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 diagnosed in a patient with postoperative cerebral infarction after beach chair position: A case report.

Cor triatriatum sinister is a rare congenital heart disease in which the left atrium is divided into two compartments by a fibromuscular membrane. In most cases, its symptoms appear in childhood, and it is rarely diagnosed in adulthood. Patients with cor triatriatum sinister are more prone to neurological diseases, such as cerebral infarction. Herein, we report the case of a patient whose cor triatriatum sinister went undiagnosed in the preoperative evaluation, but was diagnosed whilst investigating a cerebral infarction that occurred following a surgery in the beach chair position. It highlights the potential complications in otherwise healthy asymptomatic patients undergoing surgery in the beach chair position. Additionally, in high-risk patients, the provision of clear communication, in advance, regarding potential complications and their management, may reduce the patient's morbidity.

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.

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.

Adult Obstructive Cor Triatriatum with Severe Mitral Regurgitation: A Case Report.

Adult cor triatriatum sinister associated with severe mitral regurgitation is extremely rare. As these obstructive cor triatriatum feature hemodynamics that mimic mitral stenosis, a pressure load is theoretically generated only on the left atrial proximal chamber, and therefore the left ventricle is less likely to suffer volume loading. Here, we report a surgical case with such rare hemodynamics. A 22-year-old man with obstructive cor triatriatum and severe mitral regurgitation received an anomalous membrane excision and mitral annuloplasty. An abnormal membrane with an orifice 7 mm in size was completely resected while a grossly dilated mitral annulus was repaired via annuloplasty ring. Mitral regurgitation was controlled well, and the postoperative course was uneventful. Even with obstructive cor triatriatum, severe mitral annular dilatation and subsequent left ventricular dilatation may occur, causing the progressive heart failure encountered in this case.

Mid-term outcomes of cor triatriatum repair: comparison of biventricular physiology and univentricular physiology.

BACKGROUND: Cor triatriatum is the rarest of all congenital cardiac diseases, accounting for 0.1-0.4% of congenital heart diseases. Atrial septal defect is the most common associated defect; however, cor triatriatum is sometimes associated with univentricular heart. METHODS: This single-centre retrospective study involved all patients who underwent the repair of cor triatriatum at Kobe Children's Hospital between 2000 and 2020. Twenty-four patients were required surgery. We conducted a survey of survival rate, early and late pulmonary vein stenosis in each group. RESULTS: The survival rate of 5 years after cor triatriatum resection was 100% in the biventricular group and 82.1% in the univentricular group, respectively. The free rate for pulmonary stenosis of 5 years after surgery was 100% in the biventricular group and 90.0% in the univentricular group, respectively. There was no statistical difference in survival rate and 5 years free rate for pulmonary stenosis after surgery. CONCLUSIONS: The results showed that surgical correction offers good early and mid-term outcomes for both cor triatriatum with biventricular and univentricular physiologies.

Cor triatriatum with Raghib complex in partial atrioventricular septal defect and common atrium: a rare combination.

Persistent left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect (Raghib complex) is an uncommon anomaly of systemic venous drainage. We present a unique case of simultaneous presentation of cor triatriatum and persistent left superior vena cava draining into the left atrium in an adult female with partial AV canal and common atrium. Complex intra-atrial baffling including a procedure to redirect flow from a proximal atrial chamber was successful.

Cor triatriatum dexter in 17 dogs.

OBJECTIVES: The objective of this study was to report the signalment, presentation, clinical and imaging findings, interventions, and outcomes in a group of dogs with cor triatriatum dexter (CTD). ANIMALS: Seventeen client-owned dogs. METHODS: Medical records were reviewed retrospectively for signalment, history, physical examination findings, imaging and diagnostic findings, presence of concurrent congenital cardiac defects, description of interventional procedures, therapy information, and outcomes. RESULTS: Age at presentation ranged from two to 110 months, with 10 of 17 dogs (59%) aged <12 months. There was an equal distribution between the sexes. Peritoneal effusion was the most common presenting complaint, in 10 of 17 dogs (59%). The CTD was an isolated finding in 3 of 17 dogs (18%); the remaining 14 of 17 (82%) dogs had concurrent cardiac disease, with congenital anomalies present in 12 of 17 (70%). All except one of these 12 dogs had at least one additional condition affecting the right heart. Tricuspid valve dysplasia was the most common congenital comorbidity, present in 9 of 17 dogs (53%). Seven dogs (41%) underwent interventional treatment of their CTD. In 7 of 17 (41%) cases, the CTD was considered to be incidental and the dogs were asymptomatic; therefore, no interventions were performed. The remaining three cases were euthanized or lost to follow-up. CONCLUSIONS: Cor triatriatum dexter in dogs is commonly seen in association with other right-sided congenital cardiac anomalies and may be an incidental finding. Dogs with CTD obstructing right atrial inflow can have a good outcome after intervention. Dogs with no clinical signs associated with the CTD may remain asymptomatic into adulthood.

Cor triatriatum sinister with left anomalous pulmonary venous return to innominate vein.

Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.

Outcomes in patients with cor triatriatum sinister.

OBJECTIVE: To describe outcomes in patients with cor triatriatum sinister (CTS). DESIGN: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization. RESULTS: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of 57 (58%) patients had additional congenital heart disease (CHD) diagnosis. A total of 27 (47%) patients required surgical resection of CTS membrane during median follow-up of 76 months, and these patients were younger at the time of CTS diagnosis (26 vs 41, P = 0.01) and more likely to have associated CHD (55% vs 45%, P = 0.02). There was one perioperative mortality and no late mortality. There was no recurrence of CTS membrane obstruction in the patients that underwent surgery. Similarly there was no significant increase in CTS membrane gradient in the patients that were managed conservatively. CONCLUSIONS: The natural history of CTS is stability without progressive left atrial obstruction, especially in patients with isolated CTS and in those with initial CTS diagnosis made in adulthood. In patients requiring surgical membrane resection due to flow obstruction, surgery is safe and effective with very low risk of recurrence.

Cor triatriatum sinistrum: presentation of syncope and atrial tachycardia.

We present a rather unusual cause for syncope associated with atrial tachycardia. A man aged 39 years presented with an episode of syncope and narrow complex tachycardia. Further investigations, including transoesophageal echocardiography, identified cor triatriatum sinistrum (CTS), a rare congenital abnormality characterised by the atrium being divided by a fibrous membrane. Although it is rare, there has been an increase in diagnosis due to developments in diagnostic imaging techniques. Symptoms are related to the size of fenestrations within the fibrous membrane. Presenting symptoms can mimic those seen in mitral stenosis. It is a condition that can occur in isolation, but it can also be associated with other cardiac abnormalities such as an atrial septal defect (ASD) (as in this case). Surgery is the definitive treatment (this man had surgical repair of CTS and closure of ASD) and should be considered at any age if there are any associated symptoms or complications.

Imperforated cor triatriatum dexter in a dog with concurrent caudal vena cava wall mineralization.

BACKGROUND: Cor triatriatum dexter (CTD) is a rare congenital cardiac malformation with various manifestations and has been sporadically described in dogs. Clinically the dogs present with nonspecific signs of right heart failure or Budd-Chiari-like syndrome. Other associated concurrent cardiovascular anomalies are commonly reported. Diagnosis and full characterization of this complex malformation requires careful investigation and often a multimodal imaging approach. CASE PRESENTATION: A 10-week-old, male intact, Golden Retriever was presented with clinical signs of stunted growth, anorexia, and progressive ascites. CTD imperforate with sole separation of the caudal vena cava (CdVC) and concurrent venous wall mineralization was conjointly diagnosed and fully characterized by echocardiography, non-selective angiography, computed tomography angiography and cardiac magnetic resonance imaging (MRI). This was successfully treated surgically and the dog returned to normal activity. CONCLUSION: To the author's knowledge, this is the first case of CTD imperforate separating the CdVC from the right atrium (RA) with presumed secondary CdCV wall and hepatic parenchyma mineralization reported in a dog. CTD is an important and potentially correctable cause for the development of ascites in a young puppy. Accurate diagnosis of this complex cardiac anomaly is important for selection of the most appropriate curative treatment option.

Catheter-Based Palliation in an Infant With Obstructed Cor Triatriatum.

A 33-day-old infant with obstructed cor triatriatum sinister and partial anomalous pulmonary venous drainage presented with respiratory distress and fever. Her suprasystemic pulmonary hypertension was relieved by opening the connection to the right atrium using balloon atrial septoplasty and septostomy, and to the inferior chamber using balloon dilation of a fenestration in the dividing membrane. This enabled extubation and discharge, with elective surgical repair at 2 months. To our knowledge, this is the youngest patient to receive a catheter intervention for obstructed cor triatriatum sinister, providing relief of pulmonary hypertension and postponement of surgical repair.

[Cor Triatriatum Diagnosed in Adulthood;Report of a Case].

A 64-years-old man had cor triatriatum (Lucas-Schmidt type I A) with severe mitral regurgitation and atrial fibrillation. We perfomed resection of the anomalous septum between the accessory chamber and left atrium, and conducted mitral annuloplasty and maze procedure. Arrhythmia were not encountered after surgery. The maze procedure and resection of the anomalous septum with mitral surgery proved to be effective for atrial fibrillation with cor triatriatum.

Unique combination of atrioventricular septal defect with cor triatriatum and complete vascular ring.

Atrioventricular septal defect can present with one or more associated anomalies. Cor triatriatum (subdivided left atrium) and vascular rings are among the less frequent. We describe a two-month-old patient with these three cardiovascular anomalies. This case highlights the importance of exhaustive preoperative evaluation in order to achieve successful surgical correction in one stage.