Echocardiograpnhic Evaluation of Ebstein Anomaly: Definition

Echocardiograpnhic Evaluation of Ebstein Anomaly: Definition

Echocardiograpnhic Evaluation of
Ebstein Anomaly:
Definition, Detection and
Determinants of Outcome
P. W. O’Leary, M.D.
Division of Pediatric Cardiology
Mayo Clinic
No Conflicts to Disclose
What is Ebstein Anomaly?
• Failure of the TV to “de-laminate” from the RV
• An abnormality of both myocardial and valvular
development
• Affects RV & TV >> LV & MV
• Ventricular Dysfunction is nearly universal
• Anterior and Apical Rotation of the Functional
Orifice
• Echocardiographic Manifestations
• Abnormal TV attachment/displacement in RV
• Tricuspid Regurgitation
• Associated RV “Myopathy” (sometimes LV too)
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Normal Delamination (Separation)
of the TV from the RV Myocardium
Spectrum of Failed TV Delamination
seen in Ebstein Anomaly
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Displacement of the
TV Functional Orifice
Rotational – Anterior & toward the RVOT
Not just toward the apex
Rotational Displacement of the TV
orifice in Ebstein Anomaly
3
The Valve and Its Orifice has been
displaced anteriorly and apically
toward the RVOT
Failed Delamination
The Valve and Its Orifice has been
displaced anteriorly and apically
toward the RVOT
Failed Delamination
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3 Dimensional Echo
Ebstein Anomaly – Functional Orifice
3 Dimensional Echo
Ebstein Anomaly – Functional Orifice
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Sometimes it is Easy to Detect
Ebstein Anomaly
Failed Delamination
Sometimes its not …
Use the Apical Displacement
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Recognizing Ebstein Anomaly
Apical Displacement Index
Ebstein Anomaly
Apical Displacement Index
Normal
< 8 mm/m2
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Ebstein Anomaly
Apical Displacement Index
RA
LV
RV
Normal
< 8 mm/m2
Ebstein Anomaly
23mm/1.5 m2 = 15.3 mm/m2
Ebstein Anomaly vs. TV Dysplasia
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Ebstein Anomaly vs. TV Dysplasia
ADI – 11 mm/m2
ADI – 6 mm/m2
Determinants of Outcome
• Mortality – early and late
• Ventricular Performance
• Valve Repair vs Replacement
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Ebstein Anomaly
Mortality in the Neonate
• Hydrops Fetalis
• Ventricular Dysfunction
• Consider 1.5 ventricle repair for severe
RV dysfunction with preserved LV fxn
• Massive Cardiac Enlargement
• Impact on Lung Mechanics
• Celermajer Index (apical 4)
• Ratio of areas
RA+aRV to RV+LA+LV
• Value > 1 suggests a poor prognosis
in the newborn
Celermajer Index in Ebstein Anomaly
Two children with Ebstein Anomaly
Which will have the better outcome:?
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Celermajer Index in Ebstein Anomaly
CI < 1
CI >> 1
Two children with Ebstein Anomaly
Ebstein Anomaly
Features Associated with NonNon-neonatal
Mortality (’72 –’06)
•
•
•
•
•
•
•
Need for post op ECMO/VAD (HR – 18)
RVOT or PA stenosis (HR – 5)
MR requiring surgical intervention (HR – 3)
Prior cardiac surgical procedure(s)
> Moderate RV dysfunction (HR – 3)
↑’d Hct values (cyanosis, HR - 2)
TV replacement (HR – 2)
The outcomes of operations for 539 patients
with Ebstein anomaly. Brown, et al. JTCVS 2008
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Ebstein Anomaly
Features Associated with NonNon-neonatal
Mortality (’72 –’06)
•
•
•
•
•
•
•
Need for post op ECMO/VAD (HR – 18)
RVOT or PA stenosis (HR – 5)
MR requiring surgical intervention (HR – 3)
Prior cardiac surgical procedure(s)
> Moderate RV dysfunction (HR – 3)
↑’d Hct values (cyanosis, HR - 2)
TV replacement (HR – 2)
The outcomes of operations for 539 patients
with Ebstein anomaly. Brown, et al. JTCVS 2008
Ebstein Anomaly
Selected* Features Associated with
Late Reoperation (’72 –’06)
• MV regurgitation requiring surgical intervention
(HR 7.1)
•
•
•
•
Age at operation < 12 years (HR 3.2)
Post operative RV dysfunction (HR 2.4)
Preoperative LV dysfunction (HR 2.3)
RVOT or PA stenosis (HR 2.3)
*Excludes features related to dysrhythmias
The outcomes of operations for 539 patients
with Ebstein anomaly. Brown, et al. JTCVS 2008
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Ebstein Anomaly
Late Ventricular Performance
• Predictive of declining • Not associated with
function
•Length of Follow up
•Preoperative RV
function (FAC)
•TV replacement
(instead of repair)
late ventricular
function
•TVR position
relative to the CS
Ebstein Anomaly
Valve “Repairability”
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Ebstein Anomaly
Components of Classic Monocusp
Valve “Repair”
• ASD Closure
• Right Reduction
Atrioplasty
• Anuloplasty
• + RV elliptical
excision/plication
• + Papillary
advancement
• + Anti
Anti--arrhythmia
procedures
Ebstein Anomaly
Features Favoring Successful Monocusp Repair
• Freely mobile Anterior TV Leaflet
• Body of Leaflet and the Leading Edge
can reach the septum
• No Direct papillary muscle insertions
• Mobility is Best Assessed in Apical 4
Chamber (at the level of the mitral valve/RV
inflow)
• Single Central Jet of TR
• No TV Chordal attachments in the RVOT
• Adequate Postop Functional RV size
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Ebstein Anomaly
Favorable Findings and Outcome
Preop at age 12
Post Monocusp
Repair
No additional intervention – 9 yrs
Ebstein Anomaly
Unfavorable Features for Monocusp Repair
• Tethered Anterior Leaflet with
restricted mobility
• Body of Leaflet and the Leading Edge
• Direct papillary muscle insertions onto
valve tissue (no chordae)
• Mobility is Best Assessed in Apical 4
Chamber (at the level of the mitral valve)
• Multiple Jets of TR (fenestrations)
• TV Chordal attachments in the RVOT
(near the PV)
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Ebstein Anomaly
Unfavorable Findings
Ebstein Anomaly
Unfavorable Findings
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Ebstein Anomaly
Unfavorable Findings
Ebstein Anomaly
Unfavorable Findings
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Ebstein Anomaly
Unfavorable Findings
Ebstein Anomaly
Unfavorable Findings
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Ebstein Anomaly
Features Favoring Successful
“Cone” Reconstruction
• The Rules Have Changed
• Mobility of the Anterior Leaflet is
•
still key to success, but
“Surgical Delamination” can now
increase the amount of mobile
tissue available for repairs
Cone Reconstruction of the TV in
Ebstein Anomaly
“Surgical Delamination”
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2 year old boy with
Ebstein Anomaly
2 year old boy with
Ebstein Anomaly
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2 year old boy with
Ebstein Anomaly
Preoperative
One year Post-operative
2 year old boy with
Ebstein Anomaly
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2 year old boy with
Ebstein Anomaly
More anatomic repair than the monocusp
Long Term Outcome ??
10 year old girl with Ebstein Anomaly
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10 year old girl with Ebstein Anomaly
10 year old girl with Ebstein Anomaly
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10 year old girl with Ebstein Anomaly
Successful Cone Reconstruction
10 year old girl with Ebstein Anomaly
Successful Cone Reconstruction
Predicting successful valve reconstruction - ?
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Determinants of Outcome
- we are still learning
• Can we repair the Valve?
• Mobile ATL tissue is helpful
• Presence of Septal Leaflet
improves the geometry of
the “cone”
• Creation of SVC – RPA
connections allow a smaller
“cone”
Echocardiograpnhic Evaluation of
Ebstein Anomaly:
Definition, Detection and
Determinants of Outcome
P. W. O’Leary, M.D.
Division of Pediatric Cardiology
Mayo Clinic
No Conflicts to Disclose
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