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PubMed1981

Cor triatriatum.

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Heart Atria↗
PubMed1994

[A rare case of total anomalous pulmonary venous connection to the right atrium confounding with cor triatrium].

Three-days-old newborn baby underwent ligation of the ductus arteriosus under the diagnosis of TAPVC and PDA with high flow heart failure. Her UCG showed the direct connection of common pulmonary venous chamber (CPVC) to the upper and medial portion of right atrium. CPVC positioned just above the left atrium but does not communicated with the left atrium. These findings characterized this anomaly as II A cor triatriatum classified by Lucus-Schumidt. At 5-month-old, corrective operation was performed. Both right and left venous chambers joined just before the connection to right atrium and drained to right atrium at the upper portion of atrial septum. CPVC and left atrium had independent wall. Accordingly, operative diagnosis was made as TAPVC (IIb). Intraatrial routing was performed using porcine pericardial patch. At 6 months after operation, patient is doing well without the sign of venous obstruction. This type of TAPVC is rare and may confound with cor triatriatum. Our case may suggest the pathological mechanism of cor triatriatum and that at least some of cor triatriatum may be identical with TAPVC.

Cardiac Surgical Procedures↗
PubMedDate not supplied

Congenital left ventricular inflow obstructions--an echocardiographic study.

Thirty nine consecutive cases of congenital left ventricular inflow obstructions (LVIO) diagnosed by two dimensional and Doppler echocardiography are described. The commonest referral diagnosis was severe pulmonary arterial hypertension. Confirmation by cardiac catheterisation and/or surgery was available in twenty one of these cases. The ages ranged from one month to 15 years (mean 3.8 +/- 4.5 years) and 28 were males. Four types of LVIO were defined: i) Congenital mitral stenosis (22 cases). Ventricular septal defect was the commonest association. ii) Cor-triatriatum (7 cases) Out of three patients operated, in one it was found to be a supravalvular mitral ring and not cor-triatriatum as earlier thought on echocardiography. iii) Pulmonary venous obstruction (8 cases). Atrial septal defect and ventricular septal defect were the common associations. iv) Supravalvular mitral ring (2 cases). Double outlet right ventricle and a large ventricular septal defect were associated with one case each. In our experience, two dimensional and Doppler echocardiography is a very useful and reliable investigation for defining the various types of congenital left ventricular inflow obstructions and their hemodynamic significance.

Adolescent↗
PubMed2005

Spontaneous echo contrast of unexpected etiology.

Cor triatriatum sinister is a rare congenital anomaly characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers: one entering the four pulmonary veins, the other connecting to the mitral valve. The extent of the communication between the two chambers and the presence of associated lesions determine the severity of symptoms and the complications. We report the case of a 20-year-old man firstly diagnosed with obstructive cor triatriatum sinister and severe pulmonary hypertension.

Adult↗
PubMed2006

Right atrial and tricuspid hypoplasia.

Cor triatriatum dexter is a rare cardiac anomaly with division between the sinus and primitive atrial portions of the right atrium. It is supposed that the failure of the venous valves to regress appropriately may create abnormalities in fetal circulation predisposing the fetus to maldevelopment of the right heart structures. The underdevelopment of the right ventricle with hypoplasia or atresia of the tricuspid valve is a well known abnormality, but underdevelopment of the right atrium with tricuspid hypoplasia due to multiple venous valves is extremely rare. This is a case of complex type of cor triatriatum dexter in which the right atrium is hypoplastic and like a tube formed by the superior and inferior vena cava and is divided into 3 portions by 2 membranes.

Adult↗
PubMedDate not supplied

[Color doppler-echocardiography study of the triatrial heart].

Two-dimensional echocardiography has proved its utility in the diagnosis of cor triatriatum sinister by visualizing the defect and its relation to the other structures. Recently pulsed and continuous wave Doppler have been used to determine the degree of obstruction of the membrane. In this study we present three patients with ages six, eight and fourteen months respectively, all with a clinical history of growth retardation, respiratory infections and dyspnea. The patients were studied with two-dimensional and color flow Doppler echocardiography. In all, a membrane was visualized, which divided the left atrium in two chambers, a superior one which received the pulmonary veins and an inferior in continuity with the mitral valve and the left atrial appendage and with color flow Doppler the absence of obstruction at this level was demonstrated. In one patient besides the anomaly of cor triatriatum complete transposition of great arteries with pulmonary stenosis was diagnosed and in another one, patent ductus arteriosus and ventricular septal defect. Diagnosis were confirmed by hemodynamic studies. It is concluded that the color flow Doppler is a diagnostic method of great utility because it permits to obtain more information which complements conventional echocardiography.

Cor Triatriatum↗
PubMed1985

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.

Angiocardiography↗
PubMedDate not supplied

Intrauterine closure of membranous ventricular septal defects: mechanism of closure in two autopsy specimens.

Anatomic evidence of intrauterine closure of ventricular septal defects (VSDs) has been reported rarely. Between 1985 and 1990, 112 autopsies were performed at the Mayo Clinic on third trimester stillborns and infants who died during the first week of life. There were 21 (19%) cases of congenital heart disease. VSD was found in 12 (11%) cases: in eight (7%) as a part of a more complex heart defect and in four (4%) as an isolated lesion. Two cases with membranous VSDs with tricuspid valve tissue partially occluding the ventricular septal defect were found. A 2280-g female infant (case 1) with trisomy 18 died at 4 days of age. Autopsy revealed bilateral superior venae cavae, a large atrial septal defect, cor triatriatum, an atypical tricuspid valve with large septal leaflet partially obstructing a large membranous VSD, a hypoplastic right ventricle, and severe pulmonic stenosis. A 2610-g female infant (case 2), born with congenital heart block died at 4 days of age. Autopsy revealed cor triatriatum dexter obstructing the tricuspid orifice, a large membranous VSD partially obstructed by the septal leaflet of the tricuspid valve, four small muscular VSDs, and pulmonic stenosis. These cases suggest that closure of membranous VSDs may begin in utero and the mechanism of closure is similar to that reported postnatally.

Female↗
PubMedDate not supplied

Pathogenesis of persistent left superior vena cava with a coronary sinus connection.

The basis for persistence of the left superior vena cava (LSVC), usually associated with cardiac malformations, is poorly understood. We examined 351 staged, serially sectioned human embryos in the Carnegie Embryological Collection and 1208 specimens with congenital cardiovascular malformations in the Pathology Collection of the Johns Hopkins Hospital. A standardized questionnaire was answered for each embryo and autopsy case and a computer program was employed to tabulate concurrent anatomic features. In the normal embryos a symmetric venous system appeared with the heart tube at Carnegie stage 9; the sinoatrial junction translocated to the right and the relationship of the coronary sinus to the LSVC was established by stage 12. The LSVC was patent through stage 20 and subsequently underwent luminal obliteration by compression between the left atrium and the hilum of the left lung. Among the 1208 hearts with a congenital abnormality, 104 (9%) had a persistent LSVC with a coronary sinus connection. Statistically, significantly more frequent associations were found between persistent LSVC and atrioventricular canal defects, cor triatriatum, and mitral atresia and a significantly less frequent association was observed between persistent LSVC and atrial septal defect or patent foramen ovale as a primary defect. The normally late embryonic obliteration of the LSVC suggests that its persistence would be secondary to reduce cardiac compression or to blood flow redistribution at an early stage, and the malformations associated with persistent LSVC support that view. Identification of a persistent left superior vena cava with coronary sinus connection should suggest an associated malformation, especially atrioventricular canal, cor triatriatum, or mitral atresia.

Adolescent↗