Transcript Anatomic Types of Ebstein`s Anomaly

Ebstein’s Malformation
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Ebstein’s Malformation
1. Definition
Congenital defect of tricuspid valve in which the origins of
the septal or posterior leaflet or both, are displaced downward
into the right ventricle, & the leaflets are variably deformed.
Characteristically, the anterior leaflet is enlarged and sail-like.
2. History
Ebstein
: Description in 1866
MacCallum
: 1st description in English literature in 1900
Van Lingen, Soloff : Diagnosis during life in 1951
Lev
: WPW syndrome in 1955
Gasul, Scott
: Glenn operation in 1959
Barnard, Schrire
: Prosthetic valve replace in 1962
Hardy
: 1st successful valvuloplasty in 1964
Ebstein’s Malformation
Pathophysiology
• Downward displacement of the posterior and septal
leaflets of the tricuspid valve and atrialization of the right
ventricle leads to diminished right ventricular output and
congestive heart failure, stemming from tricuspid
insufficiency and/or right ventricular dysfunction.
• High right atrial pressures from tricuspid insufficiency
and high pulmonary vascular resistance during early
postnatal period result in right-to-left shunting across an
interatrial communication leading to varying degrees of
cyanosis.
• An increased incidence of accessory conduction pathways
leads to paroxysmal supraventricular arrhythmias.
Ebstein’s Malformation
Ebstein’s
Malformation
TV
Septal leaflet
Anatomic Types of Ebstein’s Anomaly
1. Small, contractile, atrialized chamber with
a mobile anterior leaflet
2. Large, noncontractile, atrialized chamber
with a mobile anterior leaflet
3. Restricted motion of the anterior leaflet
4. “Tricuspid sac”
Leaflet tissue forms a continuous sac
adherent to the dilated right ventricle.
Types of Ebstein’s Anomaly
Ebstein’s Malformation
Surgical Morphology
1. Tricuspid valve
2. Right ventricle
3. Right atrium
4. Left ventricle
5. The lung
6. Associated defects ;
ASD, Pulmoanry atresia or stenosis, VSD,
TOF, PDA, TGA, CoA, Congenital MS
Clinical Features & Diagnosis
1. Mechanism
.
.
.
.
Right ventricular dysplasia & dysfunction
Tricuspid valve incompetence
Pulmonary hypoplasia
Wolff-Parkinson-White syndrome
2. Symptoms & signs
.
.
.
.
3.
4.
5.
6.
Breathlessness, cyanosis, cardiomegaly during the 1st week of life
Cyanosis (1/2) : onset in infancy and early childhood
Palpitation, malar flush (mitral facies) in 1/3
Wide splitting of the 1st heart sound
Chest radiography : rounded or boxlike
EKG : RAH, low amplitude R-wave, WPW (5%)
Echocardiography
Cardiac catheterization & cineangiography
Radiographic Findings
• Cardiomegaly, Atrialized RV, TR
Pathologic Features
 Three primary pathologic features
predominate in patients with Ebstein anomaly
• RV abnormalities
• Tricuspid valve abnormalities
• Accessory conduction pathways
• Pulmonary hypoplasia
• Their severity & extent determine secondary
pathophysiologic features, clinical presentation,
and natural history. Also extent and severity
of these relate to age at presentation
Natural History
1. Incidence
. Less than 1% of CHD( 0.2~0.5%), equal in both sex,
rarely familial reported
2. Presentation during the 1st week of life
. Stillborn, cyanosis, CHF
. 20~40% die in newborn
3. Presentation in infancy
. Less risk of death or severe symptoms
4. Presentation in childhood & adult life
. Mild symptoms & prognosis is good
5. Modes of death
. CHF(1/2) . Sudden death(arrhythmia related WPW in 60%)
. Others (cerebral abscess, emboli)
Operative Techniques
1. Repair of the TV and ASD closure
2. Placement of tricuspid valve
About 20~30%
3. Simple ASD closure
Rarely indicated
4. Technique for neonate in extremis
Starnes Operation (TV closure, ASD creation,
systemic-pulmonary shunt)
5. One and a half ventricle repair
Operative Technique
(Danielson Method)
Operative Technique
Single-stitch Technique
Repair by Carpentier Method
Operative Technique
Carpentier Method
Tricuspid Valve Replacement
Surgical Results
1. Survival
Early ; acute cardiac failure
Late ; sudden death, rhythm disturbances,
chronic cardiac failure
2.
3.
4.
5.
Functional status
Tricuspid valve function after repair
Postoperative rhythm disturbances
Results of other procedures
1)
2)
3)
4)
Starnes operation
Repair of ASD alone
Tricuspid valvotomy
Pulmonary valvotomy
Ebstein’s Malformation
Advantages of ventricular unloading
1. Alteration in the tricuspid valve function
by a combination of geometric changes
in the right ventricle and valve apparatus
2. Improve Lt. ventricular function by
minimizing the deleterious effects of right
to left ventricular interaction
Thrombosis in Tricuspid Valves
Possible reasons of high incidence
• Peak velocity of rapid filling flow is low.
• Pressure half time or deceleration time is
prolonged.
• Velocity is liable to variation under the
influence of respiration.
• Difference of prostacyclin (PGI2) level in
arterial & venous blood (Lungs produce high
amount.)
Operative Indications
1. Neonates presenting in extremis
* Starnes procedure after stabilization
2. Valve repair & closure of ASD
* Patients with important TR, moderate cyanosis, or
symptoms of CHF and cardiomegaly
3. Simple repair of ASD
* Lt. to Rt. shunt with or without symptoms of CHF,
and no or little tricuspid valve incompetence
4. Ablation of accessory conduction pathway
* Division or ablation of accessory conduction with
concomitant repair
Special Situations & Controversies
1. Morphology
2. Tricuspid valve replacement
. Bioprosthesis
. Homograft
. Mechanical prosthesis
3. Indications for plication of atrialized ventricle in TVR
. Controversial in all cases
. When the atrialized portion of RV is very thin &
aneurysmal (10~20%) & when TV is repaired
4. Excision or plication of right atrial wall