COR TRIATRIATUM

Edited by_ HAPPY MALIK (3rd

year student)
 COR TRIATRIATUM
1.DEFINATION
•  Cor triatriatum (or triatrial heart) is a congenital heart defect where the left atrium
( cor triatriatum sinistrum) or right atrium ( cor triatriatum dextrum) is subdivided by
a thin membrane(fibromuscular membrane), resulting in three atrial chambers (hence the
name).

2. HISTORY
• Church : 1st description in 1868
• Borst : The term of cor triatriatum in 1905
• Miller et al : Angiographic diagnosis in 1964
• Ostman-Smith : Description by Echo. In 1984
• Vineberg & Gialloreto : 1st surgical correction in 1956
 CORTRIATRIATUM NATURAL
HISTORY
1. Rare congenital cardiac anomaly

2. Natural history depends on effective size of hole in the partition between the
common pulmonary venous chamber and left atrium
3. Mostly, the hole is severely restrictive and about 75% die in infancy without
treatment
4. When communicates with right atrium through ASD, the prognosis is better
( depend on the size of hole).
 PATHOPHYSIOLOGY
• Persistence of a common pulmonary vein forms an accessory left atrial chamber
called cor triatriatum sinister that communicates with the true left atrium via the
restrictive fibro muscular diaphragm. Pulmonary venous return is restricted,
resulting in pulmonary venous congestion and hypertension, right ventricular
hypertrophy, and congestive heart failure.
• Cor triatriatum dextrum (means in right atrial chamber) is extremely rare and
results from the complete persistence of the right sinus valve of the embryonic
heart. The membrane divides the right atrium into a proximal (upper) and a
distal (lower) chamber. The upper chamber receives the venous blood from both
vena cavae and the lower chamber is in contact with the tricuspid valve and the
right atrial appendage.
HOW IT LOOKS!!!!
 The transthoracic 
echocardiogram shows the membranous structure
dividing left atrium into two chambers in long-axis view (a)
and four-chamber view (b). Ao, aorta; LV, left ventricle; LA,
left atrium; RA, right atrium; RV, right ventricle.
COR TRIATRIATUM

1. Prevalence

 - 0.1-0.4% of coronary heart disease Equal frequency in both sex Isolated : 33-50%

2. Embryology

- Dorsal eventration of common atrium (from Common pulmonary vein), enlarges

to join pulmonary segment of splanchnic plexus.

- Malformations result from abnormal growth or regression of common pulmonary vein.

3. Types

• Communication with LA (classic)

a. With intact atrial septum
b. CPVC (Catecholaminergic polymorphic ventricular tachycardia)
communicating with RA
c. Atrial defect into lower chamber

• No communication with LA (imperforate diaphragm) Drainage into RA Drainage into coronary sinus
Drainage into systemic vein
MAJOR TYPES OF COR
TRIATRIATUM
 CLASSIFICATION OF COR
TRIATRIATUM
• cor triatriatum is divided into 3 distinct groups: type 1 has no opening in the accessory
membrane, with the proximal left atrium draining into the right atrium; type 2 has one or
more small restrictive openings (fenestrations), resulting in significant left ventricular
inflow obstruction; and type 3 has a large (nonrestrictive) opening in the membrane.

• No opening or 1 or more restrictive opening is commonly seen in the newborn, infants,

and children. In the adult, cor triatriatum often contains a relatively wide opening.
 CLINICAL MENIFESTATION

❑… It depend upon the size of the opening in the septum and the presence of associated
congenital cardiac defects. In most patients, the opening is severely restrictive and
produces symptoms similar to those of severe mitral stenosis
 CLINICAL FEATURES AND
DIAGNOSIS
1. Patients with small opening present with evidence of LCO ( pallor, tachypnea, poor
peripheral pulse, growth failure with pulmonary venous hypertension)

2. When there is associated Lt. to Rt. Shunt, evidence of pulmonary overcirculation and
venous obstruction may be present in X-ray, and right ventricular enlargement is prominent

3. Signs and symptoms of pulmonary venous hypertension

4. Diagnosis can be suspected by echocardiography, MRI

 PULMONARY VENOUS
CONNECTION
 Patterns of malformation:

1. Total anomalous pulmonary venous drainage

The common pulmonary vein fails to establish communication with pulmonary venous plexus,
then pulmonary vein to systemic venous connection will persist.

2. Atresia of common pulmonary vein

The common pulmonary vein is obliterated after the venous channels have disappeared.

3. Cor triatriatum sinister

After connection between atrium and confluence of pulmonary veins accomplished by common
pulmonary vein, abnormal incorporation into left atrium causes stenosis or obstruction.
 INDICATIONS FOR OPERATIONS

1. Urgent indication for restrictive aperture

2. Operation is necessary in the 1st year of life

3. In older patients with chronic symptom, operation is also urgently indicated.

4. In complex cor triatriatum, operation is indicated on an urgent basis.

 TECHNIQUES OF OPERATION

 1. Typical cor triatriatum

Diaphragm is excised to make an opening as large as possible and the
opening in the atrial septum closed.

2. Atypical cor triatriatum

Combination of typical repair, and anomalous pulmonary venous connection.
OPERATIVE VIEW OF COR
TRIATRIATUM
 Operative Results

1. Early death
Uncommon, but occurs in critically ill with inadquate myocardial management

2. Survival
Approaches that of general population and good functional result

3. Complications
● Restenosis due to inadequate resection
● Pulmonary vein stenosis
 COR TRIATRIATUM DEXTER
1. Definition
A rather unusual congenital cardiac malformation in which a persistent right valve of sinus
venosus subdivides right atrium

2. History
Rokitansky : 1st description in 1875

3. Embryology
Right horn of sinus venosus is incorporated into right atrium, giving rise to orifice of the SVC
and IVC. At one point the right valve of sinus venosus nearly divides right atrium, the valve
regresses and moves caudally, leaving the crista terminalis, and the valve of IVC (Eustachian)
and coronary sinus (Thebesian).
 CLINICAL FEATURES

1. pathology
Some minor degree of septation of right atrium by the remnant of right valve of sinus venous is
well tolerated, and a prominent eustachian valve is common finding

2.clinical presentation
● Somewhat variable
● Asymptomatic in vast majority
● Recurrent supraventricular tachycardia
● Right heart failure in symptomatic patients
● Cyanosis in the presence of ASD
COR TRIATRIATUM DEXTER
COR TRIATRIATUM   may be associated
with major congenital cardiac lesions  

 1. Classical morphology

● Thick common pulmonary vein chamber & thin distal LA chamber
● One or more openings, thick and fibromuscular, or tubular
● RV enlarged due to Lt. to Rt. shunt
● LV usually normal or small
● Foramen ovale usually patent and stretched
● PAPVR (Partial anomalous pulmonary venous return ) may coexist
2. Relationship to a left SVC
● Frequently than other type of CHD,
● Theory of impingement(a painful condition caused by rubbing or pressure on a tendon, nerve,
etc., by adjacent structures.) of SVC on developing LA

3. Associated anomalies
● PAPVR, TAPVR (total….)
● Unroofed coronary sinus with Lt SVC
● VSD (ventricular septal defect, (Coarctation of the aorta is a narrowing of the aorta,), AV canal
(Complete atrioventricular canal (CAVC) is a severe congenital heart disease in which there is
a large hole in the tissue (the septum) that separates the left and right sides of the heart. The hole
is in the center of the heart, where the upper chambers (the atria) and the lower chambers (the
ventricles) meet) , TOF ( tetralogy of fallot)
TETRALOGY OF FALLOT