What is Ebstein Anomaly?

Transcription

1 Echocardiograpnhic Evaluation of : Definition, Detection and Determinants of Outcome P. W. O Leary, M.D. Division of Pediatric Cardiology Mayo Clinic No Conflicts to Disclose What is? 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) 1

2 Normal Delamination (Separation) of the TV from the RV Myocardium Spectrum of Failed TV Delamination seen in 2

3 Displacement of the TV Functional Orifice Rotational Anterior & toward the RVOT Not just toward the apex Rotational Displacement of the TV orifice in 3

4 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 4

5 3 Dimensional Echo Functional Orifice 3 Dimensional Echo Functional Orifice 5

6 Sometimes it is Easy to Detect Failed Delamination Sometimes its not Use the Apical Displacement 6

7 Recognizing Apical Displacement Index Apical Displacement Index Normal < 8 mm/m2 7

8 Apical Displacement Index RA LV RV Normal < 8 mm/m2 23mm/1.5 m2 = 15.3 mm/m2 vs. TV Dysplasia 8

9 vs. TV Dysplasia ADI 11 mm/m2 ADI 6 mm/m2 Determinants of Outcome Mortality early and late Ventricular Performance Valve Repair vs Replacement 9

10 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 Two children with Which will have the better outcome:? 10

11 Celermajer Index in CI < 1 CI >> 1 Two children with Features Associated with Non-neonatal 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

12 Features Associated with Non-neonatal 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 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

13 Late Ventricular Performance Predictive of declining function Length of Follow up Preoperative RV function (FAC) TV replacement (instead of repair) Not associated with late ventricular function TVR position relative to the CS Valve Repairability 13

14 Components of Classic Monocusp Valve Repair ASD Closure Right Reduction Atrioplasty Anuloplasty + RV elliptical excision/plication + Papillary advancement + Anti-arrhythmia procedures 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 No TV Chordal attachments in the RVOT Adequate Postop Functional RV size 14

15 Favorable Findings and Outcome Preop at age 12 Post Monocusp Repair No additional intervention 9 yrs 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) 15

16 Unfavorable Findings Unfavorable Findings 16

17 Unfavorable Findings Unfavorable Findings 17

18 Unfavorable Findings Unfavorable Findings 18

19 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 Surgical Delamination 19

20 2 year old boy with 2 year old boy with 20

21 2 year old boy with Preoperative One year Post-operative 2 year old boy with 21

22 2 year old boy with More anatomic repair than the monocusp Long Term Outcome?? 10 year old girl with 22

23 10 year old girl with 10 year old girl with 23

24 10 year old girl with Successful Cone Reconstruction 10 year old girl with Successful Cone Reconstruction Predicting successful valve reconstruction -? 24

25 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 : Definition, Detection and Determinants of Outcome P. W. O Leary, M.D. Division of Pediatric Cardiology Mayo Clinic No Conflicts to Disclose 25