Cor Triatriatum Sinister Research Articles

Radiofrequency catheter ablation of persistent atrial fibrillation in a patient with cor triatriatum sinister

Radiofrequency catheter ablation of persistent atrial fibrillation in a patient with cor triatriatum sinister

Successful left atrial catheter ablation in a patient with cor triatriatum sinister navigated by 3D volume rendering technique computed tomography

Successful left atrial catheter ablation in a patient with cor triatriatum sinister navigated by 3D volume rendering technique computed tomography

Exploring new frontiers: a rare case of catheter ablation for persistent atrial fibrillation in a patient with cor triatriatum sinister guided by intracardiac echocardiography

BackgroundCor 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 presentationWe 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.ConclusionsThis 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.

Clinical Application of Exercise Stress Echocardiography in an Outpatient Pediatric Population.

Background: Exercise stress echocardiography (ESE) is commonly employed in adults, but its applicability in pediatric populations remains to be clarified. Methods: A total of 309 consecutive children (C), with a mean age of 14.1 ± 2.6 years (range 6-17 years), underwent treadmill ESE starting in 2002. They were divided into two groups: Group I comprised 258 children, including 237 with symptoms related to exercise (such as chest pain, fatigue, lipothymia/syncope, or one aborted sudden death), 15 with electrocardiogram (ECG) abnormalities, and 6 with a positive ECG stress test showing ST changes. Group II consisted of 10 asymptomatic children whose parents requested routine screening, 11 with symptoms unrelated to exercise, 12 with a family history of sudden death, and 17 with known pathologies (including 10 with hypertrophic cardiomyopathy, 2 with aortic coarctation, and the remainder with various conditions, such as Cortriatriatum sinister, pulmonary stenosis, subaortic stenosis, bicuspid aortic valve, left ventricular hypertrophy related to arterial hypertension, and aortic switch operation). Regional wall motion abnormalities (RWMAs) and transvalvular or intraventricular (IVG) gradients were assessed using 2D and continuous-wave Doppler, respectively, in all cases. Results: The success rate was 100% (309/309). Stress-induced RWMAs were observed in two children. A significant IVG (>30 mmHg) was detected in 101 out of the 258 children (39%) in Group I, who presented with exercise-related symptoms, ECG abnormalities, or positive stress ECG. In Group I, the odds ratio (OR) of ESE reproducing the symptoms in children with IVG compared to those without IVG was 8.22 (95% CI: 4.84-13.99, p < 0.001). Conclusions: Treadmill ESE is both feasible and safe for pediatric populations. RWMAs demonstrated limited utility in our cohort of children, while IVG induced by exercise was frequently observed in symptomatic children.

Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes.

A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality. This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020. Fetuses with other hypoplastic-left-heart variants, inadequate images, lack of follow-up and fetal atrial-septal interventions were excluded. RAS was defined as the ratio of pulmonary-vein forward-to-reverse velocity-time-integral (VTI)≤5 and severe-RAS defined as VTI-ratio <3. Kaplan-Meier survival-analysis was performed for the primary outcome of transplant-free survival for 62weeks after gestational-age of 30weeks (∼1year). Of the 156 consecutive fetuses with HLHS, 11 (7.7%) had L-cor and 8/11 (72.7%) of these had RAS. When compared to HLHS-RAS without L-cor, fetuses with HLHS-RAS and L-cor were less likely to survive to 28days (87% vs. 62.5%, p=0.017) and to 1year (69.6% vs. 25%, p=0.029). When comparing by survival analysis, fetuses with severe-RAS with L-cor had lower survival compared severe-RAS without L-cor (p=0.020). L-cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial-septal-intervention planning.

Three-dimensional (3D) transthoracic echocardiography in Cor Triatriatum Sinister: Make new friends but keep the old

AbstractCor 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.

Multimodality imaging approach to an adult case with cor triatriatum sinister.

Multimodality imaging approach to an adult case with cor triatriatum sinister.

Cor Triatriatum: A Review

Cor triatriatum is a rare congenital cardiac anomaly, characterized by a fibromuscular partition dividing the left (cor triatriatum sinister) or, rarely, the right atrium (cor triatriatum dexter). Occurring in 0.1–0.4% of congenital heart disease cases, it exhibits diverse clinical presentations, often mimicking mitral stenosis and left-sided heart failure, while occasionally remaining asymptomatic into adulthood. The embryological origin of cor triatriatum remains controversial. Recent years have seen the emergence of new classification systems that offer enhanced prognostic insights. Transthoracic echocardiography is the diagnostic cornerstone. Surgical resection, preferably under cardiopulmonary bypass, is the mainstay treatment, and is associated with favorable long-term outcomes.

Obstructed Supramitral Inflow: Cor Triatriatum Sinister Presentation in Adulthood

Cor triatriatum is a rare congenital heart defect that occurs when the atrium is divided into two chambers by a fibromuscular membrane 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, we review the case of a 40-year-old man 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, the intraoperative transesophageal echocardiography (TEE) was used to more accurately evaluate cor triatriatum sinister's morphology, hemodynamic significance and associated anomalies.

Cor triatriatum sinister with dextrocardia in association with ostium secundum atrial septal defect, subpulmonary ventricular septal defect and bicuspid pulmonary valve in a pig

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.

Effective and aesthetic results of horizontal right axillary minithoracotomy for congenital heart defects repair in children

Background: horizontal right axillary minithoracotomy is one of the treatment options for many simple congenital heart defects. This approach shows many aesthetic advantages besides effectiveness and safety. This study aims to: comment on the technical characteristics and early results of horizontal right axillary minithoracotomy in children at Hanoi Heart Hospital.&#x0D; Methods: a descriptive cross-sectional study. We retrospectively reviewed 156 patients who underwent horizontal right axillary minithoracotomy at Hanoi Heart Hospital from January 2022 to December 2022.&#x0D; Results: horizontal axillary approach could be applied even for children with low weight, average weight was 12.22 ± 8.67 kg, the lowest was 2.6 kg and the highest was 51.0 kg. A wide range of congenital heart defects were repaired, including ventricular septal defect closure (84 patients), secundum atrial septal defect closure (22 patients), ventricular septal defect with stenosis repair (31 patients), partial atrio-ventricular septal canal repair (6 patients), repair of atrial septal defect with partial abnormal return of the pulmonary veins (3 patients), repair of ventricular septal defect and atrial septal defect (4 patients), Cor triatriatum sinister repair (4 patients), mitral valve repair (1 patient), atrial septal defect and pulmonary valve stenosis repair (1 patient). Cannulation of the aorta and vena cavae was performed through the main incision, whose size ranged from 3.5 to 5.5cm. The average postoperative hospital stay was 7.34 ± 2.16 days, the rate of severe complications and hospital mortality were 0%.&#x0D; Conclusion: horizontal right axillary minithoracotomy was a good approach to correct many simple congenital heart defects in children. This technique is being applied at Hanoi Heart Hospital safely and provided outstanding advantages in terms of aesthetics.

Cor Triatriatum Sinister Associated with Total Anomalous Pulmonary Venous Connection.

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.

A retrospective study of congenital cardiac malformations in 29 goats.

Cardiac malformations are sporadically diagnosed in domestic species; however, little literature is available for this group of developmental anomalies in goats. We performed a retrospective study to catalog congenital cardiac conditions in goats submitted to the University of California-Davis, Veterinary Medical Teaching Hospital, Anatomic Pathology Autopsy Service. From 2000 to 2021, of 1,886 goat autopsies, 29 cases of cardiac malformations were identified (1.5%). Thirteen were ≤ 2-wk-old, 8 were 1-6-mo-old, and 8 were adults 2-9-y-old. The most common malformations were ventricular septal defect (VSD; 21 of 29), atrial septal defect or persistent foramen ovale (10 of 29), and double-outlet right ventricle (3 of 29). Nine cases had > 1 malformation, typically including a VSD. Conditions that had not been reported in the goat included double-outlet right ventricle (3), tetralogy of Fallot (1), cor triatriatum sinister (1), and mitral valve dysplasia (1). Two adult cases were incidental and not suspected clinically. Cardiac malformations occur not uncommonly in goats and should be considered in a wide age range.

Persistent left superior vena cava without right superior vena cava during fetal life.

Enlarged coronary sinus detected during cardiac examination in the prenatal period is the first finding that raises suspicion for persistent left superior vena cava. In this report, a patient was presented who was referred with the prediagnosis of cor triatriatum sinister in the antenatal period and diagnosed with isolated persistent left superior vena cava (SVC).

PO-04-079 FIRING EXTRA CHAMBERS: COR TRIATRIATUM SINISTER WITH ATRIAL FIBRILLATION

Cor triatriatum sinister (CTS) is a rare congenital defect in which the left atrium is divided into two chambers by a fenestrated fibromuscular septum. It is found in less than 0.1% of clinically diagnosed cardiomyopathy and 0.4% of autopsies of congenital heart disease patients. Dyspnea was reported as the most common symptom and atrial fibrillation (AF) was described in 30% of the published cases of CTS. We report a young patient with CTS who had recurrent paroxysmal atrial fibrillation (PAF) that underwent standard radiofrequency (RF) pulmonary vein isolation (PVI). 1. To recognize the relation of a rare congenital anomaly to AF 2. Effectiveness of standard PVI in CTS with AF NA A 39-year-old male was referred for symptomatic PAF with CHAD2VASc score of 3. Medical history was significant for CTS, tachybrady syndrome status post leadless pacemaker, diabetes, hypertension, HFpEF, and sleep apnea on CPAP. Pertinent medications were rivaroxaban, diltiazem and metoprolol. AF burden could not be determined. Echocardiogram revealed LA volume of 119 ml, CTS with volume index 52ml/m2 with an opening of 0.8 cm2 and moderate mitral regurgitation with valve area 2.9cm2, PHT 77 ms. Right heart cath showed PAP 50/30 mmHg, PCWP 30 mmHg and PVR 0.8 Woods unit. Cardiac index was 3.5L/min/m2. Despite optimization of rate control therapy and DC cardioversion, the patient had recurrent debilitating AF and decided to undergo RF AF ablation. Intracardiac echo showed the membrane to be attached to the posterolateral ridge laterally and to the interatrial septum medially. Voltage mapping revealed a healthy posterior wall. PVI was achieved successfully with first pass ablation. Patient maintained sinus rhythm at the 3 months follow up. CTS was first described in 1868 by Church. Development of AF from CTS in adults can be related to calcification of the membrane fenestrations leading to mitral stenosis and eventual development of mitral regurgitation with aging.

Asymptomatic Coarctation of the Aorta in Adults with Preserved Exercise Capacity: A Case Report.

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.

Anaesthetic management of three Maine Coon cats undergoing hybrid intervention for treatment of cor triatriatum sinister

AbstractThis report describes the anaesthetic management of three Maine Coon cats undergoing hybrid cutting balloon dilatation for treatment of cor triatriatum sinister. In cat 1, cor triatriatum sinister was an incidental finding during routine transthoracic echocardiographic screening. Cats 2 and 3 presented with tachypnoea and underwent preoperative therapy with diuretics and antithrombotics. All cats were premedicated with fentanyl. Anaesthesia was co‐induced with midazolam and either etomidate (cats 1 and 2) or alfaxalone (cat 3). Sevoflurane, a fentanyl continuous‐rate infusion and a dexmedetomidine continuous‐rate infusion were used for maintenance of anaesthesia. Intravenous methadone, epidural morphine and methadone, an intercostal block and a subcutaneous wound splash with local anaesthetics complemented the analgesic protocol. Intraoperative complications included arrhythmias, bleeding, hypotension and hypothermia. Postoperative emergence delirium and haemothorax developed in two and one cat, respectively. All three cats recovered well and were discharged home.

A 3D transesophageal echocardiography-facilitated diagnosis and cryoballoon ablation of paroxysmal atrial fibrillation inapatient with cor triatriatum sinister.

A 3D transesophageal echocardiography-facilitated diagnosis and cryoballoon ablation of paroxysmal atrial fibrillation inapatient with cor triatriatum sinister.

Right Aortic Arch with Isolated Left Subclavian Artery in Association with Double Outlet Right Ventricle and Cor Triatriatum Sinister: Hitherto Unreported Association.

Right Aortic Arch with Isolated Left Subclavian Artery in Association with Double Outlet Right Ventricle and Cor Triatriatum Sinister: Hitherto Unreported Association.

Cor Triatriatum Sinister Presenting as Cardioembolic Stroke in a Young Woman.

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.