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Cor Triatriatum Sinister (CTS) is a rare congenital anomaly with an estimated incidence of 0.4%, resulting from abnormal left atrial septation. It may present in isolation or in association with other heart defects. High percentage of patients require surgery with low mortality and recurrence rates. CTS has been classified in the past however we aim to describe a case series with more comprehensive and inclusive classification.This was a single-center retrospective cohort study of 16 children with the diagnosis of CTS between 2000 and 2020. Medical records were reviewed for clinical presentations, hospital, and postoperative courses.Sixteen patients (63% female), with a median age at diagnosis of 4.3 months, five (31%) were neonates. Six (38%) had isolated CTS, two (13%) with functional single ventricle (SV), and the remaining eight patients (50%) had other associated heart defects (septal defects in three, coarctation of the aorta in another three, and anomalous pulmonary venous connections in three). Eight patients (50%) presented with obstructed CTS. Twelve patients (75%) underwent surgical intervention. Mortality occurred in three patients (19%) with two surgical (one with total anomalous pulmonary venous connection and another with SV) and one nonsurgical (septal defect with Fanconi anemia). The surgical median follow up was 4.7 years. Recurrence of the membrane occurred in two patients (17%).This study showed good long-term outcomes for patients with isolated and complex CTS. Complete and proper classification of CTS ensures appropriate diagnosis, surgical planning, and better family counseling which may correlate with outcomes.

Cor triatriatum is a rare congenital heart disease. A 57-year-old woman had cor triatriatum with severe mitral valve regurgitation (MR) and atrial fibrillation (AF). We performed mitral valve repair, left atrial appendage resection, and maze procedure by resection of the anomalous septum in the left atrium. As a result, MR was controllable and AF disappeared after the operation. Although there is no established maze procedure with cor triatriatum, removing the septum was effective to complete it.

Cor triatriatum is a rare congenital heart defect that is associated with an increased risk for developing atrial fibrillation. We report a case of a healthy 38-year-old man who presented in decompensated heart failure and atrial fibrillation with a rapid ventricular response. A transthoracic echocardiogram (TTE) demonstrated severe biventricular dysfunction and dilatation in addition to cor triatriatum sinister. He was diuresed with resolution of his symptoms and spontaneously converted back to sinus rhythm. There is limited evidence in the literature surrounding anticoagulation and associated left ventricular dysfunction in the setting of cor triatriatum which posed difficult therapeutic decisions.

There are multiple cardiac etiologies for wheezing and respiratory distress which require a high degree of suspicion for the pediatrician to diagnose. We present a case of a patient with a history of long-standing mild persistent asthma with minimal improvement on controller and bronchodilator therapies who presented to the emergency room with acute respiratory distress. When he demonstrated a lack of improvement with traditional respiratory therapies, additional etiologies of respiratory distress were considered. Ultimately an echocardiogram was performed, which revealed the diagnosis of cor triatriatum. He underwent surgical resection of his accessory membrane and has had no additional symptoms of asthma since repair.

Background: Adults with congenital heart disease (CHD) and coronary artery disease (CAD) have unique clinical manifestation due to the coexistence of intracardiac anomalies and CAD. Case reports are rare in surgical management of CHD combined with CAD. Our goal is to study the outcome of surgical intervention of CHD and CAD concomitantly. Methods: From February 2002 to August 2009, 29 adult patients underwent coronary artery bypass grafting (CABG) and surgical correction of CHD concomitantly. Congenital cardiac anomalies include atrial septal defect (ASD) in 21 cases, ventricular septal defect in four cases, atrioventricular septal defect in three cases, and cor triatriatum in one case. Coronary angiography demonstrated: one-vessel disease in 10 cases, two-vessel disease in 11 cases, and three-vessel disease in eight cases. Coronary revascularization and intracardiac anomalies were corrected with cardiopulmonary bypass in 23 cases. There were six patients who had off-pump coronary artery pass grafting (OPCAB) and intraoperative device closure of ASD. Results: One patient died of pulmonary infection and multiorgan failure. Follow-up time was from 2 to 89 months (mean, 42 ± 25 months). One patient with recurrent angina did not need intervention of the revascularization. Six patients who acquired OPCAB and intraoperative device closure of ASD had no complications after surgery. Conclusions: Surgery for adult patients who had CHD with CAD was a safe and effective management. OPCAB with intraoperative device closure of ASD was a reasonable approach for some selective patients. (J Card Surg 2010;25:629-632)

We report a female patient with asymptomatic cor triatriatum sinister, associated with 4q34.3 deletion. Her child, carrying the same imbalance, suffers from tetralogy of Fallot. To the best of our knowledge, this is the first reported case of cor triatriatum associated with deletion of the long arm of the chromosome 4; furthermore, the majority of patients with chromosome 4 long arm syndrome have de novo deletions and only few familial cases have been reported so far.

We present a rare case of a patient with a dilated unroofed coronary sinus mimicking cor triatriatum that altered mitral inflow in a cardiac-type total anomalous pulmonary venous connection. The membranous tissue in the left atrium, thought to be cor triatriatum tissue preoperatively, was actually a remnant of a dilated coronary sinus tissue. Although this tissue did not create a significant pressure gradient and merely induced a turbulent mitral inflow, we resected it to avoid future problems with mitral leaflet function.

Cor triatriatum sinister is a rare congenital heart disease and rarely found in adults. The authors describe an asymptomatic 20-year old man presenting with heart murmur by check up. Transthoracic and transesophageal echocardiogram demonstrate atrial septal defect (ASD) secundum 26 mm and cor triatriatum sinister with 20 mm of fenestration. Transcatheter closure of ASD using Occlutech FigullaRdevice was successfully performed without complications. Echocardiogram post procedure demonstrate good position of device without obstruction of blood flow, no residual shunt and residual 12 mm of fenestration of cor triatriatum.

We report a rare case of simultaneous repair for Cor triatriatum (CT), partially unroofed coronary sinus (CS) with persistent left superior vena cava (PLSVC), moderately severe tricuspid regurgitation, and atrial flutter 35 years after radical operation for tetralogy of Fallot (TOF). A 40-year-old woman underwent patch closure of atrial septal defect as to drain blood from left SVC to right atrium (RA), excision of diaphragm in left atrium (LA), tricuspid annuloplasty, and cryoablation. Postoperative course was uneventful with successful anatomical correction.

Introduction. Cor triatriatum is a rare congenital heart disease found incidentally in children. Although cor triatriatum can be an incidental finding in asymptomatic adults; it is extremely rare to find elderly patients without symptoms and is unique in a long distance runner.Case Presentation. We present the case of an 83-year-old long-distance runner with cor triatriatum sinistrum and atrial fibrillation who continues to be asymptomatic and has continued to run long distances, retaining his excellent functional capacity.Conclusion. Cor triatriatum sinistrum is a rare congenital disease, which is often found incidentally in children. Although it is also seen in adults without symptoms at normal exertion, it is rare to have this condition in long-distance runners especially in elderly people with other structural heart disease, that is, atrial fibrillation and mitral regurgitation.