Tricuspid Atresia
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Tricuspid Atresia
1. Definition
Congenital cardiac malformation in which the right
atrium fail to open into a ventricle through an A-V valve.
The atrial situs is almost solitus in association with a
ventricular D-loop, and right ventricle is hypoplastic.
2. History
Kuhne :
Bellet
:
Glenn :
Fontan :
Kreutzer :
Recognized entity in 1906
Description of clinical feature in 1933
Glenn shunt in 1958
Successful repair in 1968
Modification in 1973
Tricuspid Atresia
Pathophysiology
• Atretic tricuspid valve, hypoplastic right ventricle, ASD,
VSD and restricted pulmonary blood flow(70%) lead to
right-to-left shunting and cyanosis.
• In patients with unrestricted pulmonary blood flow
(30%), pulmonary overcirculation and congestive heart
failure develop.
• Manifestations of left ventricular volume overload (e.g.,
ventricular dilation, mitral regurgitation, ventricular
dysfunction) result from combined systemic and
pulmonary venous return.
Tricuspid Atresia
Hemodynamics
•
•
Deoxygenated blood
Oxygenated blood
Tricuspid Atresia
 Surgical Morphology
1. Types of atresia
. Muscular : 75%
. Membranous
Fibrous diaphragm
Ebstein variant
A-V canal defect variant ; rarer variant
2. Origin of great arteries
. Ventriculoarterial concordant
: 60~70%
. Ventriculoarterial discordant : 30~40%
. DORV, DOLV, single outlet with truncus : rarely
3. Others
. Abnormality of mitral valve & LV ; high
. Juxtaposition of atrial appendage in 11%
Classification of Tricuspid Atresia
1. TA with normal origin of great arteries (type I)
. ASD always (4% restrictive)
Uncommonly ostium primum defect
. Obstruction to pulmonary blood flow : 85% (atretic in 10%)
. LSVC (15%), LSVC to LA (1~5%)
2. TA with transposition of great arteries (Type II)
. D-malposition, uncommonly L-malposition (Type III)
. ASD always (small in 50%)
. Lt. atrial juxtaposition (10%), CoA (30%), IAA (rare)
. Pulmonary blood flow
Usually large flow
Subpulmonic stenosis (20~30%, rarely atretic)
Classification
of TA
Most frequent
Clinical Features & Diagnosis
1. Symptoms & signs
.
.
.
.
Usually cyanosis, dyspnea, hypoxic spell depends on severity of PS
Clubbing in childhood
Excessive pulmonary flow with mild cyanosis & CHF in minority
Harsh ejection systolic murmur in most patients
2. Chest radiography
. Variable, depends on pulmonary blood flow
3. Electrocardiography
. LAD, LVH, abnormal P-wave
4. Echocardiography
5. Cardiac catheterization & cineangiography
Natural History of TA
1. Incidence
1~3% of CHD
2. Survival
1) Normal origin of great arteries
. With PS : 10% survival in
one year
Rapid narrowing of VSD :
progressive cyanosis
. Without PS : 10% survival
in 10 years
Congestive heart failure
Narrowing of VSD :
increasing cyanosis
2) Transposed great arteries
. Without PS : almost dead
in one year
Tendency to close of VSD :
subaortic stenosis
. With PS : 50% survival
in 2 years
Narrowing of VSD is slower
3. Modes of death
. Hypoxia, LCO in young
. Volume overload & hypoxia :
cardiomyopathy in old
Tricuspid
Atresia
Muscular atresia
Tricuspid
Atresia
RV
LV
VSD
Tricuspid
Atresia
Membranous atresia
Operative Techniques of TA
1. General plan of Fontan operation
. Risk of thrombosis around catheter
. Coronary sinus to pulmonary venous chamber
. Pericardiopleural window
2. Procedures
.
.
.
.
.
.
Systemic-pulmonary artery shunting
Pulmonary artery banding
Bidirectional superior cavopulmonary shunt
Hemi-Fontan operation
Fontan operation by right atrial-pulmonary artery connection
Fontan operation by total cavopulmonary connection
with or without incomplete atrial partition
. Total cavopulmonary connection with extracardiac conduit
Repair for Systemic Obstruction
• Repair of hypoplastic aortic arch and pulmonary trunk
band placement via thoracotomy
Repair for Systemic Obstruction
• Construction of proximal pulmonary trunk to aortic anastomosis
( Damus-Kaye-Stansel operation ) for systemic outflow tract obstruction
Repair for Systemic Obstruction
• Direct relief of subaortic obstruction in the univentricular
heart with transposed great arteries ( VSD extension )
Bidirectional Cavopulmonary Shunt
Hemi-Fontan Operation
Fontan Operation
Lateral Tunnel
Fontan Operation
Extracardiac Conduit
Results of Palliative Operation
1. Systemic pulmonary artery shunting
2. Pulmonary artery banding
3. Glenn operation
Pulmonary A-V fistula
4. Incomplete ( partial ) Fontan operation
BCPC ( SVC, IVC )
Hemi - Fontan operation
Fontan operation with deliberately incomplete
atrial partitioning
Results of Fontan Operation
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
Survival ; Early & time-related survival
Modes of death ; Early & Late
Incremental risk factors for death
Functional & hemodynamic status
Cardiac rhythm
Abnormality of pulmonary circulation
Protein-losing enteropathy
Thromboembolic complication
Neurologic complications
Desaturation after Fontan operation
Reoperation
Incremental Risk Factors for Death
1.
2.
3.
4.
5.
6.
7.
8.
9.
Acute ventricular decompression
Late pulmonary & ventricular deterioration
Age at operation
Cardiac morphology
Pulmonary artery size
PA pressure & PVR
Ventricular hypertrophy
Atrial isomerism
RA-PA connection technique
Operative Indications for TA
1. Indications for palliative procedure
. Shunt procedure
. Banding
2. Indications for Fontan operation
Indicated in whom one ventricle is too small or dysplastic or
both, be unable to generate pulmonary or systemic blood flow
. Optimal Age : 18 ~ 30 months of age (early : 10~12 months)
. Hemi - Fontan or BCPC around 6 months of age
. PVR 4units more : contraindicated
. Ventricular end-diastolic pressure of greater than 15mmHg ,
important A-V valve incompetence , or EF of less than 45%
raises a question of suitability
Special Situations & Controversies
• Types of operation for tricuspid atresia
• Valved Fontan pathway
• Persistent left superior vena cava with
hemiazygos extension of inferior vena cava
No longer Kawashima variant
• Ideal Fontan technique
Currently, extracardiac conduit or lateral
tunnel Fontan variants are preferred