Cor Triatriatum 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

A Case of A Middle-Aged Woman with Cor Triatriatum Dexter and Sick Sinus Syndrome: Comprehensive Cardiovascular Evaluation

This case study discusses a 51-year-old woman with a complex cardiovascular condition, cor triatriatum dexter, complicated by sick sinus syndrome. The patient presented with a history of bradycardia, dizziness, and amaurosis, and was admitted to the emergency department due to numbness in her right limb. Diagnostic examinations, including computed tomography (CT) and cardiac color-ultrasound screening, revealed cor triatriatum dexter along with an enlarged left atrium and ventricle. Additional findings included the absence of the inferior vena cava and polysplenic syndrome. Based on these results, double-chamber pacemaker surgery was recommended, supported by cardiac and thoracic-abdominal CT angiography and three-dimensional vascular reconstruction. This case underscores the importance of comprehensive examinations in identifying associated cardiovascular abnormalities.

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

Saline agitated echocardiography in the diagnosis of cor triatriatum dexter: Case series and literature review.

Saline agitated echocardiography in the diagnosis of cor triatriatum dexter: Case series and literature review.

Minimally Invasive Surgical Repair of Simple Congenital Heart Defects Using the Right Vertical Infra-Axillary Thoracotomy Approach.

To evaluate the safety and efficacy of surgical repair for patients diagnosed with simple congenital heart defects (CHD) using the minimally invasive right vertical infra-axillary minithoracotomy (RVIAT) approach. We retrospectively reviewed the clinical data of consecutive patients who underwent minimally invasive RVIAT for repair of CHD between August 2019 and August 2022. There were 382 patients who underwent 8 primary procedures and were included in this study. The median age of the patients was 16.2 (interquartile range [IQR], 7.2 to 41.9) months, and the median weight of the patients was 8.8 (IQR, 6.5 to 14) kg. The preoperative diagnoses were as follows: ventricular septal defect, atrial septal defect, partial anomalous pulmonary venous return, partial atrioventricular septal defect, cor triatriatum, complete atrioventricular septal defect, and myxoma. The mean aortic cross-clamp time, bypass time, and operation time were 45.4 ± 19.3 min, 65.6 ± 23.1 min, and 154.5 ± 29.7 min, respectively. There was no in-hospital mortality or conversion to median sternotomy. Two patients (0.5%) required early reoperation; 1 due to postoperative bleeding and 1 for permanent pacemaker implantation. Other complications included trivial residual shunts (23 of 382, 6%), pleural effusion (3 of 382, 0.8%), pneumothorax (0.8%), and wound infection (4 of 382, 1%). There were 2 late noncardiac deaths. Late reoperation was performed on 1 patient with progressive aortic valve regurgitation who required aortic valvuloplasty. RVIAT is a minimally invasive approach that can be safely performed on patients with simple CHDs. RVIAT may be a good alternative approach for median sternotomy and cardiac intervention.

Cor Triatriatum Dexter. Role of Echocardiography, Computed Tomography, and Magnetic Resonance Imaging in Its Diagnosis

Cor triatriatum dexter is a rare congenital heart anomaly in which the right atrium is divided into 2 chambers by a membrane. Clinical manifestations vary depending on the degree of the right atrium separation and the presence of concomitant anomalies. We report a case of asymptomatic cor triatriatum dexter diagnosed during transthoracic echocardiography. The diagnosis was confirmed by transesophageal echocardiography, computed tomography, and magnetic resonance imaging. Transthoracic echocardiography is a sensitive primary diagnostic tool for such congenital heart disease as cor triatriatum dexter.

Early outcomes of minimal invasive surgery via the axillary approach for complex congenital heart disease at the Cardiovascular Center - E hospital

Objective: This study aims to outline the initial outcomes of implementing minimally invasive surgery via the right axillary approach to completely repair complex congenital heart diseases. Summary: Minimally invasive surgical techniques have gained widespread adoption in managing simple congenital heart diseases such as atrial septal defect, ventricular septal defect, partial atrioventricular septal defect, cor triatriatum, and partial anomalous pulmonary venous return. Postoperative results have demonstrated equivalence, if not superiority, to traditional sternotomy procedures while offering enhanced cosmetic benefits. However, for more intricate congenital heart conditions such as tetralogy of Fallot (TOF), complete atrioventricular septal defect (CAVSD), and Ebstein syndrome, this study evaluates the early outcomes of employing minimally invasive surgery via the right axillary approach, a technique that remains relatively uncommon globally. Our study seeks to assess the initial outcomes of this approach in managing complex congenital heart diseases. Subjects and methods: descriptive method based on a series of cases. From April 2023 to December 2023, 45 patients diagnosed with complex congenital heart disease underwent minimally invasive surgery utilizing the axillary approach. This cohort comprised 40 patients with tetralogy of Fallot, 3 patients with complete atrioventricular septal defect, and 2 patients with Ebstein syndrome. Results: All procedures were technically successful, without requiring sternotomy. The cardiopulmonary bypass times for the TOF, complete AVSD, and Ebstein syndrome groups were 81 minutes, 91 minutes, 96 minutes, respectively. Corresponding aortic cross - clamp times were 70 minutes, 72 minutes, and 74 minutes. There were no postoperative fatalities or significant complications. Postoperative echocardiographic evaluations demonstrated favorable outcomes. Conclusion: Minimally invasive surgery via the axillary approach for managing complex congenital heart diseases yields promising early results and offers notable cosmetic advantages.

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.

The role of natural compensatory mechanisms in complex congenital heart disease: haemodynamic evolution and clinical outcomes in a case of Cor triatriatum sinistrum syndrome.

The role of natural compensatory mechanisms in complex congenital heart disease: haemodynamic evolution and clinical outcomes in a case of Cor triatriatum sinistrum syndrome.

A Pediatric Cardiology Outreach Clinic in a Local Hospital: An Experience Review from South A'Sharqiyah, Oman.

Specialized pediatric cardiology clinics conducted in local hospitals are an important part of delivering specialized care to patients close to their homes. This study aimed to review our experience with a specialized pediatric cardiology outreach clinic at Jaalan Bani Bu Ali Hospital, South A'Sharqiyah, Oman. Patient records for each individual, seen in the outreach clinic between March 2018 and June 2022, were reviewed to determine demographic information, reason for referral, underlying diagnosis, and clinic visit outcomes. Over the study period, 29 clinics were conducted, with 360 patients seen. Of these, 200 (55.6%) were male with a median age of 13 months. The majority of patients (n = 271; 75.3%) were referred due to a cardiac murmur. Most patients had a normal cardiac evaluation (n = 177; 49.2%). The most common congenital heart diseases detected were mild pulmonary valve stenosis (14.8%) and moderate to large secundum atrial septal defects (13.7%). Significant cardiac lesions detected included severe pulmonary hypertension (2.2%), tetralogy of Fallot (1.6%), and cor triatriatum sinistrum (0.5%). Overall, 70 (19.4%) patients were referred to tertiary care hospitals, and 179 (49.7%) were reassured and discharged. Conducting specialized pediatric cardiology outreach clinics in overpopulated areas is effective and well-received by families. It reassures many families and reduces the need for unnecessary travel to specialized centers. These clinics also play a crucial role in detecting patients with significant cardiac defects requiring urgent care. Implementing specialized clinics in primary and secondary health centers could be beneficial for other subspecialties in reducing long waiting lists.

Ebstein anomaly, right-to-left atrial septal defect, and cor triatriatum dexter in a cat: a case report

A 6-month-old male Ragdoll cat presented with exercise intolerance. On physical examination, there was a grade 2/6 systolic murmur at the right apex. Diagnostic tests, including SpO2 measurement, blood tests, radiography, echocardiography, contrast echocardiography, and electrocardiography, were performed. Severe right atrial dilation, tricuspid valve leaflets and orifice displacement, right ventricular atrialization, septal leaflet adherence, anterior leaflet tethering, and right atrioventricular junction dilation were noted on echocardiography, alongside a right-to-left atrial septal defect. Cor triatriatum dexter and left ventricular aneurysm were observed. We diagnosed this case as having Ebstein anomaly with rare congenital heart deformities; which is rare in cats.

Staged Norwood operation for patients with hypoplastic left heart syndrome and cor triatriatum: a report of two cases

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

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.

Navigating rare complex heart anatomy: percutaneous left atrial appendage closure in a patient with triatrial heart.

Navigating rare complex heart anatomy: percutaneous left atrial appendage closure in a patient with triatrial heart.

Clinical Case of Cor Triatriatum

Cor Triatriatum is utterly rare congenital heart disease, which has characteristic magnetic resonance and echocardiographic symptoms. Complexity of the diagnostics is an asymptomatic clinical flow, so timely diagnostics and therapy will help to defer possible complications and improve the quality of the life of these patients.

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.

Abstract 14201: Double the Trouble: Extremely Rare Incidental Finding of a Double Interatrial Septum With Atrial Septal Aneurysm and Coexisting Patent Foramen Ovale

Case: A 59-year-old&amp;nbsp;female with a past medical history of hypothyroidism and IgG deficiency presents to cardiology clinic due to palpitations and premature ventricular complexes (PVCs) noted at a recent urgent care visit. In-office vitals, physical exam, labs, and EKG were unremarkable. Ambulatory cardiac monitoring showed predominantly sinus rhythm with a PVC burden of 1.4%. Echocardiogram revealed an extremely rare structural anomaly – a double interatrial septum (DIAS) with atrial septal aneurysm. Cardiac MRI (cMRI) further confirmed the presence of a DIAS enclosing an interatrial space, along with blood flow within the interatrial space and a discontinuity in the anterior right lateral atrial septum suggestive of a patent foramen ovale (PFO). There was no evidence of thrombus within the interatrial or intracardiac spaces. Mild right atrial enlargement was seen. The patient was initiated on aspirin for cardioembolic stroke prophylaxis. Discussion: DIAS is a highly rare congenital cardiac anomaly with limited number of cases in literature. It has been described as a midline third atrium resulting in a “tri-atrial heart”. As of 2018, less than 20 cases have been cited. DIAS is characterized by two parallel interatrial septa along with a clear echo lucent midline space that expands in systole and collapses in diastole. This case is unique as it is one of the few to include additional characterization with cMRI. Patients with DIAS are usually asymptomatic but can experience cardioembolic events as the interatrial space is at risk for thrombus formation. Communication across the DIAS makes these events more likely. Unlike with atrial fibrillation/flutter, no current criteria for anticoagulation (AC) initiation exists for DIAS given the scarcity of cases. While some sources advocate for use of anti-platelet agents, others have indicated a preference for oral AC. Our patient had an interatrial space and a PFO but lacked communication across the DIAS, had low bleeding risk, and no other classic risk factors of Virchow’s triad. We determined her risk for thrombus formation to be relatively low, and thus initiated her on aspirin over oral AC. Percutaneous closure of the interatrial space was deferred as it has been dismissed as a viable treatment option in the literature.