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Background: Cor triatriatum sinister (CTS) is an uncommon congenital cardiac anomaly. Atrial fibrillation (AF) is commonly the initial symptom in patients with CTS, occurring in approximately 32% of the cases. The complexity of performing AF catheter ablation, particularly in cases with persistent AF, increases in patients with CTS due to its unique structural challenges., Case Presentation: We report the treatment course of a 60-year-old male patient diagnosed with CTS, who underwent catheter ablation of drug-refractory, persistent AF. The complex anatomical structure of the condition made catheter ablation of AF challenging. To navigate these challenges, we performed comprehensive assessments using transthoracic echocardiography and transesophageal echocardiography, along with cardiac computed tomography angiography, prior to treatment initiation. The intricate anatomy of CTS was further clarified during the procedure via intracardiac echocardiography (ICE). Additionally, the complexity of catheter manipulation was further reduced with the aid of the VIZIGO sheath and the vein of Marshall ethanol infusion to achieve effective mitral isthmus blockage, thereby circumventing the impact of the CTS membrane., Conclusions: This case underscores the complexity and potential of advanced ablation techniques in managing cardiac arrhythmias associated with unusual cardiac anatomies. During the procedure, ICE facilitated detailed modeling of the left atrium, including the membranous structure and its openings, thus providing a clearer understanding of CTS. It is noteworthy that the membrane within the CTS may serve as a potential substrate for arrhythmias, which warrants further validation through larger sample studies., (© 2024. The Author(s).)

Background: Cor triatriatum dexter (CTD) is an extremely rare pathology, with an incidence of < 0.4%. Its main characteristic is a partitioning of the right atrium by the persistence of the embryonic valve of the right sinus venosus., Clinical Case: In this report, we describe the case of a 7-day-old newborn who presented with persistent cyanosis associated with feeding and crying. The diagnosis of CTD was made after an echocardiogram and confirmed using cardiac magnetic resonance imaging. The patient underwent successful surgery on day 14 with a favorable outcome and without complications., Conclusion: The importance of our case lies in the identification of rare heart disease as a cause of cyanosis and desaturation in a neonatal patient in the first days of life who did not present signs of heart failure and whose condition improved with supplemental oxygen. We also demonstrate that early diagnosis with echocardiography and surgical resolution resulted in clear clinical improvement and avoided future complications., (Copyright: © 2024 Permanyer.)

Cor triatriatum dexter is a rare congenital heart defect with a varied clinical presentation ranging from asymptomatic to right heart failure. Accurate diagnosis is imperative as it may affect clinical decision making. We present a multimodality imaging assessment of cor triatriatum dexter in a 70-year-old woman with severe tricuspid regurgitation., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2022 The Author(s).)

Surgical treatment was very urgently carried out in an extremely cyanotic newborn with common pulmonary venous atresia. The diagnosis was confirmed during the procedure, and the common venous chamber was anastomosed to the left atrium through a transverse incision onto the right and the left atria. Unfortunately, pulmonary hypertension persisted during the postoperative course and even got worse gradually. Computed tomography eventually illustrated residual cor triatriatum. The initial atriotomy had been entirely above the diaphragm within the left atrium. Consequently, the abnormal structure had been overlooked. The patient is doing well 1 month after the surgical revision. Pulmonary hypertension disappeared., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Cor triatriatum sinister (CTS), or divided left atrium, is a rare congenital cardiac disease in which the left atrium is divided into 2 chambers by a fibromuscular diaphragm that will cause blood flow obstruction to the left ventricle. Recent animal studies suggested the role of hyaluronidase-2 (HYAL-2) deficiency as a risk factor for developing CTS. The histopathologic features of this diaphragm and our surgical experience with the management of this disease are reviewed., Methods: Ten patients underwent surgical correction of CTS between 2010 and 2018. All patients had complete clinical and imaging evaluation. The fibromuscular diaphragms were histologically evaluated with myosin, troponin, vimentin, smooth muscle actin, and HYAL-2 to characterize the structure of the CTS diaphragm., Results: All patients underwent excision of CTS diaphragm using cardiopulmonary bypass with no early mortality. Most patients had the classic form of CTS in which the diaphragm separates the pulmonary and the vestibular chambers with no atrial septal defect. The histologic studies demonstrated the presence of fibrous, mesenchymal cells, along with cardiac muscle cells, at the site of membrane attachments. HYAL-2 enzyme was expressed in the CTS diaphragm., Conclusions: Surgical repair of CTS provides satisfactory results with low risk of death. Our histologic studies revealed the cellular composition of the CTS diaphragm. HYAL-2 deficiency may not explain the pathogenesis of CTS, and further studies are needed to evaluate the complex mechanisms involved in the development of this disease., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Cor triatriatum is a rare congenital cardiac anomaly. Majority of the cases present in the childhood with an incidence of 0.4%. However, we report a case of cor triatrium sinister with ostium secundum atrial septal defect (ASD) in a 39-year-old female. The intraoperative 3D transesophageal echocardiography (TEE) offers an advantage over 2D TEE in visualizing the interatrial septum and the attachments of the fibromuscular accessory membrane in the left atrium (LA), which could help in surgical decision-making in this patient., Competing Interests: None

Objective: To describe outcomes in patients with cor triatriatum sinister (CTS)., Design: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization., Results: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of 57 (58%) patients had additional congenital heart disease (CHD) diagnosis. A total of 27 (47%) patients required surgical resection of CTS membrane during median follow-up of 76 months, and these patients were younger at the time of CTS diagnosis (26 vs 41, P = 0.01) and more likely to have associated CHD (55% vs 45%, P = 0.02). There was one perioperative mortality and no late mortality. There was no recurrence of CTS membrane obstruction in the patients that underwent surgery. Similarly there was no significant increase in CTS membrane gradient in the patients that were managed conservatively., Conclusions: The natural history of CTS is stability without progressive left atrial obstruction, especially in patients with isolated CTS and in those with initial CTS diagnosis made in adulthood. In patients requiring surgical membrane resection due to flow obstruction, surgery is safe and effective with very low risk of recurrence., (© 2018 Wiley Periodicals, Inc.)

Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium. We describe the first case of acquired cor triatriatum late after the Fontan procedure with successful surgical resection in a child., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Cor triatriatum dexter (CTD) is a rare congenital cardiac malformation with various manifestations and has been sporadically described in dogs. Clinically the dogs present with nonspecific signs of right heart failure or Budd-Chiari-like syndrome. Other associated concurrent cardiovascular anomalies are commonly reported. Diagnosis and full characterization of this complex malformation requires careful investigation and often a multimodal imaging approach., Case Presentation: A 10-week-old, male intact, Golden Retriever was presented with clinical signs of stunted growth, anorexia, and progressive ascites. CTD imperforate with sole separation of the caudal vena cava (CdVC) and concurrent venous wall mineralization was conjointly diagnosed and fully characterized by echocardiography, non-selective angiography, computed tomography angiography and cardiac magnetic resonance imaging (MRI). This was successfully treated surgically and the dog returned to normal activity., Conclusion: To the author's knowledge, this is the first case of CTD imperforate separating the CdVC from the right atrium (RA) with presumed secondary CdCV wall and hepatic parenchyma mineralization reported in a dog. CTD is an important and potentially correctable cause for the development of ascites in a young puppy. Accurate diagnosis of this complex cardiac anomaly is important for selection of the most appropriate curative treatment option.

Objectives: Cor triatriatum dexter (CTD) is a congenital anomaly in which the right atrium is divided into two parts by a membrane or fibromuscular band. Incomplete separation of the right atrium may occur when prominent venous valve remnants such as Eustachian valve (EV) or Chiari network (CN) incompletely divided the right atrium (incomplete CTD-iCTD). We sought to assess the incidence of EV/CN and iCTD and its clinical and technical implications in patients submitted to interatrial shunt transcatheter closure., Design: Retrospective analysis of single center registry., Setting: Secondary referral center., Patients: Five hundred eighty consecutive patients (mean age 44 ± 15.5 years, 385 females) who had been submitted over a 12 years period to intracardiac echocardiography-aided interatrial shunt catheter-based closure., Outcomes Measures: Prevalence of iCTD and EV/CN, shunt grade, right ventricle diameter, incidence of intraprocedural complications., Results: In patients with PFO, a prominent EV or a large CN and iCTD have been diagnosed in was diagnosed on ICE in 51.1% and 5.2%, respectively. In ASD patients, a prominent EV or a large CN and iCTD were apparent in 13.7% and 5.6%, respectively. PFO patients with iCTD had more frequently a curtain pattern on TC Doppler and a larger right-to-left shunt graded than prominent EV/CN patients and patients without. ASD patients with iCTD had larger right ventricle diameter than both ASD patients with EV/CN and patients without. iCTD was associated with 45.1% of patients with intraoperative complications., Conclusions: iCTD are not so infrequently observed by ICE during interatrial shunt closure procedure. Presence of this peculiar structure should be taken in account during device-based procedure in the right atrium., (© 2016 Wiley Periodicals, Inc.)

This article describes the first patient treated surgically for cor triatriatum, and also describes preoperative testing, operative findings, and procedures, as well as follow-up., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Cor triatriatum is a rare congenital cardiac abnormality, consisting of an obstructing membrane between the pulmonary veins and the mitral valve in varying patterns. The entitiy can mimick the pathophysiology of mitral stenosis, necessitating surgical resection. Occasionally, percutaneous balloon dilatation of the membrane has been successfully performed., Case Presentation: We report two cases with cor triatriatum where intraoperative balloon dilatation of the membrane was attempted followed by surgical resection, to explore the feasibility of cathether-based interventional strategies for cor triatriatum., Conclusions: Various anatomical variations exist of cor triatriatum, depending on the drainage of the pulmonary veins and the drainage of the proximal chamber in the right or left atrium. Only isolated forms of cor triatriatum where all pulmonary veins ultimately drain into the left atrium can be recommended for percutaneous strategies. In addition, several anatomical characteristics should be considered to predict technical success of cathether-based interventional strategies, such as the location of the membrane, the degree of pulmonary vein stenosis, the extent of calcification, and the presence of other (congenital) cardiovascular abnormalities. Furthermore, long-term efficacy of these strategies remains to be confirmed. As such, surgical treatment of cor triatriatum remains the mainstay of treatment in adult patients, especially when other cardiovascular anomalies are present which require surgical correction.

Objectives: Cor triatriatum sinister (CTS) is a rare developmental cardiac abnormality resulting in left ventricular inflow obstruction. In this report, we aimed to present our mid-term results of early childhood patients operated for CTS and associated cardiac abnormalities., Methods: We enrolled 15 patients with CTS who were operated by a single surgeon between 2001 and 2013. A retrospective analysis was performed in order to determine the demographics, operative and postoperative results of the patients. The median age was 14 months and the median body weight was 8.2 kg at the time of operation., Results: Fourteen patients had concomitant cardiac pathology. Three of the patients had atrial septal defect and 1 of the patients had partial abnormal pulmonary venous connection, whereas 4 of the patients had both. In 2 cases of complete atrioventricular septal defect, 1 case with ventricular septal defect, 1 case with patent ductus arteriosus, 1 case with double outlet right ventricle and another case with tetralogy of Fallot, complete repair was performed together with membrane resection in the left atrium (LA). The mean preoperative left atrial gradient was 13.3 mmHg, whereas the mean LA pressure at the first postoperative year was 4.2 mmHg. There was 1 case with early mortality due to septic multiorgan failure secondary to pneumonia., Conclusions: CTS is a rare congenital cardiac anomaly in which the results of the corrective surgery are encouraging. Early and long-term outcomes may be variable according to the associated congenital heart defects., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Remnants of the right valve of the sinus venosus, which fail to regress may present as an obstructing membrane within the right atrium as a very rare congenital anomaly. When associated with an atrial septal defect, primary cyanosis may occur due to a large right-to-left shunt. We report on a 1-week old girl with a pseudo-cor triatriatum dexter and immediate cyanosis after an uncomplicated birth. She was successfully treated with surgical excision of the obstructing membrane.

Background: Cor triatriatum is a rare congenital cardiac defect in which the atrium is divided into 2 chambers by a membrane causing obstruction to the blood flow in either the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dexter) eventually leading to cardiac failure. We sought to review our surgical experience with cor triatriatum sinister., Methods: Twenty-five patients underwent surgical correction of cor triatriatum between May 1960 and September 2012. There were 11 males and 14 females with a mean age of 27.4 years (age range, 1 day to 73 years)., Results: All patients underwent excision of cor triatriatum membrane using cardiopulmonary bypass. Twenty patients (80%) required concomitant cardiac surgical procedures. There was no early mortality. None of the patients had any residual atrial obstruction. Two infants who had concomitant repair of complex congenital anomalies died at 2 and 5 months postoperatively after discharge from hospital. Kaplan-Meier survival at 10 years was 83%. All patients were in New York Heart Association class I or II at a mean follow-up of 12.8 years (maximum 44 years)., Conclusions: Surgical repair of cor triatriatum provides satisfactory early and long-term survival with low risk for additional intervention. Cor triatriatum with complex congenital anomalies may be associated with adverse outcome., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Aims and Objectives: Cor triatriatum sinistrum (CTS) and cor triatriatum dextrum (CTD) are rare congenital anomalies characterized by the presence of a perforated septum which divides the respective atrium into a proximal and distal chamber. This report reviews the perioperative course of patients with uncorrected cor triatriatum (CT) undergoing procedures requiring anesthesia. In addition, we performed a literature search that examines the experience of others regarding the peri-operative course of patients with CT., Materials and Methods: A computerized search of a medical record database was conducted to identify patients with a clinical diagnosis of uncorrected CTD and CTS undergoing surgical procedures. Descriptive statistics were used., Results: We identified 12 adult patients with asymptomatic CTS (n = 7) and CTD (n = 5) who underwent 23 anesthetics. There were no perioperative complications which could be attributed directly to the anatomy of CT., Conclusions: Our observation and review of the literature suggest that patients with asymptomatic CT typically tolerate anesthesia and surgical procedures well.

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., (© 2013 Wiley Periodicals, Inc.)

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., (© 2012 Wiley Periodicals, Inc.)

This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy referred for oncologic care. During her evaluation, an abnormal cardiovascular examination resulted in an echocardiographic evaluation and an unanticipated diagnosis of a highly obstructive left atrial cor triatriatum was uncovered. The patient underwent repair of cor triatriatum and lymph node biopsy shortly after the diagnosis was made. The biopsies revealed reactive lymph nodes with lymphatic dilation and no inflammatory or neoplastic features. To our knowledge, this case represents the first pediatric example of extensive mediastinal lymphadenopathy mimicking the appearance of a malignant process as a result of severe pulmonary venous hypertension., (© 2012 Wiley Periodicals, Inc.)

A 14-year-old female with mitral atresia, double outlet right ventricle, l-malposition of the great arteries, pulmonary valvular stenosis, bilateral superior vena cavae, and juxtaposed right atrial appendage had undergone a staged Fontan completion at 22 months of age. Ten years later, transthoracic echocardiography detected blood flow acceleration in the left atrium and scheduled follow-up cardiac catheterization revealed a 3.3 mm Hg pressure gradient between the common pulmonary venous chamber and left atrium, confirming cor triatriatum. An abnormal septum in the left atrium was surgically resected through a left-side right atriotomy. Postoperative echocardiography showed no accelerated blood flow in the left atrium., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Cor triatriatum sinister, a congenital cardiac anomaly involving a fibromuscular membrane that partitions the left atrium into 2 chambers, has been reported in only 0.1% to 0.4% of patients with congenital heart disease. The posterosuperior chamber receives blood from the pulmonary veins, and the anteroinferior chamber contains the left atrial appendage and mitral valve orifice. Most patients are diagnosed with the condition in infancy or childhood; adult cases are rare. We describe a case of cor triatriatum sinister in a 43-year-old man whose only presenting symptom was recurrent syncope. He underwent corrective resection of the membrane and was asymptomatic thereafter. In addition to discussing the patient's case, we review the relevant medical literature.

A 20-month old girl with severe pulmonary hypertension and cardiomegaly was admitted to the paediatric intensive care unit with right ventricular failure of unknown origin. Only after decompression of the heart chambers under extracorporeal membrane oxygenation (ECMO), did the pathognomonic membrane of Cor triatriatum become visible on echocardiography. The patient underwent successful surgical correction and subsequently cardiac function recovered completely. Cor triatriatum remains a rare congenital cardiac disorder with a variable presentation, often including recurrent respiratory infections before right-sided heart failure occurs. This case illustrates that ECMO can serve not only as a bridge to diagnosis, but can also facilitate correct diagnosis. Given the excellent outcome after surgical treatment, it is crucial that cardiologists rule out the possibility of cor triatriatum when assessing a child with unexplained pulmonary hypertension.

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A combination of cor triatriatum and persistent left superior vena cava without communication to the coronary sinus is uncommon. A 62-year-old male with this diagnosis in conjunction with atrial fibrillation underwent successful intracardiac repair done with a unique method. After a maze procedure and enlargement of the route from the pulmonary veins to the mitral valve, a GoreTex graft was used to reroute the left superior vena cava into the right atrium and to close two thirds of the circumference of the patient's atrial septal defect; the rest of the defect was closed with another GoreTex patch., (Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Cor triatriatum dexter is a rare congenital anomaly, caused by the persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers and its diagnosis is rather difficult, due to the features often mimicking Ebstein's anomaly. We describe a case followed from the foetal age for a relative right ventricular hypoplasia and suspicious pulmonary stenosis and diagnosed after birth to have cor triatriatum dexter with an obstructing membrane in the right atrium, needing surgical correction, done successfully.

Cor triatriatum is a rare congenital cardiac malformation and it usually refers to the left atrium. We report an unusual case of cor triatriatum in a 33 - year old woman presented with congestive heart failure caused by left ventricular systolic dysfunction.

Introduction: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients., Objective: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome., Methods: Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed., Results: Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS., Conclusions: Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.