Clinical presentation, diagnosis and treatment of four cases of cor triatriatum

We report four cases of cor triatriatum. In two of them the fibromuscular membrane was obstructive and associated with an atrial septal defect between the left atrial accessory chamber and the right atrium; in those cases the clinical findings were due to the pulmonary hypervascularity. The third case was associated to a small patent foramen ovale, and the clinical findings were due to the obstruction to flow through the membrane in the left atrium, producing venocapilar pulmonary hypertension.In these three cases surgical treatment was carried out through a right atriotomy. In all of them a good corelationship was found between the echocardiographic and the surgical findings.The follow-up has been good, all previous symptoms disappearing, remaining symptom free. The fourth case is an asymptomatic 5-year-old girl, with a non-obstructive membrane and without any associated anomalies.     

Association of cor triatriatum and obstruction of abnormal left pulmonary venous drainage. Apropos of a case

A case of subtotal cor triatriatum associated with a partial anomalous pulmonary venous drainage of the left lung, in a 30 months old boy is reported. Diagnosis was suggested by the presence of pulmonary venous obstruction and pulmonary hypertension. Doppler, echocardiogram diagnosed the cor triatriatum and the degree of the pulmonary hypertension. The cine-angiography demonstrated the anomalous, obstructive, left pulmonary venous drainage. The pulmonary hypertension has mandatorised an early surgical cure, the result was satisfactory at 16 months.     

三房心的临床诊断和外科治疗

目的 总结三房心的临床表现、诊断要点及外科治疗经验。方法 10例三房心患者,年龄6个月～27岁,体重5.9～63kg,均于体外循环下手术,彻底切除纤维肌性隔,合并其它畸形者一并矫治。结果 手术死亡2人。存活病人康复顺利,随访6个月～5年,无并发症发生。结论三房心有较严重的血流动力学改变,明确诊断即应手术治疗。病人术后及远期效果俱佳。早期诊断及合并畸形的治疗是降低死亡率的重要因素。     

Endocardial cushion defect associated with cor triatriatum sinistrum or supravalve mitral ring

Clinical and angiographic or autopsy data, or both, on three children with a subdivided left atrium (cor triatriatum) and an associated endocardial cushion defect are reviewed. (One child had ostium primum defect, and two had complete atrioventricular [A-V] canal.) A fourth patient demonstrates the difficulties in differentiating subdivided left atrium from supravalve mitral stenosis in the presence of an endocardial cushion defect. The clinical findings are greatly influenced by the endocardial cushion defect. A pressure gradient between the pulmonary wedge and (left or right) ventricular end-diastolic pressures in patients with an endocardial cushion defect indicates pulmonary venous obstruction and should alert one to the possibility of these combined lesions. The exact diagnosis is made with injections of angiographic contrast medium into the proximal and distal left atrial chambers, to document the respective relations of the pulmonary veins, left atrial appendage and A-V valves to these atrial chambers. All three patients with an endocardial cushion defect and a subdivided left atrium had an associated patent ductus arteriosus. The common association of subdivided left atrium with intracardiac, pulmonary venous and aortic anomalies is again demonstrated.     

23例三房心临床分析

目的：总结三房心临床特点、合并畸形及影像学诊断特点。方法：1993年1月至2005年6月共收治23例三房心患者,男13例,女10例,年龄0．3～38（9．6±10．0）岁。均为左型三房心,其中21例获得手术证实,完全型15例,部分型6例。心电图检查：电轴右偏18例,电轴偏左1例,电轴正常4例。胸片检查：18例肺血增多。结果：术前经超声检查确诊12例（52．2％）,10例行心导管确诊4例（40％）。21例中16例（76．2％）合并其它畸形,合并房间隔缺损14例（66．7％）、部分肺静脉异位引流6例（28．6％）、肺动脉瓣狭窄3例（14．3％）,永存左上腔3例（14．3％）。结论：三房心临床表现多样,合并畸形发生率较高,彩色超声心动图检查有较高诊断价值。心导管检查对合并畸形诊断率较高,对合并畸形者强调术中探查。     

Outcome after repair of cor triatriatum

Cor triatriatum represents <0.1% of all congenital cardiac malformations. Symptoms in patients with cor triatriatum are related to pulmonary venous obstruction and pressure loading of the right side of the heart. The aim of this study was to describe our institutional experience with repair of cor triatriatum. From June 1963 to June 2010, 65 patients underwent repair at a median age of 7.2 months (range 2 days to 47.6 years). Among these patients, 49 (75%) had associated congenital heart defects. Atrial septal defect (n = 29), ventricular septal defect (n = 15), partially or totally anomalous pulmonary venous return (n = 14), mitral valve abnormalities (n = 11), and supravalvar mitral ring (n = 5) were the most common associated defects. Surgical treatment consisted of excision of the membrane, along with additional procedures in 47 patients (72%). Five patients had new postoperative supraventricular arrhythmias. During a median follow-up period of 5.4 years, no patients underwent reintervention for recurrent left atrial obstruction, 7 patients were noted to have minor residual cor triatriatum without obstruction, and 8 patients (including 4 diagnosed before cor triatriatum repair) had pulmonary vein stenosis, 6 of whom underwent intervention for that reason. In conclusion, in this large surgical series of patients who underwent repair of cor triatriatum, there were no cases of significant residual or recurrent cor triatriatum. Although the association between cor triatriatum and pulmonary vein stenosis has been described previously, the relative frequency of this condition in our cohort (>10%, including patients diagnosed before and after cor triatriatum repair) is noteworthy. Abnormalities of the mitral valve and a supravalvar mitral ring were also seen more often than the existing research would suggest, which may be another important consideration in evaluating and following these patients.     

The right heart in congenital heart disease

The clinical presentation of right ventricular (RV) dysfunction due to congenital heart disease (CHD) is similar to that of cor pulmonale. RV volume and pressure loads, and primary RV myocardial dysfunction are mechanisms by which CHD affects right heart function. RV volume load may arise from pre-tricuspid left to right shunts (e.g., atrial septal defect) or regurgitant lesions in the right heart (e.g., Ebstein's anomaly of the tricuspid valve and pulmonary insufficiency after repair of tetralogy of Fallot). RV pressure load may be caused by anatomic obstruction to RV outflow and by pulmonary arteriolar hypertension. The latter can result from Eisenmenger syndrome secondary to congenital and postoperative left to right shunts or from defects that cause pulmonary venous hypertension (e.g., pulmonary vein stenosis, cor triatriatum, or mitral stenosis). The RV myocardium may be affected by a primary cardiomyopathy or by congenital abnormalities of the coronary vessels. Finally, CHD may be associated with airway obstruction, scoliosis, or pulmonary emboli, which, in turn, may lead to the development of cor pulmonale. Congenital heart disease, therefore, must be included in the differential diagnosis of patients who present with right ventricular dysfunction.     

Concealed left atrial membrane: Pitfalls in the diagnosis of cor triatriatum and supravalve mitral ring

Cor triatriatum and supravalve mitral ring are forms of congenital left ventricular inflow obstruction produced by membranes within the left atrium. Typically, these defects occur as isolated anomalies with manifestations of pulmonary venous obstruction. Four children are presented whose left atrial membrane was associated with other significant cardiac defects, including, in one patient each, simple coarctation of the aorta, sinus venosus atrial septal defect, tricuspid atresia and complex coarctation of the aorta syndrome. The patient with the latter defect had undergone previous pulmonary arterial banding. None of these patients demonstrated significant pulmonary venous obstruction at cardiac catheterization. All patients had a normal value for either pulmonary arterial diastolic or pulmonary arterial wedge pressure. Three mechanisms explained the lack of pulmonary venous obstruction: (1) a large cross-sectional area of membrane openings, (2) an atrial septal defect that was confined to the pulmonary venous chamber and decompressed it by allowing blood to escape into the right atrium, and (3) decreased pulmonary blood flow. The diagnosis was facilitated by two dimensional echocardiography. Accurate diagnosis of left atrial membrane in the setting of other cardiac defects is of practical significance because pulmonary venous obstruction may occur after surgery for the associated defects.     

Subdivided left atrium: an expanded concept of cor triatriatum sinistrum.

Twenty-four hearts with cor triatriatum were studied. On the basis of this material and a review of the literature a new classification of "subdivided left atrium" is proposed. Tye A, the most common form of subdivided left atrium, is the classic cor triatriatum with its multiple variations of partial anomalous pulmonary venous drainage; the fossa ovalis can be related to the proximal left atrial chamber (type A, a) or the distal left atrial chamber (type A, b). Type B hearts are related to (but not identical with) total anomalous pulmonary venous drainage into the coronary sinus; the coronary sinus opening is atretic in these hearts; abnormal defects connect the proximal left atrial chamber usually with the right atrium only, rarely also with the distal left atrial chamber. The Type C heart, first reported in this paper, has a superiorly and medially situated proximal chamber, located between the right and distal left atrium; it does not receive any pulmonary veins; the coronary sinus is normally formed. Current morphogenetic hypotheses that satisfactorily explain the Type A b and B heart fail to account for the Type A a and C heart.     

Cor triatriatum

The coexistence of cor triatriatum and tetralogy of Fallot (TOF) is rare. Preoperative identification of cor triatriatum may be difficult owing to reduced pulmonary blood flow in patients with TOF. However, it is imperative to identify this rare combination as failure to identify obstruction to pulmonary venous egress may result in persistent pulmonary venous hypertension postoperatively. The authors discuss hemodynamic aspects of this rare coexistence in a 14‐month‐old child in whom pulmonary capillary wedge pressure was elevated despite right ventricular outflow obstruction. © 2012 Wiley Periodicals, Inc.     

Surgical case of partial anomalous pulmonary venous connection to superior vena cava with cor triatriatum: Williams’ modification and excision of diaphragm

We experienced a successful pediatric surgical case of partial anomalous pulmonary venous connection to the superior vena cava with cor triatriatum. Echocardiography and multidetector-row computed tomography showed partial anomalous pulmonary venous connection (right upper pulmonary vein connected to the high superior vena cava) and atypical cor triatriatum (analogue to type III-A2 of Lucas-Schmidt classification: left upper pulmonary vein had dual connection to the innominate vein via vertical vein and the accessory chamber). At 8 years of age, the male patient underwent extracardiac right atrial pedicle repair of partial anomalous pulmonary venous connection to the superior vena cava (Williams’ modification) and excision of the diaphragm between the accessory chamber and the left atrium simultaneously. The postoperative course was uneventful in normal sinus rhythm and there was no stenosis of newer drainage root from right upper pulmonary vein.     

Cor Triatriatum Sinistrum Presenting as Cardioembolic Stroke: An Unusual Cause of Adolescent Hemiparesis

Cor triatriatum sinistrum is a rare congenital cardiac malformation, in which the left atrium (LA) is divided into two distinct chambers by a fibromuscular membrane. Classically, the proximal (upper or superior) chamber of the LA receives pulmonary venous connections, whereas the distal (lower or inferior) chamber contains LA appendage and true atrial septum containing fossa ovalis. The distal chamber is in continuity with the atrioventricular valve, while the two chambers communicate through a defect in the membrane. The hemodynamics of cor triatriatum sinistrum are similar to that of mitral stenosis due to obstructive property of membrane. The majority of reported cases of cor triatriatum occur in infants with symptoms of pulmonary venous obstruction, with adult cases being rare. Herein, we describe an unusual case of cor triatriatum in a 17‐year‐old boy who presented for the first time with embolic cerebral infarction with left hemiparesis.     

Pitfalls in the diagnosis of tricuspid atresia: report of a new angiocardiographic sign

Two patients with the classical clinical, angiographic, and echocardiographic signs of tricuspid atresia are reported, where the correct diagnoses were made at operation; one child had cor triatriatum dexter, the other child total anomalous systemic venous drainage to the left atrium. The literature on cor triatriatum dexter and total anomalous systemic venous drainage is discussed and a new angiographic sign for the differentiation from tricuspid atresia is reported: nonopacification of the right atrial appendage with right atrial angiocardiography.     

The right ventricle in disorders causing pulmonary venous hypertension.

The right ventricle is frequently affected by pulmonary arterial hypertension of postcapillary obstructive origin. The disorders that cause states of pulmonary venous hypertension arise in the left ventricle, left atrium, or pulmonary veins. Biochemical factors, the pericardium, interventricular septum, and pulmonary arterial system combine to communicate the effects of these disorders to the right ventricle. Although not ideally suited to pressure loading, the right ventricle undergoes a series of structural and hemodynamic adaptations that allow for chronic compensation until failure supervenes, with characteristic clinical findings. Exercise may serve to magnify the abnormalities of right ventricular function. Correction of left-sided heart lesions with improvement in pulmonary venous hypertension is associated with favorable effects on the right ventricle. In states of left-sided heart failure, the level of right ventricular function may provide important prognostic information.     

Morphological dilemma: Anomalous pulmonary venous confluence or cor triatriatum—does it matter?

Cardiac variant of total anomalous pulmonary venous return is a rare entity, whereby all the pulmonary veins drain directly to the right atrium or coronary sinus. The effective left heart blood flow channels through a small stretched patent foramen ovale and can often be confused with a variant of cor triatriatum. Cor triatriatum is a rare congenital cardiac anomaly where pulmonary veins drain to a persistent chamber above the left atrium with a membrane separating these two. There persists either a small aperture directly from the true to the accessory left atrium or none at all. Where there is no such aperture, it is often physiologically akin to the cardiac variant of total anomalous pulmonary venous return described above. Such morphological differentiation is often challenging in a clinical situation, but the effective treatment remains the same. It involves removal of the common wall between the two chambers and baffling the pulmonary veins effectively to the left atrium. We describe such a case where the pulmonary venous return is to the right atrium, managed recently in our centre, and discuss the morphological differences between these two.     

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.     

Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium

Two-dimensional echocardiographic findings and surgical repair in three infants with atrioventricular (AV) canal and pulmonary venous obstruction are described. The AV canal was complicated by cor triatriatum in two patients and by double outlet right atrium in the other. In patients with AV canal, anatomic obstruction of pulmonary venous return should be excluded by detailed cardiac ultrasound examination.     

Hypoplastic left heart syndrome,cor triatriatum and partial anomalous pulmonary venous connection: Imaging of a very rare association

A newborn is presented with an association of hypoplastic left heart syndrome, cor triatriatum and partial anomalous pulmonary venous connection. The diagnosis was established with echocardiography and further confirmed with computed tomography. To our knowledge the images of such an association have never been reported before.     

Benign Mediastinal Lymphadenopathy Presenting as Cor Triatriatum

This report describes a patient with a superior mediastinal mass and extensive intrathoracic lymphadenopathy referred for oncologic care. During her evaluation, an abnormal cardiovascular examination resulted in an echocardiographic evaluation and an unanticipated diagnosis of a highly obstructive left atrial cor triatriatum was uncovered. The patient underwent repair of cor triatriatum and lymph node biopsy shortly after the diagnosis was made. The biopsies revealed reactive lymph nodes with lymphatic dilation and no inflammatory or neoplastic features. To our knowledge, this case represents the first pediatric example of extensive mediastinal lymphadenopathy mimicking the appearance of a malignant process as a result of severe pulmonary venous hypertension.     

Cor triatriatum sinistrum. Diagnostic features on cross sectional echocardiography.

M mode and cross sectional echocardiography was carried out in three cases of cor triatriatum sinistrum (two infants and one adult). In two cases a peculiar double arch appearance, not previously reported, was found. All three cases were referred for surgery without cardiac catheterisation, and the diagnosis proved to be correct. The characteristic echocardiographic feature of cor triatriatum is an intra-atrial membrane detected in multiple planes of examination, curving anteroinferiorly and inserting some distance away from the mitral valve ring, proximal to the left atrial appendage. Superiorly the membrane runs parallel to, and a short distance behind, the aortic root creating a superior recess of the distal left atrial chamber. These features differentiate cor triatriatum from a supravalvar mitral ring. During diastole the membrane moves forward towards the mitral valve funnel. This, together with the arching appearance of the membrane on four chamber views and the more superior position of the membrane, makes it possible to distinguish cor triatriatum from total anomalous pulmonary venous drainage to the coronary sinus. From a review of past experience at the Brompton Hospital of the diagnostic accuracy of cardiac catheterisation in this condition, it is concluded that cross sectional echocardiography is superior to angiography as a technique for diagnosing cor triatriatum.