Cases reported "Cor Triatriatum"

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1/114. The diagnosis of cor triatriatum sinistrum in children: a continuing dilemma.

    cor triatriatum (CT) is a rare congenital cardiac anomaly. The salient clinical, roentgenographic, electrocardiographic, echocardiographic, and hemodynamic findings are presented in two asymptomatic children and one with nonspecific dyspnea on exertion. Two male children had a classical form of cor triatriatum with normal physical and inconsequential roentgenographic and electrocardiographic findings. One of the male patients had surgery for a large atrial septal defect ostium secundum (ASD 2 degrees) and pulmonary hypertension in infancy. The female patient had CT with a communicating accessory chamber to right atrium and a rare patent foramen ovale. Her clinical findings confirmed an atrial level shunt. All patients had excision of the fibromuscular membrane from the right and left atrial transseptal approach with excellent results and with no recurrence during 2 to 4 year follow-up. We report the dilemma encountered in the clinical diagnosis of CT in children and the pivotal role played by echocardiography in the diagnosis of this anomaly. ( info)

2/114. 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. ( info)

3/114. 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. ( info)

4/114. ascites and weight loss in a child: due to congenital division of the right atrium.

    Congenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results. ( info)

5/114. 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. ( info)

6/114. Divided right atrium. diagnosis by echocardiography, and considerations on the functional role of the Eustachian valve.

    A child presented at birth with severe cyanosis. echocardiography showed hypoplasia of the right heart with a right-to-left shunt at atrial level. A conservative approach was adopted initially, and the situation improved over a few months, with reversal of the atrial shunt. Surgery was successfully performed at 4 years of age after further echocardiography revealed a congenitally large Eustachian valve and an atrial septal defect. ( info)

7/114. cor triatriatum in adults: three new cases and a brief review.

    We report 3 cases of cor triatriatum that were diagnosed late, in the 4th and 5th decades of life. The presentations of these 2 men and 1 woman varied substantially, both in anatomic and symptomatic aspects. The woman had an associated complex congenital anomaly, which is not uncommon in cases of cor triatriatum. We present our cases, along with a review of this rare congenital cardiac anomaly. ( info)

8/114. Repair of cor triatriatum associated with partially unroofed coronary sinus.

    A young patient with cor triatriatum, secundum atrial septal defect, persistent left superior vena cava, partially unroofed coronary sinus, and moderate tricuspid regurgitation was documented to have both a left-to-right shunt from the common pulmonary venous chamber to the right atrium and a right-to-left shunt from the partially unroofed coronary sinus to the left atrium. Resection of the membrane dividing the left atrium, closure of the atrial septal defect and the partially unroofed coronary sinus with pericardial patches, and a tricuspid annuloplasty resulted in an excellent hemodynamic result. ( info)

9/114. 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. ( info)

10/114. diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal echocardiography and catheterization.

    This is an unusual case of a 37-year-old male whose initial presentation to medical care was for dyspnea. A transthoracic echocardiogram was suspicious for cor triatriatum, which was confirmed by transesophageal echocardiography. Since the resting transmembrane gradient was low, a cardiac catheterization with exercise hemodynamics was performed and demonstrated a marked increase in pulmonary capillary wedge and pulmonary artery pressures. The cor triatriatum was successfully resected at surgery. We have reviewed the English literature and find this to be a unique approach to diagnosis and management. ( info)
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