Cor Triatriatum with Single Atrium Presenting in Adulthood

World journal for pediatric & congenital heart surgery, 2015

Atrioventricular septal defect can present with one or more associated anomalies. Cor triatriatum (subdivided left atrium) and vascular rings are among the less frequent. We describe a two-month-old patient with these three cardiovascular anomalies. This case highlights the importance of exhaustive preoperative evaluation in order to achieve successful surgical correction in one stage.

PubMed, 2011

Cor triloculare biventriculare is a rare congenital malformation of the heart in which there is a complete absence of the atrial septum. It is usually associated with other anomalies like complete atrioventricular canal defect, polysplenic syndrome, isolated dextrocardia, Ellis-van Creveld syndrome, or persistent left superior vena cava. We report a case of a stillborn male foetus of 35 weeks gestation with common atrium, complete atrioventricular canal defect, and persistent left superior vena cava. The possible embryological basis and clinical implication of this variation are discussed.

Congenital Heart Disease, 2012

An eustachian valve (EV) remnant, if present, is usually noted by the presence of a thin ridge or a crescent-shaped fold of endocardium arising from the anterior rim of the inferior vena cava orifice due to the persistence of the right sinus venosus valve. Though the embryologic explanation of cor triatriatum dexter (CTD) is the same as that of the normal formation of the EV-lack of regression of the right sinus venosus valve-it is usually called CTD or divided right atrium when there are attachments on the atrial septum giving the appearance of a divided atrium. However, it's called prominent eustachian valve when the right sinus venosus valve has partly regressed, with no remaining septal attachments and without the appearance of a divided atrium. We present the case of an adult patient with an atrial septal defect with a high insertion of a giant EV, which mimics the echocardiographic appearance of divided right atrium.

ABC Imagem Cardiovascular, 2021

Congenital Heart Disease, 2014

We report a unique case of a 4-year-old boy with intermediate-type atrioventricular septal defect, cor triatriatum sinister, persistent left superior vena cava, unroofed coronary sinus, and absent right superior vena cava. Persistent left vena cava draining into the unroofed coronary sinus was demonstrated easily using the agitated saline-contrast echocardiography. After conformation with angiographic evaluation, surgery was performed at a single session. Roofing of the coronary sinus with polytetrafluoroethylene patch, mitral cleft repair, tricuspid annuloplasty, atrioventricular defect repair with pericardial patch, and resection of the membrane in the left atrium was succeeded without complication.

2021

Background. Cor triatriatum is a rare congenital cardiac anomaly, represent 0.1% of all congenital cardiac malformations and may be associated with other cardiac diseases in as many as 50% of cases. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chamber. Case Presentation. We reported case of cor triatriatum in a 12 years old girl with chief complaint of shortness of breath, middle chest discomfort and palpitation since 5 days prior admission. The diagnosis was based on clinical features, chest radiography, electrocardiography and transthoracic echocardiography. Chest radiograph showed rounded cardiac apex and double contour appearance. ECG showed sinus rhythm, 75 beat per minute, RAD, CCWR, RVH, RV strain pattern with ST depression and T-wave inversion in II, III, aVF, V1-V5. TTE revealed 2 chambers of left atrium, with restrictive supramitral membrane, dilated right atrium, right ventricle and left atrium, sma...

Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2014

Cor triatriatum dexter is a rare congenital heart anomaly in which a membrane divides the right atrium into 2 chambers. We report the case of a 43-year-old woman who had cor triatriatum dexter and a large atrial septal defect. During attempted percutaneous closure, the balloon disrupted the membrane and revealed that the defect had no inferior rim, precluding secure placement of an Amplatzer Septal Occluder. Surgical treatment subsequently proved to be successful. In patients with an incomplete membrane and a septal defect with well-defined rims, percutaneous treatment can be the first choice. In patients who have cor triatriatum dexter and unfavorable anatomic features or concomitant complex heart anomalies, open-heart surgery remains the gold standard for treatment.

World journal for pediatric & congenital heart surgery, 2013

Congenital division of the left atrium (cor triatriatum) and congenitally corrected transposition of the great arteries are both rare congenital cardiac malformations; their coexistence is exceedingly rare with only two previous reports identified in the literature. This combination of lesions is characterized by a pressure-loaded morphologically left ventricle and a propensity for pulmonary edema dependent on the degree of pulmonary venous obstruction caused by the dividing left atrial shelf. The probable impact of this on the natural history and surgical decision making is discussed.

F1000Research, 2022

Cor triatriatum is an extremely rare cardiac congenital malformation characterized by subdivision of the left atrium into two chambers. This division is due to the presence of an abnormal fibromuscular membrane interrupted by one or more openings that communicate the two chambers. The size and number of those orifices is variable, affecting the age of onset of symptoms and their severity. Our case describes the fortuitous discovery of this pathology in a young adult in her forties, following the finding of atrial fibrillation during a preoperative assessment of a strangulated hernia. The cardiac ultrasound allowed in our case to invoke the diagnosis, confirmed thereafter with an angioscanner. Surgical resection was not required in our patient who was put on medical treatment involving a beta-blocker to reduce atrial fibrillation and an anticoagulant treatment to decrease the thromboembolic risk, with a good clinical evolution. It seems therefore interesting to consider this uncomm...

The Annals of Thoracic Surgery, 1990

Twelve patients with cor triatriatum have been seen at our institution since 1979. The clinical presentation, diagnostic evaluation, and surgical results are outlined in this retrospective review. Operation is the treatment of choice for this rare congenital cardiac defect. One patient died 1 day before scheduled operation, and 2 patients died postoperatively, yielding a surgical mortality rate of 17% and an overall mortality rate of 25%. Resection of the obstructing atrial membrane was performed using hypothermic cardiopulmonary bypass in all cases. Left atriotomy was performed in 6 patients, and right atriot-omy was performed in 7. The two postoperative deaths occurred in patients who had serious associated cardiac defects. Associated anomalies include atrial septal defect, persistent left superior vena cava, and partial anomalous pulmonary venous return. The postoperative course has been excellent in all 9 surviving patients; all remain asymptomatic. Cor triatriatum is amenable to surgical repair with excellent results when diagnosed early and when not complicated by other severe cardiac anomalies.