Download Morphology of Cor Triatriatum

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
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
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
abnormal growth or regression
Drainage into coronary sinus
of common pulmonary vein.
Drainage into systemic vein
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
LV
Diaphragm
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 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
Excised daiphragm
Diaphragm
Opening
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 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
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