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PubMed2000

[Divided left atrium: operative results and follow-up in the cor triatriatum].

INTRODUCTION AND OBJECTIVES: 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. PATIENTS AND METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Female↗
PubMed1976

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.

Adult↗
PubMed1990

[Cor triatriatum associated with coarctation of the aorta and VSD].

A case of cor triatriatum associated with coarctation of the aorta (CoA) and VSD is reported. A 4-month-old boy underwent subclavian flap aortoplasty and ligation of PDA with the diagnosis of CoA and VSD. At the age of 1 year and 2 months, echocardiography revealed an abnormal subdividing membrane in the left atrium. Under the definite diagnosis of cor triatriatum (Lucas & Schmidt type I B1) and VSD, complete removal of the abnormal septum, patch closure of ASD and VSD was performed with the aid of cardiopulmonary bypass, blood cardioplegia and topical cardiac cooling. Postoperative course was uneventful and the patient was discharged from the hospital on the 38th postoperative day. An association of cor triatriatum, VSD, and coarctation of the aorta is very rare, and to our knowledge, the case presented here seems to be the first report of successful total correction by a staged approach in the literature.

Aortic Coarctation↗
PubMed1984

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.

Adult↗
PubMed1977

Echocardiographic features of cor triatriatum.

A patient is presented who had cor triatriatum documented with angiography and surgery. On echocardiographic study, an abnormal, dense linear echo on "sector scan" was consistently seen to traverse the left atrial cavity obliquely; the ends of the echo were attached to the posterior aortic and lateral walls of the left atrium. In addition, high frequency oscillatory movements were evident in the echo of the posterior mitral cusp. Both echocardiographic features disappeared promptly after successful resection of the intraatrial fibrinous membrane. This experience indicates that in the presence of strong evidence of mitral stenosis, an unexpectedly normal mitral valve echogram should initiate a thorough echographic search for cor triatriatum, a treatable cardiac malformation whose diagnosis can easily be missed on "routine" echocardiographic studies.

Adult↗
PubMed1994

[Left cor triatriatum in a man over 60].

A case of cor triatriatum in a 66-year-old man is reported. The patient died of pneumonia; ante mortem diagnosis was made with both transthoracic and transesophageal echocardiography. Autopsy finding showed a very good correlation between anatomical and echocardiographic abnormalities. Cor triatriatum is amenable to surgical correction and echocardiography is extremely helpful in the diagnosis.

Age Factors↗
PubMed2003

Cor triatriatum sinister presenting in the adult as mitral stenosis.

Cor triatriatum sinister is a rare congenital defect in which the left atrium is divided by a fibromuscular membrane into two distinct chambers. Classically, patients present in infancy although in some cases they remain asymptomatic until adulthood. The clinical features on presentation can mimic those of mitral stenosis due to the obstructive properties of the membrane. Cor triatriatum sinister presented in this case in an adult as mitral stenosis. Factors that may be relevant in determining late presentation are also discussed.

Cor Triatriatum↗
PubMed1977

Subtotal cor triatriatum with left partial anomalous pulmonary venous return. Successful surgical repair in an infant.

A 10-week-old infant underwent successful repair of subtotal cor triatriatum associated with left-sided partial anomalous pulmonary venous return to the innominate vein. This unusual type of cor triatriatum represents an intermediate embryologic form between pure cor triatriatum and total anomalous pulmonary venous return. Important hemodynamic differences exist between this defect and pure cor triatriatum. Exact anatomic diagnosis prior to operation is highly desirable. To our knowledge, this case represents the first successful repair of this very rare congenital cardiac defect.

Electrocardiography↗
PubMedDate not supplied

Early diagnosis and follow-up by echocardiography of acquired cor triatriatum after orthotopic heart transplantation.

Acquired cor triatriatum in a heart transplant recipient, secondary to a prominent left atrial suture line and torsion of the atria, was diagnosed immediately after cardiopulmonary bypass by transesophageal echocardiography. The patient was followed with serial echocardiograms and right heart catheterizations, showing resolution of the obstruction.

Cardiac Catheterization↗
PubMed1990

Cor triatriatum sinistrum: diagnosis by Doppler echocardiography.

Cor triatriatum (CTT) sinistrum is a rare, congenital left ventricular inflow anomaly. Previous reports have used cardiac angiography, two-dimensional echocardiography, transesophageal echocardiography and magnetic resonance imaging to establish the diagnosis prior to surgery. We report a case of CTT diagnosed by echocardiography whose hemodynamic significance was established by the use of pulsed wave and color Doppler techniques. Immediate surgery was recommended based on anatomical and hemodynamic parameters.

Cor Triatriatum↗
PubMed1992

Transoesophageal echocardiography in the diagnosis of cor triatriatum; incremental value of colour Doppler.

Cor triatriatum sinistrum is a rare congenital cardiac abnormality often accompanied by other malformations. Transthoracic echocardiography does not always define the membrane and associated abnormalities; so, transoesophageal colour Doppler echocardiography was used to visualize intra-atrial blood flow through the intra-atrial membrane in a patient in whom the membranous orifice could not be seen by precordial echocardiography. There was no anomalous pulmonary venous drainage or associated atrial septal defect.

Adult↗
PubMed1991

Cor triatriatum. Clinical presentation and operative results.

From November 1973 to January 1988, 15 patients with cor triatriatum underwent surgical correction at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. Their ages ranged from 15 days to 48 years. Eight patients (53%) were younger than age 1 year. Evidence of congestive heart failure was present in five infants, whereas three infants, three children, and two adults initially had signs of pulmonary venous obstruction, and two children had a heart murmur only. Five patients had cor triatriatum alone; in ten cases there also was an atrial septal defect. Associated anomalies in four patients included left superior vena cava, ventricular septal defect and left superior vena cava, partial anomalous pulmonary venous connection, and bilateral partial anomalous pulmonary venous connection. Cor triatriatum was repaired with the aid of cardiopulmonary bypass in all patients. Excision of the membrane was accomplished with a right atrial approach in 13 patients and a left atriotomy in one patient. Both atria were opened in one case. All associated anomalies were simultaneously corrected. Three patients (20%) died early after operation. Among the 12 survivors, no late events have occurred, and all of them are presently in New York Heart Association functional class I. A recent echocardiogram shows absence of residual obstruction or shunt and good development of the left cardiac chambers.

Adolescent↗
PubMed2005

Cor triatriatum sinistrum: a rare congenital cardiac anomaly presenting in an adult with chronic atrial fibrillation.

Cor triatriatum is a rare congenital cardiac anomaly in which the left atrium is divided into proximal (dorsal or upper) and distal (ventral or lower) chambers by a fibromuscular septum. The upper chamber receives the pulmonary veins and the lower chamber contains the atrial appendage and the mitral valve. The 2 chambers communicate through a defect in the membrane. Cor triatriatum is often associated with other congenital cardiac anomalies. Most frequently, the upper chamber communicates with the right atrium through a patent foramen ovale or atrial septal defect, and the clinical symptoms simulate anomalous pulmonary venous return. Less commonly, the foramen ovale communicates with the distal chamber and the clinical features mimic mitral stenosis. When cor triatriatum is the only abnormality, the clinical findings are also similar to mitral stenosis with development of pulmonary hypertension and subsequent right ventricular hypertrophy and atrial enlargement. The diagnosis is usually made in infancy or childhood, and the lack of treatment results in death in 75% of patients. We report the case of a woman who presented much later in life. The patient was a 57-year-old female with a clinical history of chronic atrial fibrillation who presented to the emergency department because of a "funny sensation" in her chest, though she denied chest pain, nausea, vomiting, or diaphoresis. EKG revealed atrial fibrillation with a rapid ventricular response and a tachycardic rate of 157. She had a therapeutic level of digoxin, and cardiac enzymes were normal. The patient was admitted and placed on Cardizem drip. Serial EKGs remained normal and heart rate control was achieved. On hospital day 2, the patient became dyspneic and cyanotic. She went into cardiac arrest and died.Autopsy revealed cardiomegaly (610 g) with 4-chamber dilatation. A septum divided the left atrium into 2 chambers. The defect in the dividing membrane measured 1 cm in diameter. No other congenital defects were noted. The large size of the defect in the membrane likely accounted for the late onset of symptoms that allowed this patient to survive into adulthood without previous diagnosis or surgical intervention (which is usually required in childhood).

Atrial Fibrillation↗
PubMed2001

[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.

Child, Preschool↗
PubMed1993

[Cor triatriatum with situs inversus totalis: a case report of an infant].

Cor triatriatum associated with situs inversus is rare. A 6-month-old male who had cor triatriatum with atrial septal defect (ASD) and situs inversus totalis was successfully operated. Urgent surgical intervention was performed because of congestive cardiac failure despite drug therapy. The abnormal septum in the left atrium was resected and ASD was closed. His postoperative course has been uneventful.

Cardiac Catheterization↗
PubMed2005

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.

Child↗
PubMed1982

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.

Child↗
PubMed2003

Asymptomatic cor triatriatum incidentally revealed in an elderly patient with mitral stenosis.

A case of mitral stenosis accompanied by asymptomatic cor triatriatum, underwent surgical correction, including excision of the diaphragm in the left atrium and mitral valve replacement with a 27 mm St. Jude Medical mechanical valve. A preoperative transthoracic echocardiogram disclosed a linear structure in the left atrium which was suspicious for cor triatriatum, which was confirmed by computed tomography (CT scan) and cardiac catheterization. Cardiac catheterization revealed: 1) mitral valve stenosis of 0.9 cm2 of estimated mitral area, 2) division of the left atrium into two chambers by a diaphragm, that might have multiple ostia; blood flow from the common pulmonary venous chamber to the true left atrium seemed to be unrestricted, and all four pulmonary veins opened into the common pulmonary venous chamber, 3) there were no other apparent cardiac anomalies believed to coexist with cor triatriatum. Preoperative examinations is important for determination of operative strategies.

Aged↗