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Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cor triatriatum using ventricular septal defect translocation.

A 17-month-old girl underwent successful single-stage anatomic repair using a technique of ventricular septal defect translocation for complete atrioventricular canal, double-outlet right ventricle, pulmonary stenosis, cor triatriatum, and left superior vena cava to an unroofed coronary sinus. We describe the repair and present clinical and angiographic data from 9 years follow-up.

Cardiac Surgical Procedures↗

Postsurgical use of amplatzer septal occluder in cyanotic patients with pulmonary atresia/intact ventricular septum: significance of cor triatriatum dexter and dilated right atrium.

Percutaneous closure of secundum atrial septal defects (ASDs) has been shown to be safe and effective. However, its role after surgery in patients with cyanotic congenital heart disease who may have associated cor triatriatum dexter and a dilated right atrium has not been established. This article reports on successful closure in such patients, including precautions and results.

Child, Preschool↗

Double outlet right ventricle associated with cor triatriatum sinistrum.

Echocardiographic examination of a newborn infant showed a double outlet right ventricle with subpulmonary ventricular septal defect. The left atrium was divided by a membrane-like structure into a proximal chamber receiving pulmonary veins and a distal compartment containing left atrial appendage. To our knowledge, this is the first report of cor triatriatum sinistrum associated with double outlet right ventricle.

Aortic Coarctation↗

Cor triatriatum sinister: assessment by live/real time three-dimensional transthoracic echocardiography.

We report a 49-year-old morbidly obese female with a poor acoustic window in whom live/real time three-dimensional transthoracic echocardiography was able to make a confident diagnosis of cor triatriatum sinister. En face views of the membrane facilitated accurate assessment of the size and shape of the large nonobstructing opening in the membrane. Maximum dimensions of the opening were 3.06 x 1.03 cm and area was 2.3 cm(2).

Computer Systems↗

Left atrial calcification in a hemodialysis patient with cor triatriatum.

Myocardial calcification is a rare manifestation of abnormal calcium metabolism seen in some patients with chronic renal failure. This report describes the transesophageal echocardiographic and spiral computed tomography (CT) findings in a young hemodialysis female with severe secondary hyperparathyroidism. These findings included calcification of the multiperforated membrane of a cor triatriatum and the wall of the left atrium.

Adult↗

Management of cor triatriatum dexter by balloon dilatation in three dogs.

Two dogs, one immature and one adult, were presented with a history of progressive ascites. In a third, immature dog, increasing exercise intolerance had been noted. Echocardiography demonstrated a partition in the right atrium (cor triatriatum dexter) and echocontrast studies documented normal flow from the cranial vena cava into the right atrium and ventricle. A saphenous vein contrast study demonstrated flow from the caudal vena cava into an accessory right atrial chamber (sinus venarum). The sinus venarum communicated with the true right atrium via a small defect in the atrial membrane in one dog, and additionally with the left atrium via a right-to-left shunting foramen ovale in the other dogs. All defects were visualised on angiographic studies by selective catheterisation of the caudal vena cava via the femoral vein. Balloon dilatation of the defect was then performed using a small followed by a larger balloon angioplasty catheter to enlarge the defect in the atrial membrane. Clinical signs improved within days and were sustained in the long-term in all cases.

Animals↗

Successful management of cor triatriatum associated with anomalous pulmonary/systemic venous connection in an infant.

Diagnosis and successful surgical repair of cor triatriatum, associated with anomalous pulmonary/systemic venous connection in a 6-month-old infant, is described. Follow-up at 2 years revealed no abnormalities. The clinical findings, hemodynamics, and surgical treatment are discussed, together with a review of the literature. This is a rare, life-threatening situation presenting significant diagnostic difficulties in infancy. It is, however, amenable to surgery provided that an early diagnosis is achieved.

Arteriovenous Malformations↗

Cor triatriatum in an adult with mitral regurgitation and massive left atrial enlargement.

An unusual case of cor triatriatum in a 52-year-old woman is described in which the preoperative diagnosis was obscured by the presence of mitral valvular regurgitation and massive left atrial enlargement; such massive left atrial enlargement has not been reported before in this entity. Only the right pulmonary veins drained into the accessory chamber. The abnormal septum dividing the left atrium was demonstrated by two-dimensional but not M-mode echocardiography.

Cardiomegaly↗

Cor triatriatum sinistrum, aortic coarctation and bicuspid aortic stenosis in an adult.

Cardiac anomalies are usually diagnosed early in life, which is particularly true for their various combinations. The diagnosis in adulthood is rare. Here we report the case of a young man with an aortic coarctation corrected at the age of 16, however the associated stenotic bicuspid aortic valve and cor triatriatum sinistrum were corrected after Streptococcus viridans endocarditis 7 years later.

Abnormalities, Multiple↗

[Cor triatriatum--a rare cause of pulmonary hemosiderosis].

Pneumological examinations including open lung biopsy performed on a male patient of 30 years of age suffering from severe respiratory distress that disabled him, as well as from massive recurring attacks of hemoptysis, resulted in suspicion of idiopathic pulmonary hemosiderosis (also known as Ceelen-Gellerstedt's syndrome). Diagnosis of cor triatriatum followed by surgery was arrived at only after a pulmonary oedema had developed and after other rare cardiac diseases had been considered. This rare congenital malformation--which occasionally becomes clinically manifest only in the adult--should be suspected in differential diagnosis of respiratory distress and a sometimes also life-threatening hemoptysis. Echocardiography is the diagnostic method of choice in this regard.

Adult↗

Doppler-ultrasonographic detection of retrograde pulsatile flow in the caudal vena cava of a puppy with cor triatriatum dexter.

A three-month-old puppy had ascites, but its heart was normal by auscultation. Abdominal ultrasonography revealed an enlarged liver, distended hepatic veins and a distended caudal vena cava. Doppler ultrasonography detected retrograde flow in the caudal vena cava and abnormally pulsatile flow in the hepatic veins and caudal vena cava. A non-selective venogram was used to detect the path of the blood from the caudal vena cava. A postmortem examination showed that the puppy had cor triatriatum dexter and a defect in the atrial septum.

Animals↗

Left-ventricular inflow obstruction due to a dilated coronary sinus mimicking Cor Triatriatum.

Persistence of the left superior vena cava with drainage to the coronary sinus is a common congenital anomaly. We report an infant with such a malformation associated with marked enlargement of the coronary sinus, which produced partial supramitral obstruction and consequently impairment to the left-ventricular inflow. The patient pre-sented with cardiac failure in infancy and features mimicking cor triatriatum. Surgical relief of the supramitral obstruction resulted in immediate reversal of the pulmonary hypertension, with clinical improvement. This rare entity, only once previously reported, is an unusual cause of pulmonary hypertension in infancy.

Cardiac Catheterization↗

Cor triatriatum sinister in a cat.

A heart murmur was detected in a five-month-old Persian chinchilla cat. The animal was referred to Nihon University Animal Medical Center where radiographic examination revealed an enlarged heart with dilation of pulmonary arteries and veins and pulmonary oedema. Echocardiography revealed partition of the left atrium, with turbulent blood flow within the left atrium. The patient was placed under continuous care at the facility, but died at seven months of age. Postmortem examination revealed that the left atrium was partitioned by a fibromuscular septum, with a 1 mm diameter perforation, consistent with a diagnosis of cor triatriatum sinister.

Animals↗

Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale.

Sinus venosus atrial septal defect (SVD) is a rare cardiac abnormality in adults. Particularly, the inferior type is difficult to depict by transthoracic echocardiography because of its infero-posterior location to the fossa ovalis. We describe the case of a 33-year-old woman whose chest X-ray taken during bronchopneumonia revealed a cardiomegaly. Further echocardiographic investigations showed an underlying inferior SVD, an incomplete cor triatriatum dexter and a large patent foramen ovale (PFO). The diagnosis was confirmed by cardiac magnetic resonance imaging (CMR) and during surgical repair.

Adult↗

[Cor triatriatum of adults. Apropos of 2 new surgically treated cases in adults].

The authors report two cases of cor triatriatum in a 54 year old woman undergoing open heart surgery with a preoperative diagnosis of mitral stenosis, and a 24 year old woman in whom the diagnosis had been made before surgery. Resection of the abnormal intra-left atrial fibrous membrane successfully restored normal haemodynamics in both cases. The main clinical and diagnostic features of the condition are described.

Adult↗

Wheezing as the sole clinical manifestation of cor triatriatum.

Cardiac malformations involving low-pressure chambers (i.e., either of the atria) are more often diagnosed later in life than lesions that involve high-pressure systems such as ventricular septal defects or persistent ducti arteriosi. Patients with congenital heart disease involving the atria may present only symptoms suggesting lung disease. We report on a child with recurrent episodes of wheezing, which did not respond to albuterol nebulizations and intravenous corticosteroids; he was subsequently found to have cor triatriatum. When a patient suffers from recurrent episodes of lower pulmonary infection and wheezing, despite appropriate management for asthma, less common (including cardiac) causes should be considered.

Cor Triatriatum↗