Cor Triatrium Research Articles

P110 ACCIDENTAL FINDING OF COR TRIATRIUM SINISTER IN AN ADULT: DIAGNOSIS MANAGMENT

Abstract A 45–year old woman presented at the emergency department for palpitations and fever. The EKG, thoracic x–ray and troponins were normal, only a neutrophil leucocytosis was found. The echocardiography showed a normal bi–ventricular and valvular function and a mobile, linear, mass in the left atrium. In order to exclude an infective vegetation the patient underwent to a 2D and 3D transesophageal echocardiography (Figure 1 and 2) which showed a left ventricle concentric remodelling (iLVmass 62 g/m2; RWT 0.45), preserved left and right ventricle function (LVEF according to Simpson 70%; FAC 50%) and valves function; inter–atrial septum defects were excluded, too. The mass was described as a mobile membrane in the left atrium at 2.11 cm from the mitral valve anular plane, which divided the atrium into two separate chambers with a large fenestration without trans–mitralic flow obstruction, supporting a cor triatrium sinister. We excluded endocarditis (the blood cultures were negative, too) and supra–valvular mitral ring (since the left appendage position in the antero–inferior side of the left atrium). The cardiac MRI showed (Figure 3) (SSFP–FIESTA, IR–GRE T1, STIR T2) confirmed the finding of a left atrium divided in two chamber by a iso–intense membraine from the coumadin ridge between the left upper pulmonary vein and the left appendage to the inter–atrial septum fossa ovalis, incomplete in the infero–lateral part. Venous drenaige and other congenital abnormalities were excluded. Miocarditis was excluded since no fibrosis in IR–GRE T1 and no edema in Triple T2 sequences. Since the non–obstructive cor triatrium sinister, grading IA according the Lucas classification, was confirmed, no surgical treatment was proposed and the patient was discharged without complications.

INTRAOPERATIVE MANAGEMENT OF A NEWBORN WITH COR TRIATRIUM DEXTRUM: A CHALLENGING CASE

Introduction Cor triatrium dextrum is an extremely rare cardiac condition with an incidence of Methods CASE REPORT: Informed parental consent was obtained to present this case. A 20-day-old term infant weighing 2.8 kg was admitted for progressive cyanosis and difficulties to thrive. At physical examination the newborn had a peripheral oxygen saturation of 75%. Preoperative chest X-ray showed a clog-shaped heart. Preoperative NT pro-BNP was 2311 ng/L. Repeated transthoracic echocardiography confirmed the diagnosis of cor triatrium dextrum and a R-to-L shunt through a patent foramen ovale. Distal pulmonary arteries were small for age. Surgery was scheduled. At the induction of anesthesia specific attention was paid to maintain normovolemia and a blood pressure and heart rate as compared to baseline values. Induction of anesthesia was performed with 2 mg Ketamine, 0.2 mg midazolam and 2 µg sufentanil. Tracheal intubation was facilitated with cisatracurium. Anesthesia was maintained with 1% Sevoflurane and a continuous infusion of sufentanil. Intraoperative transesophageal echocardiography (TEE) confirmed the diagnosis. The TEE 4 chamber view showed a membrane within the RA (Figure 1A) with a spinnaker movement in diastole when it was bulging into the TV (Figure 2). Laterally, there was an opening within the membrane permitting turbulent blood flow from the RA through the TV (Figure 1B). Resection of the membrane was uneventful and the foramen ovale was closed. Weaning from cardiopulmonary bypass (CPB) was facilitated with a continuous infusion of milrinone. Results Figures 1A and 1B: Intraoperative TEE view of the membrane. Discussion Cor triatrium dextrum being an extremely rare CHD, anesthesiologists may not be familiar with its clinical presentations. Depending on the degree of the RA obstruction, poor right ventricle (RV) filling and compromised pulmonary blood flow may occur. Neonatal cyanosis may appear due to streaming of the blood from the RA across the atrial septum to the left atrium. Intraoperative actions need to be taken in order to increase RV filling. In this case the opening from the RA to the RV resulted in turbulent flow but intraoperative hemodynamic optimization increased this forward flow and prevented any oxygen saturation concentrations LEARNING POINTS 1. Anesthesiologists need to be aware of intraoperative hemodynamic instability and cyanosis due to the obstructive membrane within RA in case of cor triatrium dextrum. 2. Pre CPB TEE should carefully evaluate the TV that may mimick an Ebstein anomaly as the membrane may bulge into the TV.

Intraoperative 2D and 3D transesophageal echocardiographic assessment of cor triatriatum with ostium secundum atrial septal defect in an adult patient

Cor triatriatum is a rare congenital cardiac anomaly. Majority of the cases present in the childhood with an incidence of 0.4%. However, we report a case of cor triatrium sinister with ostium secundum atrial septal defect (ASD) in a 39-year-old female. The intraoperative 3D transesophageal echocardiography (TEE) offers an advantage over 2D TEE in visualizing the interatrial septum and the attachments of the fibromuscular accessory membrane in the left atrium (LA), which could help in surgical decision-making in this patient.

The value of three-dimensional echocardiography in the evaluation of cor triatrium sinister

Introduction Cor triatrium sinister is a very rare congenital heart disease in which the pulmonary veins enter a proximal accessory left atrial chamber that is separated from a distal true left atrial chamber by a fibromuscular membrane presenting one or more ostia. Its incidence in the adulthood is extremely rare. Adult patients are symptomatic in case the orifice across the membrane becomes obstructive mimicking a mitral stenosis. It has been reported that severe obstruction is present if maximum doppler velocity across the orifice is more than 2 m/s.1 However, in a review of case series no correlation could be found between the pressure gradient across the fenestration and the clinical symptoms.2 We report the case of a symptomatic adult female with obstructive cor triatrium in whom the intraoperative three-dimensional transesophageal echocardiography (3D TEE) revealed the severity of obstruction in contrast to the mild intraoperative pressure gradients. Methods A 34-year-old female was scheduled for surgical repair of a cor triatrium sinister. The symptoms of dyspnea had increased during the last months (NYHA III) which resulted in the echocardiographic diagnosis. The mean pressure gradient across the orifice was estimated 6 mmHg at rest and 18 mmHg at effort using modified Bernoulli equation. There were no associated cardiac abnormalities and no signs of pulmonary hypertension. The patient gave written consent to present her case. Results The induction of anesthesia was performed with the aim to keep hemodynamic stability. The intraoperative TEE confirmed the diagnosis. Color flow across the orifice showed an aspect of rather laminar flow. The mean pressure gradient across the sole orifice was 2 mmHg.Three-dimensional en face view of the membrane permitted offline analysis of the lesion in several orthogonal planes and measurement of the effective opening area of the orifice which was 1.45 cm2 (Fig 1). Discussion This case illustrates that pressure gradient across an obstructive cor triatrium may underestimate the true severity of the lesion as the blood flow may be low through the channel. Therefore, 3D echocardiography should be routinely performed in patients with cor triatrium in order to measure the exact maximal fenestration orifice area.

Forme atypique de cœur triatrial chez un patient de 59 ans

Cor triatrium is a rare congenital heart disease typically diagnosed amongst very young patients. Delayed diagnosis in the elderly is much more unusual. We report the case of a 59-year-old male with unremarkable medical history in whom we have discovered, on an echocardiography performed for an exploration of transient ischemic accident with atrial fibrillation, a particular form of an isolated cor triatrium dextrum investigated with multimodality imaging.

Combined congenital heart disease — cor triatrium, atrial septal defects and total anomalous drainage of the right pulmonary veins into the right atrium

Combined congenital heart disease — cor triatrium, atrial septal defects and total anomalous drainage of the right pulmonary veins into the right atrium

Anévrisme géant de l’artère pulmonaire : aspects étiologiques et évolutifs exceptionnels sur 17 ans

True pulmonary artery aneurysm (AAP) is rare and represent less than 1% of intra-thoracic aneurysms. We report a case of a AAP in a patient with a likely cor triatrium sinister, with an obstructive membrane responsible for pulmonary hypertension, explaining AAP. The long-term evolution of 17years is made to an uncomplicated myocardial infarction. The patient died eight months later suddenly probably due to the rupture of the PAA.

Cor triatrium presenting as mitral stenosis and severe anemia

Cor triatrium presenting as mitral stenosis and severe anemia

Anesthésie pour césarienne chez une patiente porteuse d’une cardiopathie triatriale

A 41-year-old woman suffering from a left cor triatrium, pregnant for the first time, was hospitalized for a caesarean section in the context of a pulmonary arterial hypertension with severe anaemia. The anaesthetic strategy which was decided on involved setting up a haemodynamic monitoring prior to induction of a general anaesthetia with etomidate, remifentanil and succinylcholine and maintained with propofol, sufentanil and cisatracurium. This strategy allowed the hemodynamic to be stabilized during the operation. The improvement of the arterial pulmonary hypertension immediately after coming out of the operating theatre allowed the patient to be briefly monitored in the intensive care unit and to be allowed home on the 10th day following the operation. The patient's cardiopathy was corrected in the 5th month after the birth.

Congenital tracheal stenosis: the prognostic significance of associated cardiovascular anomalies and the optimal timing of surgical treatment

Background/Purpose Cardiovascular anomalies (CA) are frequently associated with congenital tracheal stenosis (CTS), but their prognostic impact on CTS and the optimal timing of surgical treatment remain uncertain. The aim of this study was to explore the prognostic factors and the optimal timing of surgical treatment in CTS patients with CA. Methods After obtaining institutional review board approval, a retrospective review of 42 patients who underwent surgical repair of CTS between 1996 and 2006 was conducted. The patients were divided into 3 groups: CTS without CA (n = 10, group A), CTS with CA repaired simultaneously (n = 27, group B), and CTS with CA repaired in stages (n = 5, group C). Seven clinical characteristics, including gestational week and weight at birth, the age and body weight at operation, the length of tracheal stenosis (%), the duration of cardiopulmonary bypass (CPB) during surgery, and operation time were compared among the groups using analysis of variance, Fisher's Exact test, and Student's t test. Results Although no operative mortalities occurred in groups A and C, there were 3 early deaths and 1 late death in group B. The deaths occurred in cases with associated complex CA (critical pulmonary stenosis, tetralogy of Fallot with an absent pulmonary valve, right ventricular outflow block, and cor triatrium). The duration of CPB was significantly different between groups A and B ( P = .017), and furthermore, CPB time was significantly longer in early death cases than in surviving cases in group B (318.3 ± .71.1 vs 204.0 ± 67.8 minutes; P = .012). Conclusions Complex CA and long CPB duration would be prognostic factors for the outcome of surgical management for CTS and CA. Simultaneous reconstruction of CTS and simple CA appears to be a reasonable method of surgical intervention, but patients with long segment CTS with complex CA may still be difficult to cure using this strategy, and staged correction may be considered.

Cor triatrium dexter with Budd‐Chiari syndrome and a review of ascites in young dogs

AbstractAscites with accumulation of a true or modified transudate, is relatively uncommon in young dogs. When it does occur, congenital defects of the cardiovascular, gastrointestinal, or renal systems, infectious diseases and trauma must be considered. Cor triatrium dexter, an abnormal membrane obstructing the caudal vena cava at the level of the right atrium, was diagnosed in an eight‐month‐old cocker spaniel presented with ascites.

Radiologic diagnosis of cor triatrium in infants.

When cor triatrium causes symptoms in infants, roentgen examination of the chest reveals enlargement of the left atrium and right ventricle and pulmonary venous stasis. Further diagnostic investigation is necessary. By injection of contrast medium into the right ventricle or pulmonary artery, the diaphragm dividing the left atrium can be demonstrated directly.

Cor triatrium dexter.

A case of cor triatrium dexter is described in which the patient had been independently assessed in two cardiological centres as having a form of Ebstein's anomaly. The differential diognosis is discussed.

Transseptal Left Heart Catheterization and Left Atrial Angiocardiography in the Diagnosis of Cor Triatrium

Cor triatrium was diagnosed in a 16-year-old boy by transseptal left heart catheterization and left atrial angiocardiography. In patients, where the diagnosis of cor triatrium is entertained and a low cardiac output or pulmonary stasis is present, these methods should be considered as angiocardiography following injection into the pulmonary artery often fail to demonstrate the diaphragm. A short review of cor triatrium is also given.