Cor Triatriatum

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

Cor Triatriatum

1. Definition A rare congenital cardiac anomaly in which the pulmonary

veins enter a proximal left atrial chamber separated from the

distal left atrial chamber by a diaphragm in which there are

one ore more restrictive ostia.

* Cor triatriatum sinister, Cor triatriatum dexter

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

Cor Triatriatum

Pathophysiology• Persistence of a common pulmonary vein forms

an accessory left atrial chamber that communicates with the true left atrium via the restrictive fibromuscular diaphragm.

• Pulmonary venous return is restricted, resulting in pulmonary venous congestion and hypertension, right ventricular hypertrophy, and congestive heart failure.

Drawing of Cor Triatriatum

1. Prevalence 0.1-0.4% of CHD

Equal frequency in both sex

Isolated : 33-50%

2. Embryology * Dorsal eventration of common

atrium (CPV), enlarges to join

pulmonary segment of

splanchnic plexus.

* Malformations result from

abnormal growth or regression

of common pulmonary vein.

3. Types 1) Communication with LA

(classic)

a. With intact atrial septum

b. CPVC communicating with RA

c. Atrial defect into lower chamber

2) No communication with LA

(imperforate diaphragm)

Drainage into RA

Drainage into coronary sinus

Drainage into systemic vein

Cor Triatriatum

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.

Morphology of Cor Triatriatum

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 may coexist

2. Relationship to a left SVC * Frequently than other type of CHD,

* Theory of impingement of SVC on developing LA

3. Associated anomalies * PAPVR, TAPVR

* Unroofed coronary sinus with Lt SVC

* VSD, COA, AV canal, TOF

Major Types of Cor Triatriatum

Cor Triatriatum

DiaphragmDiaphragmLVLV

Clinical Features & 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

Cor Triatriatum

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).

Techniques of OperationTechniques 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

DiaphragmDiaphragm

Excised daiphragmExcised daiphragm

OpeningOpening

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

Indications for Operation

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.

Cor Triatriatum DexterCor 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).

Cor Triatriatum Dexter Cor Triatriatum Dexter

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