A rare cause of syncope: Cor triatriatum
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Cor triatriatum
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Cor triatriatum
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Objective:To explore the diagnosis and surgical treatment of cor triatriatum.Method:8 patients,from 1994 to 2002,all of them were surgically corrected simultaneously.Result:The surgical treatment of this team was satisfied.Conclusion:Color Doppler echocardiography (CDE)is of great value in the diagnosis of cor triatriatum;Cor triatriatum and associated defects should be simultaneously corrected.
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Cor triatriatum is defined as a membrane within the left atrium, which might lead to restricted pulmonary venous return. Diagnosis is usually achieved by echocardiography in early infancy. Therapy of choice is the excision of the membrane. Herein, successful correction of cor triatriatum in a 4-year-old girl is presented, and the clinical features, echocardiographic findings, and the surgical treatment are discussed.
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Cor triatriatum
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Cor triatriatum is a rare congenital heart disease (0.1% of all congenital cardiac defects), but a higher incidence, up to 0.4% has been reported in autopsies of pts with CHD (1, 2, 7). There are two types: left and right. Cor triatriatum sinister is more common that dexter. Cor triatriatum dextrum is extremely rare. Fewer than 300 cases of cor triatriatum have been reported. It can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical). It's a surgically correctable CHD and can occur as an isolated defect (classic) or in association with other congenital cardiac anomalies (atypical).A retrospective review of three patients with Cor triatriatum, diagnosed at University Children's Hospital, during a eight year period (2000-2007). Among 1671 patients with CHD, the diagnosis of cor triatriatum has been established in three patients (0.18%). There were two boys and one girl, aged two years, 6 months and nine years, respectively. All of them had cor triatriatum sinister, with a communication between the right atrium and either the proximal or distal chamber. The first patient had a classic form of cor triatriatum, with a small hole in the diaphragm between atria, which imitated mitral stenosis, while the third patient had also mitral valve prolapse, but the hole between atria was unrestrictive. The second patient manifested atypical form, with many, additional defects: except large, unrestrictive ASD with a very small hole in the diaphragm between two atria, he had also total anomalous pulmonary venous return, draining in coronary sinus, large perimembranous VSD, hypoplastic aorta with coarctation, and high pulmonary vascular resistance. In the second patient, the diagnosis has been performed at 6 months of age, but due to lack of cardio-surgery and poor possibilities for going abroad for operation--finally he had been operated seven months later, but he died a week after surgery. The first patient has been successfully operated, immediately after the diagnosis was performed, while the last patient was diagnosed incidentally at the age of nine. She was symptoms free up to now, but recently she was developing symptoms and was successfully operated.Cor triatriatum is more prevalent than is thought before echocardiography era. Echocardiography was method of choice in the diagnosis of typical forms, while in a atypical form cardiac catheterization was also performed. Two patients with classic form of CT were successfully operated, while the patient with atypical form and many additional cardiac anomalies died after cardio surgery. The main predictors for prognosis are: the size of the hole in the diaphragm between two chambers of atrium, additional cardiac malformations, and time of surgery.
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A 10-year-old boy presented with complaints of poor weight gain, progressively increasing dyspnea since early childhood, and orthopnea. Echocardiogram was suggestive of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dextrum) is divided into two compartments by an abnormal fibromuscular septum usually with an opening between the two chambers associated with varying degrees of obstruction of the pulmonary veins. The pulmonary venous return is followed by pulmonary arterial hypertension. Cor triatriatum literally means a heart with three atria (triatrial heart), namely, the superior chamber, the inferior chamber or the true atrium, and the atrial appendage. This anomaly occurs in 0.4% of patients with congenital heart defects, with a slight male predominance. The present case of cor triatriatum sinister is being reported in view of the fact that the child remained undiagnosed till the age of 10 years, despite being “sick since a little boy” requiring multiple hospital admissions for chest infections. He had been receiving treatment for “chest problem” and had been on bronchodilators. The classical echocardiographic findings would be of academic interest as well.
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The echocardiographic features of congenital left ventricular inflow obstruction are described in six patients. The echocardiograms in two patients with cor triatriatum were distinguished by normal mitral valve motion and an abnormal echo within the left atrium. In two patients with supravalvar mitral ring, in addition to abnormal mitral valve motion, an abnormal echo, presumably originating from the obstructive membrane, was located between the anterior and posterior mitral leaflets. In two cases of parachute mitral valve, mitral valve motion was abnormal. In one of these cases there were multiple mitral valve echoes similar to those found in supravalvar mitral ring. The echocardiographic identification of an obstructive membrane within the left atrium is difficult because of the occurrence of artifacts. However, membranes may be identified if careful scanning techniques are employed in patients in whom left ventricular inflow obstruction is suspected. The echocardiogram is useful in detecting mitral val...
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The echocardiographic features of congenital left ventricular inflow obstruction are described in six patients. The echocardiograms in two patients with cor triatriatum were distinguished by normal mitral valve motion and an abnormal echo within the left atrium. In two patients with supravalvar mitral ring, in addition to abnormal mitral valve motion, an abnormal echo, presumably originating from the obstructive membrane, was located between the anterior and posterior mitral leaflets. In two cases of parachute mitral valve, mitral valve motion was abnormal. In one of these cases there were multiple mitral valve echoes similar to those found in supravalvar mitral ring. The echocardiographic identification of an obstructive membrane within the left atrium is difficult because of the occurrence of artifacts. However, membranes may be identified if careful scanning techniques are employed in patients in whom left ventricular inflow obstruction is suspected. The echocardiogram is useful in detecting mitral valve abnormalities in these patients and is valuable in cases where mitral valve replacement is contemplated.
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Successful correction of cor triatriatum in a 4½-month-old infant is presented. Altogether six patients with cor triatriatum underwent surgical correction during the last eight years. All but one survived the operation. The clinical features and the surgical treatment are discussed.
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Summary: M‐mode echocardiography displayed abnormal echoes in the left atrium and posterior to the mitral leaflets in a child with severe heart failure. Cor triatriatum was demonstrated by angiography and cured by surgical excision. Subsequent echo studies no longer showed the abnormal structure.
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