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

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.

Multimodality Imaging of a Mass Arising from a Cor Triatriatum Sinister Membrane.

Cor triatriatum sinister is a rare entity characterized by a membrane within the left atrium and posterior to the atrial appendage. This defect may cause obstructive symptoms analogous to mitral stenosis. The authors present a case of an incidentally detected enhancing mass originating from a cor triatriatum sinister membrane, with imaging characteristics most suggestive of myxoma. Keywords: MR Imaging, Cardiac, Left Atrium, Congenital, CT Angiography, Echocardiography Supplemental material is available for this article.

Hidden problems in a case of atrial fibrillation.

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

A rare case on anomalous pulmonary venous drainage in congenital cardiovascular disease.

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

Do we really need a new classification for cor triatriatum sinister?

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.

Cor Triatriatum and Intracardiac Anomalous Pulmonary Venous Return: An Inborn Atrial Flow Inversion.

A neonate with a diagnosis of nonobstructive intracardiac type total anomalous pulmonary venous connection presented with profound cyanosis in the first days of life. The preoperative specialist echocardiographic examination also identified the presence of partial cor triatriatum dexter. The anatomic pattern of this exceedingly rare disease's association, its peculiar clinical presentation, and surgical management are discussed.

Cor Triatriatum in Association With a Unique Form of Partial Anomalous Pulmonary Venous Connection.

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.

Cor Triatriatum Dexter: A Rare Cause of Aneurysmal Right Atrium.

Cor triatriatum dexter (CTD) is a rare congenital cardiac anomaly with a diverse presentation in every age group. We report a case of CTD in a 36-year-old female who presented with palpitations due to giant right atrium (RA), which we managed successfully with surgical excision of the membrane and RA reduction.

Triatriatum sinistrum em adulto assintomático: relato de caso / Cor triatriatum sinistrum in an asymptomatic adult: a case report

Cor triatriatum é um anomalia cardíaca congênita rara frequentemente diagnosticada na primeira infância. Este estudo de caso apresenta um adulto com um achado acidental de cor triatriatum sinistrum. Com base na apresentação clínica, o paciente foi tratado de forma conservadora. São apresentados achados de imagens ecocardiográficas de cor triatriatum sinistrum deste paciente juntamente de revisão narrativa da literatura sobre essa doença.(AU)

Cor triatriatum is a rare congenital heart anomaly often diagnosed in early childhood. This case study features an adult with an incidental finding of cor triatriatum sinistrum. Based on the clinical presentation, the patient was treated conservatively. Cor triatriatum sinistrum echocardiographic image findings of this patient are presented along with a narrative review of the literature about this disease. (AU)

Cor triatriatum in adulthood with mitral valve regurgitation and atrial fibrillation.

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.

Cor triatriatum sinister with left anomalous pulmonary venous drainage to innominate vein: what to do with the vertical vein?

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.

Cor Triatriatum Sinister (Divided Left Atrium): Histopathologic Features and Clinical Management.

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.

Combined interventional procedure and cardiopulmonary bypass surgery in a dog with cor triatriatum dexter, patent foramen ovale, and pulmonary stenosis.

A 2-year-old American Pit Bull dog was presented for surgical evaluation of imperforate cor triatriatum dexter (CTD) and patent foramen ovale (PFO). Echocardiography identified an imperforate CTD associated with a right-to-left shunting PFO and valvular pulmonary stenosis. A 2-step interventional and surgical approach was used. Initially, a pulmonary balloon valvuloplasty was performed, and subsequently the dog underwent a surgical correction of the atrial anomaly under cardiopulmonary bypass.

Acquired Cor Triatriatum Sinister After the Fontan Operation and Successful Resection in a Child.

Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium. We describe the first case of acquired cor triatriatum late after the Fontan procedure with successful surgical resection in a child.

Thrombosed cor triatriatum sinistrum mimicking left atrial mass and causing unilateral pulmonary edema.

46 year old female with history of progressive shortness of breath for 3 years associated with recurrent right lung infiltrates and hemoptisis. A computed tomography of the chest showed a left atrial mass suggestive of atrial myxoma confirmed with transesophageal echocardiogram. Contrary with findings from a dedicated EKG gated cardiac computed tomography suggestive of cor triatriatum with associated thrombus and less likely a neoplasm; findings later on confirmed during surgery. Cardiac CT offers superior spatial resolution and multi-planar image reconstructions, allowing improved characterization of cardiac structures and cardiac masses compared to other traditional cardiovascular imaging modalities.