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A four-year-old spayed female Shiba Inu dog weighing 6.1 kg presented with ascites. Cor triatriatum dexter (CTD) without any other concurrent cardiovascular anomalies was diagnosed using echocardiography. The ostium of the abnormal membrane dividing the right atrium into two abnormal chambers was surgically excised under cardiopulmonary bypass. All clinical abnormalities were resolved after surgery. However, seven months later, ascites and CTD recurred. A balloon-expandable stent was placed at the site of recurrence, which improved caudal venous return to the right ventricle. After the second procedure, ascites were resolved, and the dog remained asymptomatic for 18 months without complications. There are previous reports of successful surgical resection of the CTD and of stenting in recurrent CTD following balloon dilation in dogs. However, to the authors' knowledge, no previous reports have described recurrent CTD in dogs after surgical resection of the CTD. In this case, as with recurrent CTD following balloon dilation, stent placement at the site of the CTD can be a viable treatment option when the abnormal membrane recurs., (Copyright © 2024. Published by Elsevier B.V.)

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., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

A hybrid surgical approach was utilized to address cor triatriatum sinister in a 10-month-old castrated male domestic shorthair cat. Prior to surgery, open-mouth breathing occurred with exertion. The procedure was guided by transesophageal echocardiography and fluoroscopy. A minithoracotomy was performed, and the left atrial membrane was accessed via an introducer placed through the left ventricular apex. Sequential balloon dilations were performed, and the mean transmembrane gradient under anesthesia was reduced from 16 mmHg to 2.23 mmHg. Four months after surgery, the transmembrane mean gradient remained lower than prior to intervention. The cat tolerated surgery well and remains free of clinical signs nine months after surgery., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Persistent junctional reciprocating tachycardia is a rare form of refractory atrioventricular reentrant tachycardia that accounts for <1% of supraventricular tachycardia in pediatrics. The accessory pathways are generally isolated with few reported underlying structural heart defects. We present a case of a five-month-old male with refractory tachyarrhythmia found to have cor triatriatum sinister, which to our knowledge, is the first reported case of these two rare anomalies coexisting., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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).)

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., Competing Interests: Declaration of Competing Interest None. The patient provided written informed consent for the publication of this e-challenge., (Published by Elsevier Inc.)

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.)

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Cor triatriatum sinister is rare congenital heart disease. It is defined as the presence of an abnormal septum dividing the left atrium into two chambers, and in some cases may be associated with a total abnormal pulmonary venous connection. Prenatal diagnosis of cor triatriatum sinister may be possible with fetal echocardiography and the diagnosis can be made earlier than postnatal imaging techniques. In the fetal echocardiography performed in our patient, cor triatriatum sinister was present and the main pathology was the flow of right and left pulmonary vein return from the accessory atrium to the right atrium., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

A 37-year-old male patient with corrected transposition of great arteries (ccTGA) with cor triatriatum sinister (CTS), left superior vena cava, and atrial septal defects is reported in our case. None of these impacted the patient's growth or development, nor daily work until age 33. Later, the patient developed symptoms of obvious impaired heart function, which improved after medical treatment. However, the symptoms reappeared and gradually worsened two years later, and we decided to treat it with surgery. In this case, we selected tricuspid mechanical valve replacement, cor triatriatum correction, and atrial septal defect repair. During the follow-up of five years, the patient had no obvious symptoms, ECG did not change significantly from five years ago, and the cardiac color Doppler ultrasound showed RVEF 0.51.

Background: 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., Methods: 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., Results: 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%)., Conclusions: 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., (© 2022 Wiley Periodicals LLC.)

Current classifications of cor triatriatum sinister (CTS) do not address the associated heart defects or single ventricle pathology. Therefore, these classifications are not prognostic classifications and only describe the anatomy and the pulmonary venous drainage. The proposed classification considered the associated congenital cardiac lesions and the single ventricle pathology, therefore, it could have prognostic value. Future multicenter studies are required to measure the performance of this classification and its prognostic value in patients with CTS., (© 2022 Wiley Periodicals LLC.)

Objectives: Report the long-term outcomes following transmembrane stent placement as a therapy for Cor Triatriatum Dexter (CTD)., Materials and Methods: Retrospective case series including six dogs with CTD treated with transmembrane stent placement. Follow-up information was obtained including the persistence of presenting clinical signs, additional therapies required, and survival., Results: The median follow-up time was 24 months (range 15-76 months). Long-term outcome was deemed excellent in four dogs (67%), good/fair in one dog (17%), and poor in one dog (17%). Three dogs had persistence of clinical signs of variable severity. These three dogs were Labrador Retrievers or their crosses with varying degrees of tricuspid valve dysplasia (TVD), two of which also had a right to left shunting patent foramen ovale (PFO). One of these three dogs died 23 months post-stent placement during attempted open-heart repair of the TVD and PFO. Another is alive 15 months post-operatively stable on medical therapy for right-sided congestive heart failure secondary to TVD. The final dog demonstrated improved but persistent mild exercise intolerance up to 76 months post-operatively associated with mild TVD and a concurrent PFO., Conclusions: Transmembrane stent placement for CTD is a viable long-term treatment option with improvement or resolution of clinical signs. In the presence of concurrent congenital heart disease, specifically Labradors with TVD, additional therapies may be necessary with a corresponding impact on prognosis., Competing Interests: Conflict of Interest Statement The authors do not have any conflicts of interest to disclose., (Copyright © 2022 Elsevier B.V. All rights reserved.)

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).)

Cor triatriatum may be associated with abnormalities of pulmonary venous anatomy. This case report describes a unique form of partial anomalous pulmonary venous connection. The patient presented at 5 weeks of age with symptoms of tachypnea and poor feeding. Echocardiography demonstrated cor triatriatum and partial anomalous pulmonary venous drainage of the right upper lung. The patient underwent urgent repair of cor triatriatum. It was elected to not address the partial anomalous pulmonary venous connection at that time. The patient returned at age 19 months for elective repair of the anomalous pulmonary venous connection. There was also a large vein connecting the right lower pulmonary veins to the superior vena cava. This was repaired by dividing the superior vena cava along a vertical axis to redirect the flow of the anomalous pulmonary veins through the connecting vein to the left atrium. This report describes the anatomy and surgical approach to a unique form of anomalous pulmonary venous connection.

Introduction: Cor triatriatum sinister (CTS) is a rare congenital heart defect characterized by fibromuscular septation of the left atrium associated with atrial fibrillation (AF). The incidence of hemodynamically insignificant CTS in the AF ablation population and effect on ablation success are not known. Furthermore, little is known about the potential effect of CTS on arrhythmogenic substrate., Objective: We define the incidence of hemodynamically insignificant CTS in patients undergoing AF ablation with RF and cryoballoon ablation, the technical challenges created by the left atrial partitioning, and the potentially arrhythmogenic effects of the membrane. We also review the literature of CA in patients with CTS., Methods: First-time AF ablation cases at our institution over a 10-year period were screened to identify patients with CTS. Retrospective review was performed to obtain clinical characteristics and ablation data., Results: Of the 3953 consecutive patients undergoing initial AF ablation during the study period, four patients (0.10%) had CTS. Ablation was successful acutely in all patients. One patient had recurrent AF and required repeat ablation for a single procedure success rate of 75% and multi-procedure success rate of 100%. The CTS membrane was associated with low voltage zones in the two patients in whom it was measured and with substrate for macro-reentrant atrial tachycardia in one of these patients., Conclusion: The incidence of hemodynamically insignificant CTS in patients undergoing CA for AF is very low, but does not serve as a significant barrier to successful ablation as long as directed access to the superoposterior chamber is obtained., (© 2021 Wiley Periodicals LLC.)

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.)

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., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

Divided atrium (or Cor triatriatum) dexter (DAD) is a rare congenital cardiopathy, usually associated with other anomalies; isolated forms are even rarer. We report the case of an 84-year-old woman presenting with isolated DAD complicated by right-left atrial shunt through patent foramen ovale (PFO), revealed by right cardiac failure and severe hypoxemia. Late destabilization was caused by total superior vena cava thrombosis, related to her pacemaker. Given the overall context, complete percutaneous treatment was performed, associating PFO occlusion and stent implantation in the right atrial membrane, providing total regression of symptoms. Thus, percutaneous correction of DAD appears to be safe and effective., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

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., Competing Interests: Disclosure: The authors report no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly representing <0.1% of all congenital cardiac malformations. It is characterized by the presence of a left atrial (LA) membrane that leads to left ventricular inflow obstruction. Uncorrected, obstructed CTS may have significant sequelae such as pulmonary hypertension or arrhythmias. Transcatheter balloon decompression has been described as a successful alternative to surgical resection of the obstructing membrane. Our review of the literature revealed no reported cases utilizing radiofrequency (RF) energy to perforate the CTS membrane prior to balloon decompression. This manuscript describes two patients with CTS who were treated successfully with a transcatheter technique using RF energy to perforate the obstructing membrane prior to balloon angioplasty and decompression of the LA., (© 2021 Wiley Periodicals LLC.)

Adult cor triatriatum sinister associated with severe mitral regurgitation is extremely rare. As these obstructive cor triatriatum feature hemodynamics that mimic mitral stenosis, a pressure load is theoretically generated only on the left atrial proximal chamber, and therefore the left ventricle is less likely to suffer volume loading. Here, we report a surgical case with such rare hemodynamics. A 22-year-old man with obstructive cor triatriatum and severe mitral regurgitation received an anomalous membrane excision and mitral annuloplasty. An abnormal membrane with an orifice 7 mm in size was completely resected while a grossly dilated mitral annulus was repaired via annuloplasty ring. Mitral regurgitation was controlled well, and the postoperative course was uneventful. Even with obstructive cor triatriatum, severe mitral annular dilatation and subsequent left ventricular dilatation may occur, causing the progressive heart failure encountered in this case.

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.

Objectives: Cor Triatriatum is a rare anomaly that can either involves the left atrium (Cor Triatriatum Sinister-CTS) or the right atrium (Cor Triatriatum Dexter- CTD). Preoperative identification of this anomaly is important in determining patient treatment course. The objective of this paper is to understand imaging findings, classification and to familiarise the reader with other associated congenital cardiac anomalies that influence patient management., Methods: From the hospital electronic health records (EHR) database, we identified 10 patients of Cor Triatriatum out of 974 patients who underwent cardiac CT between 15 July 2014 and 20 March 2020 for congenital heart disease. Medical records and imaging findings were reviewed retrospectively., Results: Out of 10 patients, nine patients had CTS (90%) and only one patient had CTD (10%). Five out of nine patients (55.5%) had CTS type II and four (44.4%) had CTS type III. The mean of the membrane orifices in CTS type III was 18.5 mm and was 5.78 mm in CTS type II. Pulmonary veins were dilated in all patients of CTS type II (62.5%), two patient of CTS type III (25%) and in only patient with CTD (12.5%). Ostium secundum atrial septal defect was the most common (66%) associated cardiac anomaly, followed by ventricular septal defect (44%)., Conclusions: CT allows excellent pre-operative evaluation of Cor Triatriatum and associated cardiac anomalies., Advances in Knowledge: CT is excellent in making a diagnosis and classifying Cor Triatriatum and for identification of cardiac anomalies and complications associated with it.

Background: Cor triatriatum is the rarest of all congenital cardiac diseases, accounting for 0.1-0.4% of congenital heart diseases. Atrial septal defect is the most common associated defect; however, cor triatriatum is sometimes associated with univentricular heart., Methods: This single-centre retrospective study involved all patients who underwent the repair of cor triatriatum at Kobe Children's Hospital between 2000 and 2020. Twenty-four patients were required surgery. We conducted a survey of survival rate, early and late pulmonary vein stenosis in each group., Results: The survival rate of 5 years after cor triatriatum resection was 100% in the biventricular group and 82.1% in the univentricular group, respectively. The free rate for pulmonary stenosis of 5 years after surgery was 100% in the biventricular group and 90.0% in the univentricular group, respectively. There was no statistical difference in survival rate and 5 years free rate for pulmonary stenosis after surgery., Conclusions: The results showed that surgical correction offers good early and mid-term outcomes for both cor triatriatum with biventricular and univentricular physiologies.

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., (© 2020 Wiley Periodicals LLC.)

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

Persistent left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect (Raghib complex) is an uncommon anomaly of systemic venous drainage. We present a unique case of simultaneous presentation of cor triatriatum and persistent left superior vena cava draining into the left atrium in an adult female with partial AV canal and common atrium. Complex intra-atrial baffling including a procedure to redirect flow from a proximal atrial chamber was successful.

Background: Cor triatriatum is an exceedingly rare congenital heart defect which can present at any age depending upon the presence of associated cardiac anomalies and the size of communication between the common chamber and the left atrium. Our aim is to present 10 years surgical outcome of cor triatriatum repair in patients who present beyond infancy., Methods: On going through our institute's surgical database from January 2009 to June 2019 for cor triatriatum, 14 patients were found to have undergone the surgical correction. Their demographic profile and midterm outcome in terms of mortality, functional status, and cardiac function was assessed., Results: There were total 14 patients with slight female predominance (57.14%) who underwent cor triatriatum surgical repair. The mean age at the time of surgery was 12.14 ± 9.97 years ranging from 1 to 29 years. Associated congenital cardiac defects were seen in 12 (85.71%) patients with atrial septal defect being the most common. Partial anomalous pulmonary venous connection was seen in 4 (28.57%) patients. Eight (57.14%) patients had severe pulmonary hypertension preoperatively. All but two patients showed marked reduction in pulmonary artery pressures postoperatively. All the patients underwent excision of the membrane and repair of associated cardiac defects simultaneously. There was no early or midterm mortality or any reintervention over a mean follow-up period of 58.06 ± 30.73 months (range 20-120 months)., Conclusion: The early and midterm surgical outcome of cor triatriatum is excellent even in patients who present beyond infancy with reversible pulmonary artery hypertension., (© 2019 Wiley Periodicals, Inc.)

Obstructive total anomalous pulmonary venous return (TAPVR) is a potentially life-threatening clinical condition and a surgical emergency. Left-sided partial anomalous pulmonary venous return (PAPVR) in association with a subdivided left atrium, also known as cor triatriatum sinister (CTS), is a very rare malformation that can mimic obstructive TAPVR. We present a case of a newborn with clinical manifestation of cardiogenic shock caused by CTS and PAPVR.

Background: We performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease., Methods: A PubMed literature search for 'cor triatriatum sinistrum' published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared., Results: A total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30-60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28-52) vs 50 years (IQR, 32-64), p=0.003). Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004).A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001)., Conclusion: The natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.

Cor triatriatum sinister is an uncommon cardiac abnormality characterized by a membrane that divides the left atrium into two chambers. Definitive management requires surgical resection, traditionally through sternotomy. Minimally invasive reparative techniques are associated with reduced blood loss, shorter hospitalization, faster recovery time, and improved cosmesis with excellent patient satisfaction. We present a 29-year-old woman with cor triatriatum sinister and associated atrial septal defect who underwent successful minimally invasive repair through a right periareolar approach.