional dyspnea for the past two-and-half years. A transthoracic echocardiogram revealed a thin linear echo-dense structure traversing the left atrium (LA). A transesophageal echocardiogram demonstrated a thin immobile membrane in the LA attached medially to the interatrial septum. No fenestration of the membrane was seen. A swirling spontaneous contrast was noted in the posterior chamber. color Doppler did not reveal any flow across the membrane. A CT scan of the chest was unhelpful to differentiate extracardiac versus intracardiac origin. Contrast echocardiogram with Optison was performed, which showed a differential opacification of the two atrial chambers and delayed emptying of contrast into true LA establishing communication between the chambers. Left and right heart catheterization were performed with simultaneous pulmonary capillary wedge and left ventricular end diastolic pressure measurement, revealing a mean gradient of 17 mmHg. Surgical correction of the membrane was recommended. Intraoperatively, an orifice of 0.7 cm was identified in an eccentric position with mosaic pattern of continuous turbulent flow across the membrane by color Doppler. DISCUSSION: Our case demonstrates the impact of contrast echocardiography in a situation where the transesophageal approaches were limited even with the aid of color Doppler.
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6/7. Unusual "midseptal" sinus venosus defect.

    An unusual case of an interatrial communication similar to a sinus venosus defect associated with an overriding pulmonary venous chamber is reported. There was no direct connection of the pulmonary venous system with either the superior vena cava or the low right atrium, as is usual with sinus venosus defects. This defect may represent the result of a sinus venosus defect associated with cor triatriatum.
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7/7. cor triatriatum associated with degenerative aortic insufficiency in an adult patient.

    cor triatriatum is a rare cardiac anomaly which can be congenital or acquired in origin. Congenital cor triatriatum is due to an alteration of the common pulmonary vein resorption and therefore the left atrium is divided into two chambers, a proximal one, in communication with the pulmonary veins, and a distal one, in communication with the mitral valve orifice. The diagnosis is usually made at birth, but in rare cases, when the communication between the two chambers is wide and the patient is asymptomatic, the lesions may be diagnosed incidentally during a routine echocardiographic examination. We report a 32-year-old man, admitted to our hospital with a diagnosis of aortic insufficiency, in whom echocardiography revealed the presence of cor triatriatum. The patient underwent aortic valve replacement and resection of the atrial membrane. histology of the aortic valve revealed myxoid degeneration of the spongiosa.
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