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Background Cor triatriatum sinister (CTS) is a rare congenital heart defect sometimes complicated with atrial fibrillation (AF). Catheter ablation (CA) relieves the AF-associated symptoms, but CA for AF with CTS has been reported rarely. Because CTS can be associated with other congenital heart disease, detailed preoperative assessment is important. Case summary An 80-year-old man was referred to our institution for shortness of breath that had persisted for 2 months when he was first diagnosed with AF. Transthoracic echocardiography revealed an enlarged left atrium (LA) divided into two chambers by a membrane. Transoesophageal echocardiography showed the membrane extending from the fossa ovalis (FO) to the Coumadin ridge, with the accessory (dorsal) chamber (AC) in closer proximity to the FO. Computed tomography showed that all pulmonary veins (PVs) flowed into the AC, with no PV anomalies. No other heart anomaly was identified, with no thrombus in the LA. With these findings, PV isolation (PVI) with CA was considered safe. Transseptal puncture was performed with intracardiac echocardiography for precise catheterization of the AC. Pulmonary vein isolation was performed successfully. The patient was discharged 4 days after the procedure, without any complications. His symptoms improved post-procedure, and sinus rhythm was maintained without antiarrhythmic drug therapy during the 18-month follow-up. Discussion Cor triatriatum sinister is a rare anomaly that accounts for 0.1% of all congenital heart diseases. Cor triatriatum sinister sometimes complicated with symptomatic AF. Detailed preoperative anatomical assessment with multiple imaging modalities helped us achieve safe and effective CA for a patient with AF and CTS, even in an octogenarian. [ABSTRACT FROM AUTHOR]

Abstract 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.

Key Clinical Message: Cor triatriatum is a rare congenital heart abnormality in which a membrane separates the left atrium (LA; sinister) or the right atrium (dexter) into two compartments. It is also a long‐forgotten cause of atrial fibrillation (AF) and substantially higher rates of blood stagnation, particularly proximal to the additional septum in the LA. In this case report, we faced a CHA2DS2‐VASc score of 1 in patients with non‐valvular AF due to Cor triatriatum sinister (CTS). The decision to start anticoagulants in this particular case was controversial, so we reviewed the literature to assess and address it. We present our case and discuss the indication of anticoagulants in this unique clinical scenario, accompanied by a literature review. Facing this dilemma of starting anticoagulants in special cases of CTS and AF should be individualized and need more investigation. However, till this moment, based on similar reports, it seems to be rational to consider CTS Per se as an additional risk stratification marker beyond the CHA2DS2‐VASc score start anticoagulant until the surgical resection. Considering CTS as the sole indication of anticoagulant in patients with normal sinus rhythm is a complex matter that needs further investigation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Key Clinical Message Cor triatriatum is a rare congenital heart abnormality in which a membrane separates the left atrium (LA; sinister) or the right atrium (dexter) into two compartments. It is also a long‐forgotten cause of atrial fibrillation (AF) and substantially higher rates of blood stagnation, particularly proximal to the additional septum in the LA. In this case report, we faced a CHA2DS2‐VASc score of 1 in patients with non‐valvular AF due to Cor triatriatum sinister (CTS). The decision to start anticoagulants in this particular case was controversial, so we reviewed the literature to assess and address it. We present our case and discuss the indication of anticoagulants in this unique clinical scenario, accompanied by a literature review. Facing this dilemma of starting anticoagulants in special cases of CTS and AF should be individualized and need more investigation. However, till this moment, based on similar reports, it seems to be rational to consider CTS Per se as an additional risk stratification marker beyond the CHA2DS2‐VASc score start anticoagulant until the surgical resection. Considering CTS as the sole indication of anticoagulant in patients with normal sinus rhythm is a complex matter that needs further investigation.

Cor Triatriatum Sinister (CTS) represents a rare congenital cardiac defect characterized by the presence of a fibro-muscular membrane separating the left atrium into two cavities. During adulthood CTS may be asymptomatic and incidentally found during imaging diagnostic workflow. Bidimensional (2D) transthoracic echocardiography (TTE) is considered the first-line imaging technique for the evaluation of CTS. Cardiac magnetic resonance (CMR) and transoesophageal echocardiography (TEE) are advanced diagnostic tools, usually required to obtain additional data. When these diagnostic techniques are contraindicated or not available, three-dimensional (3D) TTE may provide useful information which may help in the morphological assessment of CTS. Moreover, this widespread technique is safe and frequently available in the echocardiography laboratories, although the acoustic window and the training of the operator may be limiting factors. With this case report we sought to emphasize the usefulness of this technique as a valid alternative to CMR and TEE in selected settings. [ABSTRACT FROM AUTHOR]

Necropsy of a 52-day-old Camborough pig revealed numerous cardiac malformations. The positional relationship of the atria, ventricles and great vessels was a mirror image type (I, L and L): inverted arrangement of the atria, with a left-sided right atrium and right-sided left atrium (situs inversus); inverted arrangement of the ventricles, with a left-sided morphological right ventricle and right-sided morphological left ventricle (L-loop); and aortic valve to the front left relative to the pulmonary valve (L-malposed). The major malformations included an ostium secundum atrial septal defect, cor triatriatum sinister (CTS), a subpulmonary ventricular septal defect and a bicuspid pulmonary valve. Histological examination revealed myocyte hypertrophy, focal myocardial necrosis and calcification in the left morphological right ventricle of the heart. To the best of our knowledge, this is the first report of CTS in pigs. Although the individual malformations found in the present case are not unique, an unusual combination of these cardiac malformations has not been described in animals. [Display omitted] [ABSTRACT FROM AUTHOR]

We report a case of non-thrombosed cor triatriatum sinister simulating a cardiac mass on thoracic CT angiogram in a 58-year-old man presenting with acute chest pain. Following additional imaging with cardiac CT and MRI, and otherwise unremarkable cardio-pulmonary work-up, diagnosis of presumably coincidental cor triatriatum was established.

Cor triatriatum sinister (CTS) is a rare congenital cardiac malformation. In CTS, a fibromuscular membrane subdivides the left atrium into 2 chambers. The communication between the 2 chambers is through 1 or more orifices in the dividing membrane. We present an interesting case of a 2-month-old infant with obstructed CTS membrane who first presented on account of poor feeding and failure to thrive. Echocardiography showed a persistent levoatrial cardinal vein (LACV) connecting the left atrium and the innominate vein. This allowed the proximal left atrial chamber to decompress its blood volume into the innominate vein and subsequently the superior vena cava. There was minimal prograde blood flow across the Cor triatriatum membrane, so the majority of pulmonary venous blood ultimately returned to the heart by way of the decompressing vertical vein into the systemic venous circulation. Surgical repair was performed with an uneventful postoperative course. The specific anatomical variant of Cor triatriatum found in our subject has rarely been reported. [ABSTRACT FROM AUTHOR]

The present case report describes a rare case of a cardiac abnormality diagnosed as Supravalvular Mitral Stenosis in an asymptomatic cat. An 11-years old cat was presented for orthopedic evaluation, and during general clinical examination a heart rate of 180 bpm and left diastolic cardiac murmur grade III–IV/VI, between the mitral and aortic foci, were found. Radiographic, echocardiographic, angiocardiographic and post-mortem (the patient died during anesthesia performed to diagnose the orthopedic condition) magnetic resonance and pathologic findings are reported herein. [ABSTRACT FROM AUTHOR]

Cor triatriatum is a rare condition. It comprises around 0.1% to 0.4% of all congenital heart malformations. There are two types of this anomaly: cor triatriatum sinister (CTS), which is more common and accounts for 92% of all cases, and cor triatriatum dexter (CTD). This condition usually presents at an early age and is diagnosed mainly during early childhood. In some patients, who have less severe cases of CTS, diagnosis could be made in adulthood. We report an unusual case of a 78-year-old woman who was diagnosed with CTS for the first time. The patient was admitted to the cardiology department with symptoms of chest pain and dyspnoea. Physical examination revealed diffusely diminished breath sounds, with focal wheezing, and irregular heart rate, with no murmurs, while blood pressure was 140/90 mmHg. A transthoracic echocardiogram revealed an accessory membrane in the left atria suggestive of CTS.

In the literature, multiple radiological signs of clinically important congenital cardiovascular abnormalities have been described which are named as per the object they resemble. These signs are very useful in learning. The Gooseneck sign is a sign seen in left ventricle catheter angiography in case of an atrioventricular septal defect. This sign has not been demonstrated on a cardiac computed tomography (CT) so far, to the best of our knowledge. We present a case showing cardiac CT pictorial equivalent of angiographic Gooseneck sign.

In the literature, multiple radiological signs of clinically important congenital cardiovascular abnormalities have been described which are named as per the object they resemble. These signs are very useful in learning. The Gooseneck sign is a sign seen in left ventricle catheter angiography in case of an atrioventricular septal defect. This sign has not been demonstrated on a cardiac computed tomography (CT) so far, to the best of our knowledge. We present a case showing cardiac CT pictorial equivalent of angiographic Gooseneck sign. [ABSTRACT FROM AUTHOR]

Cor triatriatum sinister (CTS) is a relatively rare congenital condition characterized by an abnormal septum dividing the left atrium, morphologically presenting as three atria. Although most individuals with heart failure related to CTS undergo surgical treatment in childhood, those with larger fenestrations may remain asymptomatic until they reach an age where surgery is less viable, presenting treatment challenges. In our case study, we report on an elderly woman who declined all invasive treatments and developed heart failure due to severe functional mitral valve regurgitation triggered by atrial fibrillation. She opted for conservative treatment after a comprehensive evaluation of her condition using transesophageal echocardiography, cardiac magnetic resonance imaging, and right-heart catheterization. This multimodal evaluation highlights the importance of accurate diagnostic approaches and tailored treatments for elderly patients with CTS., Competing Interests: No conflict of interest., (© The Author(s) 2024. Published by Oxford University Press.)

Background Atrial fibrillation (AF) is a common cardiac arrhythmia, which is often associated with underlying risk factors and undiagnosed conditions, including congenital heart disease. Atrial septal defects (ASDs) come to mind, albeit arrhythmias usually present later in life. We present herewith a young patient with cor triatriatum sinister (CTS), with some delay in establishing the diagnosis, following new onset AF in early adulthood. Case summary A 31-year-old man presented with pre-syncope and coryzal symptoms and was newly diagnosed with AF in the context of an intercurrent viral illness. After treatment with oral anticoagulation and successful outpatient cardioversion, he was discharged from cardiology review. Two years later he re-presented with exercise intolerance and a 12-lead electrocardiogram revealing recurrence of AF. Subsequent investigation with transthoracic echocardiography revealed the underlying congenital cardiac defect of CTS, together with an ASD and patent foramen ovale. After corrective surgery, which involved membrane resection, a Cox-maze procedure and ASD closure, sinus rhythm was restored and at follow-up the patient had returned to baseline function. Discussion Young patients presenting with new onset AF should undergo thorough cardiovascular assessment to identify treatable causes and reversible risk factors. Cor triatriatum sinister is a rare congenital anomaly that may present in adulthood and give rise to symptomatic AF. Surgical correction including a Cox-maze procedure in our patient resulted in restoration of sinus rhythm and a return of the patient's baseline functional status and improved quality of life. [ABSTRACT FROM AUTHOR]

Atrial fibrillation is the most common presentation in adult patients with cor triatriatum sinister. The key to successful and safe catheter ablation in these patients is an accurate exploration and thorough understanding of the left atrial anatomy, both before and during the procedure. Catheter manipulation is highly dependable on left atrial anatomy, including the interatrial septum, insertion of pulmonary veins and cor triatriatum membrane. Anatomical variants such as the left common pulmonary trunk may influence the ablation approach and outcome. We report the case of a 52-year-old patient with cor triatriatum sinister and the left common pulmonary vein variant who underwent successful high-power, short-duration catheter ablation for paroxysmal atrial fibrillation. [ABSTRACT FROM AUTHOR]

Coronary computed tomography angiography (CCTA) is a noninvasive examination whose main purpose is to exclude significant stenosis in the coronary arteries. The obtained computed tomography images may also provide information about other coexisting pathologies of the heart and vessels. The paper presents images of cardiac lesions in a 44-year-old hypertensive patient who underwent CCTA, based on which significant stenosis in the coronary arteries was excluded, the suspicion of a cor triatriatum sinister was confirmed and the presence of fibroelastoma and a variant of the anatomy of the pulmonary veins ostial was confirmed. To sum up, when performing CCTA, apart from the analysis of the coronary arteries, one should remember about lesions in the remaining visible anatomical structures of the heart and large vessels. [ABSTRACT FROM AUTHOR]

• Alström syndrome is a rare inherited ciliopathy caused by mutations in ALMS1 gene. • Our report is the first description of an ALMS patient with a CTS structure. • Novel compound heterozygous variants were identified in the patient. • The exact breakpoint site of the large deletion was determined by breakpoint analysis. • The deletion maybe formed through the microhomology-mediated end joining mechanism. Alström syndrome (ALMS), a rare recessively inherited ciliopathy caused by mutations in ALMS1 , is characterized by retinal dystrophy, childhood obesity, sensorineural hearing loss, and type 2 diabetes mellitus. The majority of pathogenic variants in ALMS1 are nonsense and frameshift mutations, which would lead to premature protein truncation, whereas copy number variants are seldom reported. Herein, we present a 10-year-old Chinese girl with ALMS. The potential causative genetic variant was confirmed through whole genome sequencing, quantitative real-time PCR analysis, and Sanger sequencing. Additionally, breakpoint analysis was performed to determine the exact breakpoint site of the large deletion and elucidate its probable formation mechanism. The patient had a cor triatriatum sinister (CTS) structure. Genetic analysis identified novel compound heterozygous variants in the patient, consisting of a frameshift variant c.4414_4415delGT (p.V1472Nfs*26) in ALMS1 and a novel large deletion at chr2:73,612,355–73,626,339, which encompasses exon 1 of the ALMS1 gene. Moreover, breakpoint analysis revealed that the large deletion probably formed through the microhomology-mediated end joining (MMEJ) mechanism due to the 6-bp microhomologies (TCCTTC) observed at both ends of the breakpoints. In this study, novel compound heterozygous variants in the ALMS1 gene were identified in an ALMS patient with a CTS structure. The molecular confirmation of these variants expands the mutational spectrum of ALMS1 , while the manifestation of ALMS in the patient provides additional clinical insights into this syndrome. [ABSTRACT FROM AUTHOR]

Levoatriocardinal vein has rarely been described with cor triatriatum. We report a case of a newborn with respiratory distress who was found to have cor triatriatum sinister with a decompressing levoatriocardinal vein on transthoracic echocardiography. The pulmonary venous confluence received all pulmonary veins and drained to the proximal left atrial chamber through significant communication. Moreover, the cor triatriatum membrane separated the left atrial body into proximal and distal left atrial chambers with a restrictive opening in the membrane, causing severe flow limitation to the cardiac output, severe pulmonary arterial hypertension, and significant right ventricular dilatation. In addition, a sizeable levoatriocardinal vein decompressed the pulmonary venous confluence drained superiorly to the left innominate vein. She had a successful surgical repair by resectioning the cor triatriatum membrane and ligating the levoatriocardinal vein. The patient had improved pulmonary arterial pressures and right ventricular dimensions at the one-month follow-up. The case highlights the rare association of the levoatriocardinal vein and cor triatriatum, and its presence signifies severe obstruction at the level of cor triatriatum., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Qassim Health Cluster Research Ethics Committee issued approval 22-1501. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Bhat et al.)

Cor triatriatum sinister is a rare congenital heart disease characterized by an additional fibromuscular membrane in the left atrium. Cardioembolic stroke is a rare complication of cor triatriatum sinister, especially among women. We hereby describe the case of an 18-year-old female patient, without a past medical history, presenting with cardioembolic stroke in the territory of the right posterior cerebral artery. During extensive diagnostic work-up, nonrestrictive cor triatriatum sinister and patent foramen ovale were diagnosed using transthoracic and transesophageal echocardiography. In clinical practice, it is important to identify congenital cardiac defects as potential substrates for cardioembolism in young patients. In our case, cor triatriatum sinister presenting as ischemic stroke was diagnosed, which is an uncommon finding, especially in young females. Determining the optimal management strategy for patients with cor triatriatum sinister complicated by cardioembolic stroke requires a multidisciplinary approach.

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]

A case of Cor Triatriatum Sinister presenting with acute myocardial infarction is described. The patient underwemt a successful coronary angioplasty.

Atrial fibrillation is the most common presentation in adult patients with cor triatriatum sinister. The key to successful and safe catheter ablation in these patients is an accurate exploration and thorough understanding of the left atrial anatomy, both before and during the procedure. Catheter manipulation is highly dependable on left atrial anatomy, including the interatrial septum, insertion of pulmonary veins and cor triatriatum membrane. Anatomical variants such as the left common pulmonary trunk may influence the ablation approach and outcome. We report the case of a 52-year-old patient with cor triatriatum sinister and the left common pulmonary vein variant who underwent successful high-power, short-duration catheter ablation for paroxysmal atrial fibrillation.

Coronary computed tomography angiography (CCTA) is a noninvasive examination whose main purpose is to exclude significant stenosis in the coronary arteries. The obtained computed tomography images may also provide information about other coexisting pathologies of the heart and vessels. The paper presents images of cardiac lesions in a 44-year-old hypertensive patient who underwent CCTA, based on which significant stenosis in the coronary arteries was excluded, the suspicion of a cor triatriatum sinister was confirmed and the presence of fibroelastoma and a variant of the anatomy of the pulmonary veins ostial was confirmed. To sum up, when performing CCTA, apart from the analysis of the coronary arteries, one should remember about lesions in the remaining visible anatomical structures of the heart and large vessels.

Introduction and Importance: Cor triatriatum sinister (CTS) is an uncommon heterogeneous congenital cardiac defect that may manifest in adulthood when symptomatic blockage manifests due to a change in hemodynamic physiology or when a condition such as atrial fibrillation (AF) arises. The incidence of cor triatriatum with cardiomyopathy and congenital heart illness ranges from 0.1 to 0.4%., Case Presentation: The Department of Cardiology examined a 10-year-old girl for a diastolic murmur. The patient's medical history was ordinary, and no previously known co-morbid illnesses were present. Pneumonia was the patient's original medical condition. A cardiac murmur was also discovered during the physical examination, and the patient was forwarded for more research. Physical examination revealed just a diastolic murmur, which was noteworthy. The patient's care plan includes routine echocardiographic monitoring. Since the deformity was not clinically significant, surgical repair was not advised., Clinical Discussion: It is unclear what caused this flaw. A full, incomplete, or fenestrated septum may be the result of the common pulmonary vein failing to merge with the left atrium during embryonic development. The existence of a fibromuscular membrane, which separates the left atrium (LA) into two chambers-the proximal chamber receiving the pulmonary veins (PVs) and the distal chamber contains the mitral valves and LA appendages defines the disease., Conclusion: The diagnostic procedure should be carried out in the optimal settings, however, in cases of frail health systems, employing accessible alternatives might help the early diagnosis. Early referral to a cardiologist is required in case of CTS suspicion., Competing Interests: The authors declare no conflict of interest.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

The aim of our study is to present the case of a six weeks old infant, prematurely born, who was admitted to our hospital with systolic murmur and cyanosis. The diagnosis of cor triatriatum sinister associated with patent foramen ovale has been established by ultrasound examination. The infant died a month after admission. The necropsy confirmed the clinical diagnosis and also revealed the hypertrophy and dilation of the right heart, left ventricle hypoplasia, interstitial pneumonia and amniotic fluid aspiration bronchopneumonia. The possible cause of death in this case was right heart failure following pulmonary hypertension associated with acute respiratory failure. To our knowledge

Cor triatriatum sinister is an extremely rare congenital heart defect. Very few cases have been reported for cor triatriatum associated with mitral regurgitation. We are reporting an exceptional case of cor triatriatum associated with calcified mitral valve resulting mitral stenosis. Preoperatively, mitral stenosis was underestimated due to the presence of restricted communication between two chambers of the left atrium. The patient was operated for cor triatriatum repair with mitral valve replacement.

A key to the success of catheter ablation in complex congenital heart disease is an accurate delineation of the anatomy. Here we describe the efficiency of intracardiac echocardiogram in guiding the catheter ablation of persistent atrial fibrillation in a 55-year-old Japanese male with cor triatriatum sinister. Echo imaging provided a detailed three-dimensional anatomy of the whole left atrium and identified an ideal trans-septal puncture site that allowed catheter access to both the accessory and main chambers of the left atrium. We review similar cases from the literature. < Learning objective: Cor triatriatum sinister is a rare cardiac anomaly wherein a fibromuscular membrane divides the left atrium into two parts. An accurate delineation of the entire left atrial anatomy including the membrane by intracardiac echocardiography may facilitate the catheter ablation procedure of atrial fibrillation, especially when deciding the optimal trans-septal catheterization site to map both left atrial chambers.> [ABSTRACT FROM AUTHOR]

Background Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly defined by a fibromuscular membrane which bisects the left atrium. Cor triatriatum sinister has been associated with cardioembolic stroke through mechanisms including stagnation of blood flow within the left atrium, an association with atrial fibrillation (AF), and/or an accompanying atrial septal defect (ASD) or patent foramen ovale. We describe a case highlighting the role that CTS may play in cardioembolic stroke, provide high-quality computed tomography angiography and two- and three-dimensional echocardiography of the CTS membrane, and outline management strategies for this uncommon clinical scenario. Case summary A 35-year-old man with no prior medical history presented with acute onset weakness and aphasia. He was found to have an embolic stroke with left M1 and A1 occlusions and received tissue plasminogen activator followed by mechanical thrombectomy with successful recanalization. A thorough stroke workup revealed CTS with an associated ASD as well as potential protein C deficiency. He was managed with indefinite anticoagulation with apixaban. Discussion This is the 13th reported case of CTS associated with stroke. In most previous cases evidence of blood stasis or frank thrombus was associated with the CTS membrane, and/or existing AF was noted. In this case, none of these were identified, particularly highlighting the surreptitious risk of CTS. In addition, the presence of potential protein C deficiency in this case compounded the risk for thromboembolism and factored into multidisciplinary management decisions. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We herein report a case of coarctation of the aorta (CoA) in an asymptomatic adult who had a preserved exercise capacity. A 56-year-old man with mild hypertension exhibited left ventricular hypertrophy on an electrocardiogram during an annual medical checkup. Echocardiography showed a bicuspid aortic valve and cor triatriatum sinister, and subsequent computed tomography revealed CoA with developed collaterals. Cardiopulmonary exercise testing showed a good exercise capacity. He refused surgical repair and has been asymptomatic for five years. This case is of special interest, as CoA is usually rarely noticed during adulthood if there are no severe congenital anomalies, and in addition, this patient had good exercise capacity.

A 57-year-old male with persistent atrial fibrillation (AF) was referred for catheter ablation. Multidetector computed tomography (MDCT) revealed that a membrane divided the left atrium into two chambers, thus indicating the presence of cor triatriatum sinister. A 3D image reconstructed by MDCT showed that the accessory atrium received the left common and the right side PVs, as if it were a total common trunk, and this then flowed into the main atrium. After isolation of the pulmonary vein and posterior wall from the left atrium, AF could not be induced by any programmed pacing. The patient has remained free from AF during the 1 year of follow-up. [ABSTRACT FROM AUTHOR]

Cardiac asthma or cardiac wheezing (CW) refers to a syndrome of dyspnea and wheezing that mimicks asthma clinically. Reported herein is the case of a 2-month-old boy who presented with refractory wheezing as a sign of cor triatriatum sinister (CTS) that culminated in overwhelming multiple organ failure in a short time. On the day of admission, oxygen saturation (SpO2) was < 80%. Heart rate was 198 beats/min and respiratory rate 58 breaths/min. Chest radiogram showed pulmonary edema. Electrocardiogram showed right atrial enlargement and right ventricular hypertrophy. N-terminal pro-brain natriuretic peptide (NTproBNP) was very high at >20 000 pg/mL. Two-dimensional echocardiography with Doppler showed CTS, which was complicated with severe pulmonary arterial hypertension due to flagrant pulmonary venous obstruction. Cardiac surgery was undertaken, after which pulmonary edema subsided, SpO2 increased to =96%, and NTproBNP dropped to normal. He was discharged 11 days later, and was free of cardiac, pulmonary, renal, and neurological sequelae at 24 month follow up. [ABSTRACT FROM AUTHOR]