Cor Triatriatum Sinister Research Articles

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

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. 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. 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%). 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.

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.

Cardiac Computed Tomography Pictorial Equivalent of Angiographic Gooseneck Sign in a Case of Atrioventricular Septal Defect with Cor Triatriatum Sinister

In the literature, multiple radiological signs of clinically important congenital cardiovascular abnormalities have been described which are named as per the object they resemble. These signs are very useful in learning. The Gooseneck sign is a sign seen in left ventricle catheter angiography in case of an atrioventricular septal defect. This sign has not been demonstrated on a cardiac computed tomography (CT) so far, to the best of our knowledge. We present a case showing cardiac CT pictorial equivalent of angiographic Gooseneck sign.

A Ten Year Retrospective Study on Cor Triatriatum Sinister Associated Heart Failure in Dogs

Background: Although cortriatriatumsinister (CTs) represents a well-known and widely described cardiac malformation in humans, its description in the canine population is rare. The aim of this ten-year study is to place on record about the most unusual congenital cardiac malformation, cortriatriatum sinister that was sporadically reported among veterinary patients. Methods: The dogs were examined clinically, physically and later subjected for chest radiograph and echocardiography to confirm CTs. All were showing regular femoral pulse that was strong and synchronous to the heart beat, pale mucosa, mean systolic arterial blood pressure of 145 mm Hg and with normal hemato-biochemical parameters. Cardiomegaly with increased sternal contact, elevated trachea, pulmonary congestion and engorged caudal vena cava were radiographic findings. 2d-echocardiography revealed a left atrium that was subdivided by a transverse membrane into two distinct compartments, one proximal and one distal, thus confirming the cortriatriatum sinister. Result: Cor triatriatum sinister (CTs) was diagnosed in 4 dogs with a prevalence of 4.59% amongst those suffering from congenital heart anomalies, that were aged between 5y-7y and the breeds include Boxer, Doberman and Cocker spaniel. All these dogs were presented with similar signs suggestive of heart failure viz., exercise intolerance, dyspnoea at rest, cough that was dry nocturnal type, generalised weakness and syncope. All of these patients were successfully managed with frusemide, benazapril and pimobendan. Hence, it may be concluded that the CTS, a rare congenital cardiac anomaly in dogs should also be included in the differential diagnosis of heart failure, particularly if presented at early life, which also helps to prevent further breeding from such stock.

A case report of atrial fibrillation in early adulthood: dig deeper

BackgroundAtrial fibrillation (AF) is a common cardiac arrhythmia, which is often associated with underlying risk factors and undiagnosed conditions, including congenital heart disease. Atrial septal defects (ASDs) come to mind, albeit arrhythmias usually present later in life. We present herewith a young patient with cor triatriatum sinister (CTS), with some delay in establishing the diagnosis, following new onset AF in early adulthood.Case summaryA 31-year-old man presented with pre-syncope and coryzal symptoms and was newly diagnosed with AF in the context of an intercurrent viral illness. After treatment with oral anticoagulation and successful outpatient cardioversion, he was discharged from cardiology review. Two years later he re-presented with exercise intolerance and a 12-lead electrocardiogram revealing recurrence of AF. Subsequent investigation with transthoracic echocardiography revealed the underlying congenital cardiac defect of CTS, together with an ASD and patent foramen ovale. After corrective surgery, which involved membrane resection, a Cox-maze procedure and ASD closure, sinus rhythm was restored and at follow-up the patient had returned to baseline function.DiscussionYoung patients presenting with new onset AF should undergo thorough cardiovascular assessment to identify treatable causes and reversible risk factors. Cor triatriatum sinister is a rare congenital anomaly that may present in adulthood and give rise to symptomatic AF. Surgical correction including a Cox-maze procedure in our patient resulted in restoration of sinus rhythm and a return of the patient’s baseline functional status and improved quality of life.

Coexistence of Cor Triatriatum Sinister, Fibroelastoma and Pulmonary Veins Ostial Anatomy Variant as Incidental Findings in Coronary Computed Tomography Angiography.

Coronary computed tomography angiography (CCTA) is a noninvasive examination whose main purpose is to exclude significant stenosis in the coronary arteries. The obtained computed tomography images may also provide information about other coexisting pathologies of the heart and vessels. The paper presents images of cardiac lesions in a 44-year-old hypertensive patient who underwent CCTA, based on which significant stenosis in the coronary arteries was excluded, the suspicion of a cor triatriatum sinister was confirmed and the presence of fibroelastoma and a variant of the anatomy of the pulmonary veins ostial was confirmed. To sum up, when performing CCTA, apart from the analysis of the coronary arteries, one should remember about lesions in the remaining visible anatomical structures of the heart and large vessels.

Correspondence on Surgical Outcomes of Cor Triatriatum Sinister.

Correspondence on Surgical Outcomes of Cor Triatriatum Sinister.

Treatment of non-restrictive cor triatriatum sinister during concomitant cardiac surgery

BackgroundCor triatriatum is a rare congenital heart disease representing the 0.4% of all congenital cardiac anomalies. To date, no specific genetic alteration has been associated to cor triatriatum. The left-sided presentation (cor triatriatum sinister (CTS)) generally consists in a fibromuscular membrane that divides the left atrium into two chambers, therefore generating a varying grade of flow obstruction depending on the shape, location, and membrane fenestration size. Cor triatriatum sinister can be isolated or associated to other congenital heart defects such as ostium secundum atrial septal defect, patent foramen ovale or abnormal pulmonary veins drainage.Case presentationOur case is a 63-year-old woman who was diagnosed with a non-restrictive membrane during a hospitalization for acute heart failure. In the following 6 months, she started to become symptomatic. However, the onset of symptoms was more likely related to mitral valve regurgitation worsening and previously unknown coronary artery disease, rather than to CTS. She underwent bi-atrial surgical ablation (Cox Maze IV procedure) for atrial fibrillation (AF), surgical resection of interatrial membrane with mitral annuloplasty, and myocardial revascularization.ConclusionThe onset and severity of symptoms in patients with CTS mostly depend on membrane fenestration size, grade of stenosis generated and pulmonary veins drainage site. However, some cases may remain asymptomatic until adulthood; the degree of pulmonary hypertension and congestive heart failure is determined by the presence of additional cardiac anomalies and the fibromuscular membrane fenestration. In some cases, CTS may remain asymptomatic, thus the diagnosis can be incidental.

Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience.

BackgroundWe investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS).MethodsThirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed.ResultsThe median age and body weight at operation were 9 months (interquartile range [IQR], 3–238 months) and 7.5 kg (IQR, 5.8–49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39–195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6–112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg).ConclusionSurgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

A six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect

BackgroundCor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers.Case presentationHere, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair.ConclusionsThe association of CTS with DCRV forming a “6-chambered heart” is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair.

Cor triatriatum sinister as an incidental finding in elderly woman

Cor triatriatum is a rare condition. It comprises around 0.1% to 0.4% of all congenital heart malformations. There are two types of this anomaly: cor triatriatum sinister (CTS), which is more common and accounts for 92% of all cases, and cor triatriatum dexter (CTD). This condition usually presents at an early age and is diagnosed mainly during early childhood. In some patients, who have less severe cases of CTS, diagnosis could be made in adulthood. We report an unusual case of a 78-year-old woman who was diagnosed with CTS for the first time. The patient was admitted to the cardiology department with symptoms of chest pain and dyspnoea. Physical examination revealed diffusely diminished breath sounds, with focal wheezing, and irregular heart rate, with no murmurs, while blood pressure was 140/90 mmHg. A transthoracic echocardiogram revealed an accessory membrane in the left atria suggestive of CTS.

Abstract 12878: Left Atrial Labyrinth in Fetal Hypoplastic Left Heart Syndrome is Associated With Worse Prognosis

Introduction: Hypoplastic left heart syndrome (HLHS) with restrictive atrial septum (RAS) is associated with poor outcomes. A subset of patients with HLHS have cor-triatriatum sinister, which often presents as a complex network of folds within the LA, which we referred to as LA-labyrinth (Figure 1). Hypothesis: We hypothesize that LA labyrinth is associated with worse clinical outcomes among those with a RAS compared to patients without LA-labyrinth. Methods: In a single-center retrospective cohort study, all fetuses with HLHS (2010-2020) were included. Fetuses with inadequate images, no follow-up data, pregnancy termination, or postnatal comfort care were excluded. All fetal echocardiograms were reviewed. RAS was defined as a ratio of forward/reverse pulmonary vein velocity time integral (PV-VTI) < 5. Kaplan-Meier analysis from 20 weeks’ gestation was performed to evaluate freedom from the primary outcome (fetal demise, postnatal death, or transplant) by 1-year postnatal life (72 weeks total). Results: Of 241 fetuses with HLHS, 179 met inclusion criteria. RAS was present in 43 (24%), and LA-labyrinth was seen in 9 (5%), of which 6 also had RAS. Freedom from death/transplant was worse in RAS with LA labyrinth vs. no LA labyrinth and vs. HLHS-unrestrictive atrial septum (Figure 2). Among those with RAS, those with LA labyrinth were more likely to have early atrial septal intervention (4/6 vs 10/34), and to die or need transplant (4/6 vs 12/37, p = 0.022). Conclusions: LA-labyrinth is seen in HLHS with RAS and is associated with worse outcomes than RAS alone. The unique left atrial anatomy may need to be taken into consideration during prenatal counseling and postnatal planning.

Cor Triatriatum Sinistrum: A Rarest Aetiology of Cardiac Chamber Thrombi Mimicking Left Atrial Mass

Background. Cor triatriatum is a rare congenital cardiac anomaly, represent 0.1% of all congenital cardiac malformations and may be associated with other cardiac diseases in as many as 50% of cases. The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chamber. Case Presentation. We reported case of cor triatriatum in a 12 years old girl with chief complaint of shortness of breath, middle chest discomfort and palpitation since 5 days prior admission. The diagnosis was based on clinical features, chest radiography, electrocardiography and transthoracic echocardiography. Chest radiograph showed rounded cardiac apex and double contour appearance. ECG showed sinus rhythm, 75 beat per minute, RAD, CCWR, RVH, RV strain pattern with ST depression and T-wave inversion in II, III, aVF, V1-V5. TTE revealed 2 chambers of left atrium, with restrictive supramitral membrane, dilated right atrium, right ventricle and left atrium, smallish left ventricle, proximal left atrial thrombus (5.96 x 3.44 cm), relative mitral stenosis, severe mitral regurgitation, mild aortic regurgitation and severe tricuspid regurgitation. A diagnosis of cor triatriatum sinister was made. The only treatment is surgical correction. Medical therapy, with ampicillin and heparin, was administered during admission. Conclusion. Cor triatriatum has been reported in a 12-year-old girl. The diagnosis is confirmed by clinical manifestations, chest radiography and echocardiography. The only therapy is surgical correction. From the field of cardiac surgeon, patients are advised to improve their general conditions before underwent surgical procedures.

Catheter ablation of atrial fibrillation in patients with cor triatriatum sinister; case series and review of literature.

Cor triatriatum sinister (CTS) is a rare congenital heart defect characterized by fibromuscular septation of the left atrium associated with atrial fibrillation (AF). The incidence of hemodynamically insignificant CTS in the AF ablation population and effect on ablation success are not known. Furthermore, little is known about the potential effect of CTS on arrhythmogenic substrate. We define the incidence of hemodynamically insignificant CTS in patients undergoing AF ablation with RF and cryoballoon ablation, the technical challenges created by the left atrial partitioning, and the potentially arrhythmogenic effects of the membrane. We also review the literature of CA in patients with CTS. First-time AF ablation cases at our institution over a 10-year period were screened to identify patients with CTS. Retrospective review was performed to obtain clinical characteristics and ablation data. Of the 3953 consecutive patients undergoing initial AF ablation during the study period, four patients (0.10%) had CTS. Ablation was successful acutely in all patients. One patient had recurrent AF and required repeat ablation for a single procedure success rate of 75% and multi-procedure success rate of 100%. The CTS membrane was associated with low voltage zones in the two patients in whom it was measured and with substrate for macro-reentrant atrial tachycardia in one of these patients. The incidence of hemodynamically insignificant CTS in patients undergoing CA for AF is very low, but does not serve as a significant barrier to successful ablation as long as directed access to the superoposterior chamber is obtained.

Katétrová liečba cor triatriatum sinister - kazuistika

Cor triatriatum sinister (CTS) patrĂ­ k zriedkavĂ˝m vrodenĂ˝m srdcovĂ˝m anomĂĄliĂĄm. Zvyčajne je ÄžavĂĄ predsieň rozdelenĂĄ membrĂĄnou, ktorĂĄ brĂĄni prĂ­toku krvi cez pĞúcne Ĺžily k mitrĂĄlnej chlopni a spĂ´sobuje klinickĂŠ prĂ­znaky ako u mitrĂĄlnej stenĂłzy s pĞúcnou kongesciou a pĞúcnou hypertenziou. NajlepĹĄĂ­m spĂ´sobom liečby je chirurgickĂŠ odstrĂĄnenie membrĂĄny, ale je moĹžnĂĄ aj katetrizačnĂĄ intervencia. V naĹĄom prĂ­spevku uvĂĄdzame prĂ­pad pacienta s vrodenou anomĂĄliou CTS, u ktorĂŠho sme ĂşspeĹĄne, katetrizačne balĂłnom dilatovali stenotickĂ˝ otvor v membrĂĄne s priaznivĂ˝m hemodynamickĂ˝m a klinickĂ˝m vĂ˝sledkom. © 2021, ČKS.

Cardiac Anomalies in Two White-Tailed Eagles (Haliaeetus albicilla) in Sweden.

Severe cardiomegaly with an atrial septal defect was discovered during necropsy of a subadult White-tailed Eagle (Haliaeetus albicilla) found dead in the wild. A thin membrane composed of fibromuscular tissue separated the left atrium into two chambers, most consistent with that described for cor triatriatum sinister (CTS) in other species. Seventeen months later, necropsy of an adult White-tailed Eagle again revealed CTS. This lesion has not been reported previously in raptors.

Robotic Surgery for Pediatric and Congenital Cardiac Disease

Onan and Onan are to be congratulated for their thoughtful analysis of 242 patients undergoing robotically assisted congenital cardiac surgery published in this issue of The Annals of Thoracic Surgery.1 Mean age was 30.9 ± 12.1 years (range, 12-65 years). Thirty (12.3%) patients were younger than 18 years. In adolescents, median age was 16 years (range, 12-17 years) and median weight was 64 kg (range, 42-108 kg). Lesions repaired included secundum atrial septal defect (ASD) (74.7%), sinus venous ASD (16.1%), partial anomalous pulmonary venous connections (10.7%), widely patent foramen ovale (3.7%), ostium primum defects (2.5%), unroofed coronary sinus (1.2%), partial atrioventricular canal defects (2.5%), residual septal defects after failed percutaneous closure (1.2%), ventricular septal defect (0.4%), cor triatriatum sinister (0.4%), subvalvular aortic stenosis (0.4%), common atrium (0.4%), and double-chambered right ventricle (0.4%).

Cor Triatriatum Sinister in a 25 Year Old Patient - A Case Report

Cor triatriatum sinister is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the left atria into 2 chambers. It is even rarer when present at an adult age. Symptoms and presentation of the patient depend on the size of the opening into the membrane through which 2 chambers of left atrium communicate with each other. Cor triatriatum sinister rarely remains asymptomatic till adulthood. In adults symptoms of Cor triatriatum sinister mimic the symptoms of mitral stenosis. Corrective surgery is the choice of treatment, but when it present as an emergency case especially at an early age, balloon dilatation of the membrane opening is done. Elective balloon dilatation of membrane opening at an early age diagnosed with Cor triatriatum sinister followed by corrective surgery at an adult age can be a safer option compared to direct corrective surgery at an early age. We present a case of a 25-year-old female who was diagnosed first time at this age as cor triatriatum sinister and treated successfully with corrective surgery.

Multimodality Imaging of Cor Triatriatum Sinister in an Adult.

Supplemental material is available for this article.

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.