Cor triatriatum dexter: an uncommon cause of neonatal cyanosis.

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.

INTRODUCCIÓN: El cor triatriatum dexter es una cardiopatía muy rara, caracterizada por la división parcial del atrio derecho en dos cavidades por la persistencia de una membrana que embriológicamente representa la valva derecha del seno venoso. CASO CLÍNICO: En este reporte de caso, presentamos el caso de un neonato en su día 7 de vida que acude a valoración por presentar desaturación persistente con cianosis al llanto. El diagnóstico se realizó con ecocardiograma posterior al cual se decidió la resección quirúrgica de la membrana, procedimiento que fue llevado a cabo el día 14 de vida con éxito sin complicaciones. CONCLUSIONES: La importancia de este caso clínico radica en la identificación de una cardiopatía rara como causa de cianosis y desaturación en un paciente en etapa neonatal, el cual no presentaba datos de compromiso hemodinámico. También se muestra como un diagnóstico y tratamiento quirúrgico oportuno permitieron una resolución de los síntomas sin complicaciones futuras.

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.

Transapical balloon dilation of cor triatriatum sinister in a cat.

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.

A Case of Persistent Junctional Reciprocating Tachycardia Coincident With Cor Triatriatum Sinister.

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.

Obstructed Supramitral Inflow: Cor Triatriatum Sinister Presentation in Adulthood.

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.

An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis.

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 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.

Corrected Transposition of Great Arteries with Cor Triatriatum and Atrial Septal Defect-Case Report.

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.

Cor triatriatum sinister: Long-term surgical outcomes in children and a proposal for a new classification.

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.

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 Dexter: An Innocent Bystander.

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.

Transmembrane stent placement for cor triatriatum dexter in six dogs.

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.

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.