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Cor triatriatum is a rare congenital heart defect in which a thin, fibro-muscular membrane divides the left or right atrium into two chambers resulting in a triatrial heart. Subdivision of the left atrium named cor triatriatum sinister (CTS), is the more common form, whereas the right atrial equivalent called cor triatriatum dexter (CTD) is rarer. They account for up to 0.4% and 0.025% of the burden of congenital heart disease respectively. We present the case of CTD found incidentally with transthoracic echocardiography for a patient who underwent aortic valve replacement for symptomatic bicuspid aortic valve stenosis. [ABSTRACT FROM AUTHOR]

Cor triatriatum sinister an interesting and relatively rare congenital condition in which the left atrium is bisected by a fibromuscular membrane into 2 distinct chambers. Classically, patients are diagnosed at an early age, although in some cases they remain asymptomatic until adulthood and this is mainly due to differences in the degree of obstruction to pulmonary venous return and the presence of associated lesions. However, some patients may remain asymptomatic into adolescence or late adulthood due to incomplete membranes or other favorable circumstances. Although there are only a few cases where cardiac MRI has been used to diagnose cor triatriatum sinister, it is very suitable for making the diagnosis. important to carefully evaluate cases of cor triatriatum sinister, as it resembles the physiology of mitral stenosis. Such cases in elderly individuals accompanied by mitral and tricuspid regurgitation are very rare. We want to present a case of a diagnosed cor triatriatum with mitral and tricuspid regurgitation in a 73-year-old male, who was hospitalized due to signs of heart failure. The diagnosis was made using MRI.

Two patients with hypoplastic left heart syndrome (HLHS) concomitant with cor triatriatum underwent the staged Norwood procedure following hybrid palliation. Cor triatriatum was diagnosed after birth in both cases. Case 1 with aortic stenosis and mitral atresia underwent bilateral pulmonary artery banding at 2 days of age. Subsequently, balloon atrial septostomy was conducted at 21 days of age due to hypoxia and increased intra-atrial pressure gradient. At 2 months of age, concomitant Norwood operation, cor triatriatum repair, and tricuspid valve repair were successfully performed. She has suffered from branch pulmonary artery hypoplasia since then; thus, Fontan operation was out of indication. Case 2 with aortic stenosis and mitral stenosis underwent bilateral pulmonary artery banding at 6 days of age. After that, the patient's hemodynamic condition remained stable. The Norwood operation concomitant with cor triatriatum repair was performed at 2 months of age. However, during the procedure, massive pulmonary edema and hemorrhage occurred right after the initiation of cardiopulmonary bypass. She could not be weaned from cardiopulmonary bypass. [ABSTRACT FROM AUTHOR]

Cor triatriatum is a rare congenital heart defect that occurs when a fibromuscular membrane divides the atrium into two chambers, which may impair blood flow to the ventricle. When it does, the symptoms usually manifest during infancy or early childhood. In this E-challenge, though, the case of a 40-year-old man is reviewed whose symptoms of shortness of breath progressed over the years and were attributed to the diminished mitral valve inflow due to the restricted cor triatriatum sinister associated with pulmonary hypertension, tachycardia-bradycardia syndrome, and atrial fibrillation. Despite routine preoperative evaluation, intraoperative transesophageal echocardiography was used to more accurately evaluate cor triatriatum sinister's morphology, hemodynamic significance, and associated anomalies. [ABSTRACT FROM AUTHOR]

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cor triatriatum is a rare yet important anomaly. We want to highlight the importance of ICE for realtime assessment of the cardiac anatomy during interventional electrophysiology procedures especially when ancillary imaging modalities have failed to detect abnormalities. In these cases, ICE with color flow Doppler imaging guidance was essential for prompt diagnosis of cor triatriatum including definition of the membrane structure with fenestrations or identifying modest atrial obstruction, and guiding proper and uneventful catheter manipulation around the membrane, especially when manipulating near the pulmonary vein orifices. The development of symptoms in patients with cor triatriatum may be insidious and may be delayed even until late adult life. In our patients the cor triatriatum was an incidental ICE finding without significant hemodynamic consequences, because of the sizable defect in the membrane allowing communication. The incidence of cor triatriatum in our adult patients population is 0.09% (4 of 4269) using ICE during radio frequency ablation procedures for tachyarrhythmias. [ABSTRACT FROM AUTHOR]

Resumen: Presentamos el caso de un paciente escolar, derivado por la presencia de dolor torácico recurrente, que fue valorado por nuestro servicio donde se diagnosticó cor triatriatum, siendo una enfermedad sumamente rara, con una frecuencia del 0,1 al 0,4% de todas las cardiopatías congénitas. Se caracteriza por una membrana fibromuscular que divide la cámara de la aurícula izquierda en 2: una posterior, proximal a la entrada de las venas pulmonares, y otra anterior, que contiene el apéndice auricular y la válvula mitral. Clínicamente puede presentarse como arritmias cardiacas o como una enfermedad obstructiva grave en el periodo neonatal, dependiendo del grado de integridad de la membrana. En este caso se realizó la corrección quirúrgica del defecto, siendo exitosa. Actualmente el paciente se encuentra asintomático y continúa en seguimiento. Abstract: We present the case of a school patient, referred due to the presence of recurrent chest pain, who was evaluated by our service where cor triatriatum was diagnosed, being an extremely rare pathology with a frequency of 0.1 to 0.4% of all congenital heart diseases, characterized by a fibromuscular membrane that divides the left atrium chamber into 2, one posterior, proximal to the entrance of the pulmonary veins, and the other anterior, which contains the atrial appendage and the mitral valve. Clinically it can present as cardiac arrhythmias or as a severe obstructive pathology in the neonatal period, depending on the degree of integrity of the membrane. In this case, the surgical correction of the defect was performed, being successful. The patient is currently asymptomatic and continues to be monitored.

Objective: to describe the surgical technique for the correction of Cor triatriatum by real photos and video of the intervention. Methods: literature review and illustration with images of the real anatomy of this pathology in an intervention performed on a 68-year-old female patient who consulted for dyspnea and cor triatriatum sinister was documented. Results: Illustrated surgical intervention of cor triatriatum sinister in an adult patient, with recovery of functional class. Conclusions: The objective of the cor triatriatum surgical treatment is to restore a normal cardiac anatomy in addition to correct associated anomalies that are frequently present in this entity. [ABSTRACT FROM AUTHOR]

Atrial fibrillation (AF) is the most common cardiac arrhythmia. However, the relation between congenital heart defects and the predisposition to AF is not fully understood. A 65‐year‐old male was admitted into the emergency department due to progressive dyspnea, orthopnea, palpitations, and edema. Transthoracic echocardiogram showed bi‐atrial enlargement and dysplasia of the mitral leaflets with severe mitral regurgitation. Also, a membrane was noted in the LA, dividing the chamber into two parts, suggestive of cor triatriatum sinister. Coronary computed tomography angiography demonstrated a soft tissue septum in the left atrium. Multimodal evaluation is of vital importance for a complete approach, since, detected in time, it has an excellent prognosis. [ABSTRACT FROM AUTHOR]

Both cor triatriatum (CorT) and partial anomalous pulmonary venous connection (PAPVC) come under the rare spectrum of congenital heart diseases. These entities may coexist in a patient or may appear independent of each other. The diagnosis is established routinely by echocardiography. Here, we describe the case of a 45-year-old female presenting with exertional dyspnea and palpitations. She was diagnosed to have CorT in the preoperative echocardiography; later, intraoperatively, transesophageal echocardiography revealed the additional presence of PAPVC with CorT. This case report highlights the limitations of echocardiographic findings in the patient and the importance of multimodal imaging techniques in such situations. [ABSTRACT FROM AUTHOR]

Cor triatriatum sinister is a rare congenital heart disease in which the left atrium is divided into two compartments by a fibromuscular membrane. In most cases, its symptoms appear in childhood, and it is rarely diagnosed in adulthood. Patients with cor triatriatum sinister are more prone to neurological diseases, such as cerebral infarction. Herein, we report the case of a patient whose cor triatriatum sinister went undiagnosed in the preoperative evaluation, but was diagnosed whilst investigating a cerebral infarction that occurred following a surgery in the beach chair position. It highlights the potential complications in otherwise healthy asymptomatic patients undergoing surgery in the beach chair position. Additionally, in high‐risk patients, the provision of clear communication, in advance, regarding potential complications and their management, may reduce the patient's morbidity. [ABSTRACT FROM AUTHOR]

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac malformation in which the left atrium is divided by a fenestrated membrane, which can restrict blood flow and cause symptoms of congestive heart failure. Rarely, the condition can present in adulthood. This case report illustrates a case of sudden cardiac death (SCD) due to the sequelae of untreated CTS. To date, there are no reported cases of SCD attributable to CTS. Cor triatriatum sinistrum is among the rarest of congenital heart diseases. In this case report, we describe the prevalence, etiology, diagnosis, and management of this disease. [ABSTRACT FROM AUTHOR]

Cor triatriatum is a rare congenital heart disease (0.1% of all congenital cardiac defects). Most patients are identified shortly after birth with the evaluation of a distressed or cyanotic neonate. However, when the presentation is delayed, primary symptoms may mimic reactive airway disease. We present an 11-year-old adolescent male who presented with obstructive sleep apnea-like features initially, on further evaluation showed features of severe pneumonia. There was clinical and radiological dissociation with the findings. [ABSTRACT FROM AUTHOR]

Background: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). Methods: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. Results: The median age and body weight at operation were 9 months (interquartile range [IQR], 3–238 months) and 7.5 kg (IQR, 5.8–49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39–195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6–112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). Conclusion: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

Abstract Background Cor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers. Case presentation Here, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair. Conclusions The association of CTS with DCRV forming a “6-chambered heart” is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair.

This article discusses a case of Cor triatriatum sinistrum (CTS) syndrome, a rare congenital heart disease where a fibrous membrane divides the left atrium into two chambers. The case involves a 19-year-old woman who experienced symptoms of hypoxia in infancy but grew normally into adulthood, with pulmonary arterial hypertension being the only lasting effect. The article explains the haemodynamic adaptation that occurred in the patient, which alleviated her hypoxia symptoms. The patient underwent surgical correction for the syndrome, resulting in normalized haemodynamics and the resolution of symptoms. The article emphasizes the importance of detailed imaging diagnosis and haemodynamic evaluation in understanding the pathophysiological mechanisms of the disease and tailoring personalized treatment strategies for patients. [Extracted from the article]

Cor triatriatum is a rare congenital heart disease (0.1% of all congenital cardiac defects). Most patients are identified shortly after birth with the evaluation of a distressed or cyanotic neonate. However, when the presentation is delayed, primary symptoms may mimic reactive airway disease. We present an 11-year-old adolescent male who presented with obstructive sleep apnea-like features initially, on further evaluation showed features of severe pneumonia. There was clinical and radiological dissociation with the findings.

A woman with recurrent thromboembolic stroke was found to also have cor triatriatum. When the patient first presented with weakness, she was thought to have ischaemic stroke because she had conventional risk factors, but she was later confirmed to have cor triatriatum. The main method of treatment is surgery. However, if surgery is contraindicated, anticoagulation can be used as second-line treatment, but this can be difficult. This report describes the follow-up of a middle-aged female patient with cor triatriatum over 6 years during which she experienced multiple strokes despite different methods of anticoagulation.

Cor triatriatum sinister is a rare congenital anomaly characterized by the left-sided triatrial form of the heart. Diverse theories have been proposed regarding its formation, and the failure of incorporation of the common pulmonary vein into the left atrium (LA) during embryogenesis is the most widely accepted theory. Accordingly, cor triatriatum sinister may be associated with pulmonary venous obstruction and post-capillary pulmonary hypertension in the setting of restricted fenestration. A high proportion of patients with cor triatriatum sinister also have an associated secundum atrial septal defect. Pre-capillary pulmonary hypertension, which is unusual in patients with small atrial septal defects (

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 uncommon pathology which may be latent for a long time, manifesting after some physiological or pathological situations.

Background: Cor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers. Case presentation: Here, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair. Conclusions: The association of CTS with DCRV forming a "6-chambered heart" is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair. [ABSTRACT FROM AUTHOR]

Anomalous pulmonary veins drain into the right side of the left atrium is an uncommon variety of anomalous pulmonary venous return. Rarely, anomalous pulmonary venous drainage combined with cor triatriatum and atrial septal defect. We presented the imaging findings of a male patient who had anomalous pulmonary venous drainage which has not previously been described. [ABSTRACT FROM AUTHOR]

Background and Aim: The morphological variations when one, or both, of the atrial chambers is subdivided, are many and varied. We sought to address clinical presentations, potentially misdiagnosed cases, diagnostic modalities, surgical approaches, and outcomes of this "family" of uncommon lesions. Methods: A total of 193 published investigations were synthesized. Diagnostic information was provided by clinical presentation, and multimodality imaging studies. Results: Almost three‐quarters of patients with divided atrial chambers present during infancy with severe pulmonary hypertension and cardiac failure. Associated cardiac and extra‐cardiac defects are present in between half and nine‐tenths of cases. Acquired division of the left atrium has been reported after the Fontan operation, orthotopic cardiac transplantation, and complicated aortic valvar infective endocarditis. Surgery under cardiopulmonary bypass remains the definitive treatment. Balloon dilation may be considered in anatomically compatible variants in the setting of cardiac failure and pregnancy as a bridge todefinitive treatment. Overall, mortality has been cited between nil to 29%. Presentation during infancy, associated congenital anomalies, pulmonary hypertension, and surgery in the previous era, have been the reported causes of death. The operative survivors have long‐term favourable outcomes, with near normal cardiac dimensions and low risk of recurrence. While asymptomatic patients with division of the right atrium do not need treatment, surgical resection of the dividing partition under cardiopulmonary bypass is recommended in symptomatic patients with complex anatomy, the spinnaker malformation, or associated cardiac anomalies. Balloon dilation may be considered in uncomplicated patients with less obstructive lesions. Hybrid intervention and endoscopic robotic correction also have been performed. Conclusions: Resection of the dividing shelf allows the survivors to regain near normal dimensions with a low risk of recurrence. We submit that an increased appreciation of the anatomic background to division of the atrial chambers will contribute to improved surgical management. [ABSTRACT FROM AUTHOR]

Purpose: Reports concerning clinical characteristics of cor triatriatum and approaches for catheter ablation of complex atrial tachyarrhythmias remain limited. Here, we describe successful catheter ablation treatments for complex atrial tachyarrhythmias in patients with cor triatriatum and address the clinical caveats. Methods: Demographic characteristics, electrophysiologic findings, and ablation results in four patients with cor triatriatum were described. Results: Catheter ablation was performed in four patients with cor triatriatum (three sinister and one dexter) and complex atrial arrhythmias (three with persistent atrial fibrillation (AF) and one with atypical left atrial flutter). A transseptal puncture was selectively directed into the accessory compartment containing the pulmonary veins. A comprehensive preview involving transthoracic echocardiography, transesophageal echocardiography, and computed tomography of the pulmonary veins was critical for proper positioning of ablation catheters. The pulmonary veins remain the major triggers or initiators for AF, and four pulmonary vein isolation procedures were sufficient to achieve successful results with negative inducibility test in the patients with AF. Heterogeneous conduction and complex fractionated signals were observed on the fibromuscular membrane. Atypical flutter was terminated during ablation over the connection between membrane and left atrial roof. The procedure was successfully performed on all patients without complications. No acute recurrences of atrial tachyarrhythmias were observed in any of the patients during short-term follow-up. Conclusions: Catheter ablation is a feasible and efficient therapeutic strategy for treating complex atrial tachyarrhythmias in patients with cor triatriatum. Atrial remodeling due to anatomical obstruction or heterogeneous conduction of the fibromuscular membrane may serve as an arrhythmic substrate. [ABSTRACT FROM AUTHOR]

Resumen: El cor triatriatum es una anomalía congénita rara, que representa el 0,4% de todas las malformaciones cardíacas. En esta malformación la aurícula es separada en dos compartimientos por una membrana fibromuscular, que puede ser enteramente intacta o contener fenestraciones de diámetros variables. La aparición de la sintomatología está en relación con las propiedades obstructivas que mimetizan la estenosis mitral y la presencia de una comunicación interauricular. La ecocardiografía bidimensional y el Doppler color son las modalidades diagnósticas no invasivas de elección para el diagnóstico preciso del cor triatriatum y de su repercusión hemodinámica. Se reporta un caso de cor triatriatum en un paciente adulto mayor, en quien se hizo el diagnóstico tardío y casual de esta malformación. Abstract: Cor triatriatum is a rare congenital anomaly that represents 0.4% of all cardiac malformations. In this malformation the atrium is separated into two compartments by a fibrous-muscular membrane that may be completely intact or contain fenestrations with variable diameters. The appearance of symptoms is associated with obstruction-like properties that mimic mitral stenosis and an atrial septal defect. Two-dimensional cardiac ultrasound and colour Doppler are non-invasive methods of choice for the accurate diagnosis of cor triatriatum and its haemodynamic repercussions. A case of cor triatriatum is reported in an elderly patient, in whom a delayed and casual diagnosis was made of this malformation.

A 10-year-old boy presented with complaints of poor weight gain, progressively increasing dyspnea since early childhood, and orthopnea. Echocardiogram was suggestive of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dextrum) is divided into two compartments by an abnormal fibromuscular septum usually with an opening between the two chambers associated with varying degrees of obstruction of the pulmonary veins. The pulmonary venous return is followed by pulmonary arterial hypertension. Cor triatriatum literally means a heart with three atria (triatrial heart), namely, the superior chamber, the inferior chamber or the true atrium, and the atrial appendage. This anomaly occurs in 0.4% of patients with congenital heart defects, with a slight male predominance. The present case of cor triatriatum sinister is being reported in view of the fact that the child remained undiagnosed till the age of 10 years, despite being “sick since a little boy” requiring multiple hospital admissions for chest infections. He had been receiving treatment for “chest problem” and had been on bronchodilators. The classical echocardiographic findings would be of academic interest as well.

A 39-year-old pregnant patient with acute appendicitis was planned for emergency laparoscopic appendectomy in the second trimester of pregnancy. Preoperative two-dimensional transthoracic echocardiography revealed asymptomatic cor triatriatum sinisiter (CTS), dividing left atrium into two chambers. There was no associated cardiac anomaly, wall motion abnormality, or pulmonary hypertension. We report the case of a pregnant patient with CTS who uneventfully underwent laparoscopic appendectomy without invasive cardiac monitoring using total intravenous anesthesia.

A 35-year-old female presented to our emergency department with clinical signs of acute heart failure. Clinical workup identified severe right heart (RH) dilation and dysfunction with a crossing membrane structure in the right atrium. Right heart catheterization confirmed high output heart failure (HOHF), pulmonary hypertension (PH), and left-to-right blood shunting followed by the documentation of multiple liver and pulmonary arteriovenous malformations (AVMs). Hereditary Hemorrhagic Telangiectasia (HHT) diagnosis was made according to clinical criteria and was established as the cause of HOHF because of chronic volume overload from systemic to pulmonary shunts. With this illustrative case, we aim to discuss the broad spectrum of clinical manifestations of HHT and the unusual phenotype of HOHF secondary to HHT. This case also highlights the broad diagnosis of atrial echocardiographic abnormalities and cardiac structural distortion secondary to high output that can be misleading at imaging evaluation.

A 25-year-old male with no prior medical history presented with a one-month history of nausea, weight loss, and dyspnea that progressed to syncope. The initial echocardiogram showed a dilated right ventricle with signs of systolic failure. The patient was admitted for suspected pulmonary embolism, but chest computed tomography (CT) revealed interstitial pneumonia. A transthoracic echocardiogram on day 6 of admission diagnosed cor triatriatum sinister (CTS), severe pulmonary hypertension, chronic cor pulmonale, and reduced right ventricular function. The patient was managed conservatively in the intensive care unit (ICU) without the need for mechanical ventilation and discharged after clinical improvement. This case highlights the importance of the early diagnosis of rare congenital heart defects such as cor triatriatum sinister, which can present with nonspecific symptoms and rapidly progress to right heart failure., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Domínguez-López et al.)

This article discusses a case of a 38-year-old woman with scimitar syndrome and hemi-cor triatriatum. The patient presented with symptoms of exertional dyspnea and was initially diagnosed with an atrial septal defect. Further examination revealed dextrocardia, cardiomegaly, and other cardiac abnormalities. Imaging tests confirmed the presence of scimitar syndrome, hemi-cor triatriatum, and anomalous pulmonary venous drainage. This case highlights the need for careful evaluation and vigilance when assessing similar cases, as well as the embryological basis of left atrial development and pulmonary venous incorporation. [Extracted from the article]

In a newborn in whom cor triatriatum was missed on echocardiography, infracardiac total anomalous pulmonary venous connection was successfully repaired with the aid of cardiac computed tomography (CT). In rare combinations, as in this case, an accurate diagnosis prior to surgery, which is of vital importance for successful repair, can be made through a high index of suspicion and the use of a supplemental imaging modality such as CT.

Among congenital cardiac defects, cor triatriatum sinistrum is a rare formation of 3 distinct atrial chambers in the heart. Depending on the size of the defect in the membranous septum, the symptoms can range from asymptomatic to severe pulmonary hypertension. Individuals can go years, sometimes to the second and third decades of life, before presenting with symptoms. Although it is well known to be associated with pulmonary veno-occlusive disease, the association with hemolytic anemia is much less well known. Identifying the subtle signs and symptoms of cor triatriatum in the emergency department can save a life. Here, we present the case of a 6-month-old infant with hemolytic anemia in the setting of right-sided congestive heart failure leading to the diagnosis of cor triatriatum. Anemia developed secondary to shearing stress caused by the heart defect itself. Complete resolution of symptoms occurred status post septectomy and repair of the defect. [ABSTRACT FROM AUTHOR]

Background: Cor triatriatum and supramitral ring are congenital anomalies which result in formation of three chambers of atria. To the best of our knowledge, simultaneous presence of both entities in the same patient resulting in the formation of four chambers of atria has not been described in the literature. Here, we report a case of simultaneous presence of cor triatriatum and supramitral ring associated with Raghib syndrome and Eisenmenger syndrome. Case presentation: We report the case of a middle-aged gentleman, who presented to us with features of atrial septal defect with Eisenmenger syndrome. Multimodality imaging confirmed the simultaneous presence of supramital ring and cor triatriatum resulting in "cor tetratriatum" along with Raghib syndrome. Presence of Eisenmenger syndrome compelled us to offer medical therapy for the patient. Conclusion: This is the first case report describing the simultaneous presence of supramitral ring and cor triatriatum resulting in a new entity—"cor tetratriatum". [ABSTRACT FROM AUTHOR]

Copyright of Ümraniye Pediatri Dergisi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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.

Severe cardiomegaly with an atrial septal defect was discovered during necropsy of a subadult White-tailed Eagle (Haliaeetus albicilla) found dead in the wild. A thin membrane composed of fibromuscular tissue separated the left atrium into two chambers, most consistent with that described for cor triatriatum sinister (CTS) in other species. Seventeen months later, necropsy of an adult White-tailed Eagle again revealed CTS. This lesion has not been reported previously in raptors. [ABSTRACT FROM AUTHOR]

We treated a surgical case of a 47-day-old male infant diagnosed with an unusual type of cor triatriatum sinister (CTS) with left anomalous pulmonary venous drainage to the innominate vein via a vertical vein. After preoperative hemodynamic assessment of pulmonary venous (PV) return, this patient underwent a resection of the fibromuscular membrane between the accessory and the true left atrial chambers, concomitant with vertical vein banding to facilitate a left PV return through a common pulmonary venous collector (CPVC). Catheterization three months after this surgery revealed no obstruction of the PV return to the mitral orifice as well as good growth of the CPVC as a left PV return pathway. The patient has been doing well on aspirin. [ABSTRACT FROM AUTHOR]

Copyright of Gaceta Médica de Caracas is the property of Academia Nacional de Medicina and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)