Divided Left Atrium Research Articles

Is it really a levoatrial cardinal vein?

With the advent of computed tomographic interrogation, it is increasingly frequent to find venous channels that provide direct connections between the pulmonary and systemic veins. These channels, before the introduction of three-dimensional techniques for clinical imaging, were usually found providing an "overflow" for the obstructed left atrium in settings such as hypoplastic left heart syndrome, or divided left atrium. Similar channels, however, had been described almost 100 years ago, with one accurately described as a jugulo-pulmonary vein. Nowadays, however, it is much more usual to find the channels described as levoatrial cardinal veins, even though it is recognized that they are not "levo," often not "atrial," and for sure not "cardinal." In this review, we assemble the evidence supporting the notion that they are better considered as pulmonary-to-systemic collateral channels. We emphasize their similarity, in terms of development, to the sinus venosus and coronary sinus defects.

Cor triatriatrum or divided left atrium presenting as mitral stenosis in an adult patient

Cor triatriatrum or divided left atrium presenting as mitral stenosis in an adult patient

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

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. 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. 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. 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.

Anomalous Left Coronary Artery Connected to the Pulmonary Artery Associated With Other Cardiac Defects: A Difficult Joint Diagnosis

Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) can be associated rarely with other congenital heart defects. The preoperative joint diagnosis is challenging. From 1987 to 2012, a retrospective bicentric assessment of 12 patients with ALCAPA related to other cardiac defects focused on the associated heart defect, the moment of complete diagnosis related to surgery, and outcome. Coarctation was the most frequently associated heart defect (n = 5) followed by tetralogy of Fallot with or without pulmonary atresia (n = 3). The study group comprised one case of hypoplastic left heart syndrome, one right aortic arch, one congenital mitral malformation, and one infant with divided left atrium and anomalous pulmonary venous return. Only four patients had a complete diagnosis of both the cardiac defect and the coronary abnormality before surgery. In two cases, the coronary anomaly was discovered during surgery performed for another cardiac defect and treated at the same time. The diagnosis of the six remaining patients was determined after cardiac repair. Of the 12 patients, 7 (58 %) died after surgery. Half of these patients died within the first 30 days after repair. At this writing, the remaining patients are in good health after a median follow-up period of 5.4 years (range, 2.1-8.5 years). This study confirmed that ALCAPA associated with other cardiac defects often is misdiagnosed before surgery, mostly due to specific hemodynamics masking myocardial ischemia preoperatively. Survival was compromised due to the unrecognized diagnosis of an associated coronary abnormality but also because of midterm complications related to the other cardiac defects.

Hepatic Venous Occlusion During Cardiopulmonary Bypass in Patients With Heterotaxy Syndrome: A Safe but Underutilized Option

Direct intracardiac drainage of separate right and left hepatic veins remote from each other (independent hepatic veins) in heterotaxy patients complicates procedures requiring cardiopulmonary bypass (CPB). Temporary occlusion of such independent hepatic veins during CPB is an alternative to cannulation but is rarely used because of concerns about acute congestive hepatopathy. Consequently, temporary single hepatic venous occlusion has not been well described as a safe and simple alternative to hepatic venous cannulation during CPB. We report 2 patients with the polysplenia variant of heterotaxy in whom independent hepatic veins were safely occluded for 55 and 86 minutes, respectively, in the course of intracardiac repair. Temporary hepatic venous occlusion simplified the CPB technique and minimized clutter of the operative field. The intrahepatic hemodynamics during CPB using temporary hepatic venous occlusion is illustrated.

Divided Left Atrium (Cor Triatriatum) in the Setting of Common Atrium

Cor triatriatum is commonly used to describe the congenital malformation in which an anomalous fibromuscular shelf divides the left atrium. We report the successful surgical repair of an extremely rare combination of divided left atrium, virtual absence of the interatrial septum, and anomalous right pulmonary venous connection in a 7 year-old girl. Preoperative transthoracic echocardiography could not detect the dividing left atrial shelf. In this report, we point out the limitations of transthoracic echocardiography in the diagnosis of atypical cases of so-called cor triatriatum and highlight errors in the scientific literature resulting from the variance in terminology ascribed to congenital division of the left atrium.

Concordant Ventriculoarterial Connections With Parallel Arterial Trunks, Divided Left Atrium, and Juxtaposed Atrial Appendages

We describe a patient with concordant ventriculoarterial connections with parallel arterial trunks, divided left atrium (cor triatriatum sinister), ventricular septal defect, bilateral superior caval veins, and juxtaposed atrial appendages. The aorta was anterior and left sided. We discuss the morphological features of this rare condition, and the diagnostic dilemma it produced.

Cor triatriatum and coronary artery fistula in tetralogy of Fallot

Coexistence of divided left atrium with tetralogy of Fallot is rare. Preoperative diagnosis of this rare association is difficult. We here report preoperative diagnosis of this rare combination. In addition, the patient also had coronary to left ventricle fistula.

An Unusual Presentation of an Atrial Septal Defect

Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies found in the adult population. Most lesions are classified into ostium primum, ostium secundum, sinus venosus, and coronary sinus type by anatomic site of defect. A 38-year-old woman visited the cardiovascular department for further evaluation of ASD diagnosed by screening transthoracic echocardiography (TTE) during peripartum period. She had suffered from mild exertional dyspnea for several years. TTE showed ASD (suspicious sinus venosus type, defect size=0.97 cm), normal left ventricular function with paradoxical septal wall motion, right ventricular and atrial enlargement, mild pulmonary stenosis, and moderate tricuspid regurgitation. Right ventricular systolic pressure was 56 mmHg, so we could confirm moderate degree of pulmonary hypertension. In addition, left atrium looked like divided chamber by abnormal membranous structure (Fig. 1). For precise evaluation of the ASD, we carried out transesophageal echocardiography (TEE). TEE revealed 4.07 cm sized large ASD, so right atrium looked like single large chamber. Furthermore, membrane-like structures were persent in left atrum, so left atrium looked like divided chambers (Fig. 2). Patient underwent operation for ASD one month later, and the operator confirmed that the lesion was large secundum type ASD with anomalous structure in left atrium and performed autopericardial patch closure for large ASD. Follow-up TTE after operation showed membranous structure in left atrium without remnant shunt flow (Fig. 3). In conclusion, this case was thought to be unusually large ASD with membrane in left atrium. Fig. 1 Transthoracic echocardiography showed 0.97 cm sized atrial septal defect suspected to be sinus venosus type. Left atrium looked like divided chambers by abnormal membranous structure. Fig. 2 Transesophageal echocardiography demonstrated 4.07 cm sized large atrial septal defect and membranous structures divided left atrium into plural chambers. Therefore, it made atrial chambers of large right atrium and small left atrium. Fig. 3 Follow-up transthoracic echocardiography showed no remnant atrial septal defect, but left atrium still looked like divided chambers by membranous structure.

Successful surgical treatment of hypoplastic left heart syndrome associated with a divided left atrium that was diagnosed intraoperatively

Hypoplastic left heart syndrome (HLHS) often involves abnormalities in atrial septal morphology, including a restrictive atrial communication and anomalous attachment of a malaligned septum primum. Cor triatriatum is a rare congenital heart abnormality in which the left atrium (LA) is divided into 2 chambers by a diaphragm. Association of cor triatriatum with HLHS is rare, and cor triatriatum is difficult to diagnose preoperatively. We herein describe an unusual case of HLHS with cor triatriatum.

Cor triatriatum associated with severe valvular pulmonary stenosis and patent ductus arteriosus: An unreported constellation

A divided left atrium because of cor triatriatum is a relatively rare cardiac anomaly requiring corrective surgery. Although association of cor triatriatum with different congenital heart diseases had been reported, to our knowledge, its association with severe valvular pulmonary stenosis and patent ductus arteriozus had not been reported previously. In this paper, an asymptomatic case with cor triatriatum, severe valvular pulmonary stenosis and patent ductus arteriosus is defined.

Divided left atrium with obstruction of pulmonary venous return in the setting of hypoplasia of the left heart

We describe an infant with hypoplasia of the left heart diagnosed prenatally who, at birth, had signs of severe pulmonary venous obstruction. Echocardiography indicated normally connecting pulmonary veins, and showed a paradoxical right-to-left shunt across a patent oval foramen. Postmortem examination revealed that the obstruction was due to a divided left atrium, or cor triatriatum sinister, with an imperforate muscular diaphragm separating completely the two components of the divided atrium.

Preoperative diagnosis of co-existing divided left atrium and tetralogy of Fallot

We present the eighth published case of divided left atrium co-existing with tetralogy of Fallot. This is the first report of preoperative echocardiographic diagnosis of this unusual combination of defects. Demonstration of a partition within the left atrium is imperative for successful repair of the combined lesions. We draw attention to the need for careful echocardiography in patients where an obstruction to pulmonary venous drainage would dramatically affect the outcome subsequent to surgical correction.

Aurícula izquierda dividida: resultados quirúrgicos y seguimiento en el cor triatriatum

A divided left atrium because of cor triatriatum is a relatively rare cardiac anomaly requiring corrective surgery. We describe here our clinical and surgical experience with this congenital heart defect as well as the different medium and long term diagnostic, surgical and evolution aspects. From 1981 to 1999, 15 children with cor triatriatum without complex associated cardiovascular defects underwent surgery at a mean age of 13 months (excision of the obstructive membrane). The surgical reports were reviewed and the clinical and echocardiographic data were analyzed before and after the intervention; six of these patients (40%) were referred to operating room only with the 2-D echo Doppler technique and color flow mapping information. The follow-up period ranged from 8 months to 19.3 years. Diagnosis was confirmed during the surgical procedure. One 9 month old patient died 60 days after a successful corrective surgery because of sepsis (7%). No late deaths or reoperations were found in the follow-up period. All 14 patients who survived the operation have a functional class I (NYHA), and they are asymptomatic in the follow-up. The overall survival rate was 93% (70% CI: 87-90). Corrective surgery with excision of the obstructive membrane dividing the left atrium restores normal anatomic, hemodynamic and clinical status in children with cor triatriatum without complex associated defects.

An evolving role of transesophageal echocardiography for the monitoring of interventional catheterization in children.

Transesophageal echocardiography (TEE) is increasingly utilized for the monitoring of interventional cardiac catheterization in children. The initial nondiscriminant use is giving way to a more selective approach. The current role and usefulness of this imaging modality are described. Between 1991 and 1995, 44 patients (mean age 7.4 +/- 5 years, weight 22.8 +/- 14.2 kg) underwent interventional cardiac catheterizations performed under TEE monitoring. The interventional procedures included (1) those involving creation, enlargement, or closure of an interatrial communication; (2) balloon dilatation of stenotic lesions; and (3) occlusion of arterial ducts. Transesophageal echocardiographic monitoring was judged to be either of great value, contributory, or of little use if the interventional procedure and choice of instrument relied heavily upon, was guided, or not affected by the information obtained from the ultrasonic investigation, respectively. Transesophageal echocardiography was judged to be of great value in the monitoring of 18 (40%) procedures involving either the creation, enlargement, or closure of interatrial communications. This included 4 transseptal punctures, 4 blade septectomies for relieving restrictive atrial septal defects (n = 3) and an enlargement of the communication between the superior and inferior chambers of the divided left atrium in cortriatriatum, and 11 closures of a secundum atrial septal defect. Only the ultrasonic monitoring could clearly visualize the interatrial partition to provide real-time guidance on the positioning of the instrument to carry out the invasive procedures. Transesophageal echocardiography provided clear images of the hinge points of the aortic and mitral valves, the dimension of the narrowed pulmonary venous pathway in patients after venous switch operation. This facilitated the choice of the correct size of the balloon for dilatation in 11 patients (24%) and provided immediate postdilatation hemodynamic assessment. As for balloon dilation of subvalvar ventricular outflow tract obstruction (n = 3), aortic coarctation (n = 6), and occlusion of persistent arterial duct (n = 7), TEE offered no obvious advantage over fluoroscopy with angiography. The monitoring was judged to be of little value for the 16 (36%) procedures. Transesophageal echocardiographic monitoring is of great value in interventional procedures involving the atrial septum and enhances interventional procedures involving obstructive lesions of the left heart in children.

Repair of divided left atrium associated with unroofed coronary sinus

AbstractIn a young infant with divided left atrium, unroofed coronary sinus syndrome, and persistent left superior caval vein, the pulmonary venous blood was successfully routed, after atrial septectomy, to the mitral valve by constructing a baffle using the partially detached diaphragm which had initially separated the pulmonary venous and vestibular components of the left atrium. The baffle was augmented with a patch of untreated autologous pericardium.

Divided left atrium (cor triatriatum sinistrum) presenting with syncope

SummarySyncope has never been described as the presenting symptom of divided left atrium (cor triatriatum sinistrum). We have now encountered this mode of presentation in an otherwise asymptomatic patient. An upright tilt test elicited syncope prior to surgical correction, but failed to induce any symptom, even subsequent to infusion of isoproterenol, after surgery.

The role of color-flow mapping in confirming the association of divided left atrium with an atrioventricular septal defect

SummaryWe describe the unusual association of a divided left atrium (cor triatriatum) with an atrioventricular septal defect with separate atrioventricular orifices in an eight-year-old asymptomatic girl. The diagnosis had been made by cross-sectional echocardiography combined with Doppler technology and color-flow mapping. These methods together demonstrate both the anatomic and hemodynamic findings.

Coexistence of divided left atrium (cor triatriatum) and tetralogy of Fallot

The association of a partitioned left atrium (cor triatriatum) and tetralogy of Fallot has been reported a few times in the literature. In all of these cases, there was no clinical evidence of an obstructive lesion in the left heart, raising difficulties in establishing the proper diagnosis. We call attention to this rare combination of anomalies and to the particular morphological presentation of the dividing atrial shelf, which was partially formed by the wall of the left superior caval vein.

Doppler echocardiography in the diagnosis of divided left atrium (cor triatriatum sinister)

A case of congenitally divided left atrium detected on cross-sectional echocardiography is described. Simultaneous Doppler examination helped to diagnose the obstructive nature of the partition within the left atrium by locating a turbulent high velocity (2.2 meters/second) diastolic signal. The diagnosis was confirmed on cardiac catheterization and surgery.