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Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign.

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.

Cor Triatriatum↗

Cor triatriatum: a cause of hemoptysis.

Hemoptysis can be caused by either pulmonary or extrapulmonary causes. Congenital heart disease should be considered as a possible cause in patients who have no obvious evidence of pulmonary disease. We report on an 8-year-old girl who presented with recurrent hemoptysis without other cardiopulmonary signs, except for mild tachypnea and a prominent pulmonic component of the second heart sound, suggesting pulmonary hypertension. A chest X-ray revealed pulmonary venous congestion without other parenchymal disease. An echocardiogram revealed classical cor triatriatum, with a 6-mm orifice in the anomalous septum. Cardiac evaluation should be considered in patients with hemoptysis unexplained by pulmonary causes, even in the absence of overt cardiac symptoms.

Child↗

Cor triatriatum and anomalous pulmonary venous return: an unusual case.

In December 1954, a 29-year-old man with cor triatriatum underwent partial surgical repair consisting of left atrial implantation of a single anomalous pulmonary vein that had been draining into the innominate vein. In April 1989, at age 64, he returned for definitive cardiac repair. The contrast between these 2 procedures illustrates the tremendous progress that has been made in cardiac diagnosis and surgery during the past 35 years.

Journal Article↗

[A surgical case report--rare association of tetralogy of Fallot with cor triatriatum, unroofed coronary sinus and persistent left superior vena cava].

We experienced the rare association of Tetralogy of Fallot with cor triatriatum, unroofed coronary sinus and persistent left superior vena cava. Only one case of this association has ever been reported in the literature. We successfully performed a total correction which consisted of a resection of the intra-left atrial fibrous membrane, reconstruction of the LSVC return pathway to the right atrium with PTFE graft, atrial partition with a bovine pericardial patch and the conotruncal repair for Tetralogy of Fallot. Cardiac catheterization 6 months after operation demonstrated the smooth drainage from LSVC into the right atrium without pressure gradient. His postoperative course has been uneventful.

Cardiac Surgical Procedures↗

[A case of cor triatriatum diagnosed by echocardiography on pre-anesthetic examination].

A 69-year-old man was planned for elective surgery of the lumbar vertebral disk herniation. We performed a pre-anesthetic examination. He had a mild cardiomegaly (CTR = 55%) on chest X-ray examination, and ST-T change on electrocardiogram. His electrocardiogram showed negative T wave in III and aVF, ST elevation in I, aVL, V1-3, and flat T wave in V5-6. But he was without any symptoms of chest occlusion. He had no other abnormal laboratory data and abnormality in physical examination. We did echocardiography on him and cor triatriatum was diagnosed. The flow from the accessory chamber was 0.44 m.s-1. There were no abnormalities in the reflux of the pulmonary vein. We managed him under general anesthesia for operation, and took care to prevent right heart failure. There were no complications in peri-operative period. It was very important to perform pre-anesthetic examination by anesthetic specialist. Echocardiogram is useful for pre-anesthetic examination, if cardiovascular disease is suspected by chest symptom, electrocardiogram or chest X-ray examination.

Aged↗

[Growth in children with the exudative enteropathy syndrome due to a congenital heart defect--cor triatriatum dextrum].

Retarded growth in a child can be the sign of serious chronic disease. The authors present an account of a six-year-old boy where growth retardation persisted at least from the age of three. During this period his height dropped from the zone between the 25th and 50th percentile into the zone between the 3rd and 10th percentile. From the clinical point of view a large abdomen, loose stools and hypocalcaemia with tetany were striking, as they were moreover refractory to vitamin D2, calcitriol and calcium administration by the oral route. The authors revealed severe hypoproteinaemia, a 150 times increased value of alpha-1-antitrypsin in faeces, and exudative enteropathy syndrome was diagnosed. The cause was venous congestion due to a rare heart disease--cor triatriatum dextrum. The septum in the right atrium was resected. Immediately after surgery the consistency and frequency of stool decreased. Calcaemia and plasma protein levels reached normal levels within two months. A growth spurt of 11 cm/year followed. Fifteen months after operation the patient's height reached almost the 50th percentile.

Child↗

Cor triatriatum with repeated episodes of syncope in an eighteen month-old girl: a rare cause of cardiogenic syncope.

Syncope can be caused by either cardiogenic or noncardiogenic causes. Structural heart diseases should be considered as a possible cause of recurrent episodes of syncope if no other causes are found. We report an 18 month-old girl who presented with recurrent syncope as well as dyspnea, failure to thrive, and frequent episodes of pulmonary infections, suggesting congestive heart failure. A prominent pulmonic component of the second heart sound and congested lungs noted from chest X-rays indicated pulmonary hypertension. An echocardiogram revealed an abnormal membrane with a small orifice in the left atrium (LA). An intact atrial septum and no evidence of other congenital heart diseases were observed. The result after surgical resection of the membrane was good during follow-up. Cor triatriatum may be one of the structural heart diseases responsible for repeated unexplained syncope. An echocardiogram performed by an experienced cardiologist would facilitate the diagnosis considerably and an excellent prognosis can be achieved via surgical resection of the obstructing membrane.

Cor Triatriatum↗

Endocardial cushion defect associated with cor triatriatum sinistrum or supravalve mitral ring.

Clinical and angiographic or autopsy data, or both, on three children with a subdivided left atrium (cor triatriatum) and an associated endocardial cushion defect are reviewed. (One child had ostium primum defect, and two had complete atrioventricular [A-V] canal.) A fourth patient demonstrates the difficulties in differentiating subdivided left atrium from supravalve mitral stenosis in the presence of an endocardial cushion defect. The clinical findings are greatly influenced by the endocardial cushion defect. A pressure gradient between the pulmonary wedge and (left or right) ventricular end-diastolic pressures in patients with an endocardial cushion defect indicates pulmonary venous obstruction and should alert one to the possibility of these combined lesions. The exact diagnosis is made with injections of angiographic contrast medium into the proximal and distal left atrial chambers, to documented the respective relations of the pulmonary veins, left atrial appendage and A-V valves to these atrial chambers. All three patients with an endocardial cushion defect and a subdivided left atrium had an associated patent ductus arteriosus. The common association of subdivided left atrium with intracardiac, pulmonary venous and aortic anomalies is again demonstrated.

Cardiac Catheterization↗

Echocardiographic diagnosis of cor triatriatum.

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.

Cardiac Catheterization↗

[Cor triatriatum sinister: unusual cause of neonatal respiratory distress].

We report the case of a newborn presenting with neonatal respiratory distress due to acute pulmonary edema, the underlying diagnosis being cor triatriatum sinister. This rare anomaly can be lethal in the short term. However, it can be completely cured surgically provided that diagnosis is made on time.

Child, Preschool↗

Cor triatriatum.

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Cor Triatriatum↗

Double outlet right ventricle and left-sided aorta. A previously undescribed association with cor triatriatum and double right ventricular chamber.

Double-outlet right ventricle with viscero-atrial situs solitus, right-sided right ventricle and left-sided aorta is an unusual finding. In the present case it was found in association with a cor triatriatum and a double right ventricular chamber, a condition which has not been described previously. Dextrocardia, subaortic ventricular septal defect, atrial septal defect ostium secundum type and absence of pulmonary and aortic stenosis at any level were also noted.

Aorta↗