1. IN THE NAME OF GOD

2. Valved-Skirt: A New Surgical Approach To Ebstein’s Anomaly in GUCHs Mohammad ABBASI-TESHNIZI Mathias H. AAZAMI Saeed Hafez Cardiac Surgery Department Imam Reza University Hospital

3. Anatomy of Ebstein I TV anomalies: Default of Delamination Downward displacement of Dysplastic Leaflets into Inlet- portion: Septal >> Posterior > >> Anterior Anterior Leaflet : Redundancy / Tethering / Dysplasia / Fenestration / Degenerative changes Abnormal Sub-valve apparatus Atrio-Ventricular Junction: Dilatation Anomalies of RV Functional Portion: thin and small reduced RV systolic function reduced trabecular portion Infundibular stenosis (functional TV stenosis) Artialized Portion WPW / multiple accessory pathways + + + Less than 1% of all congenital heart defects

4. Spectrum of anatomical severity

5. (90% success rate of repair, or valve replacement) (systolic function, size, and diastolic function) Is not just a surgical issue of TV competency (90% success rate of repair, or valve replacement) But: surgical strategy and outcomes depends mainly on functional RV status (systolic function, size, and diastolic function)

6. Chest radiography Echocardiography Cardiac cath MRI

7. Indication for operation Limited exercise capacity (NYHA III-IV).NYHA III-IV Increasing heart size (cardiothoracic ratio greater than 65%). Important cyanosis (resting oxygen saturation of less than 90%). (Level B)cyanosisoxygen saturation Severe tricuspid regurgitation with symptoms.tricuspid regurgitation Transient ischemic attack or stroke Transient ischemic attackstroke

8. Issue I: how enough is enough in individual surgical experience !!!! Issue II: an universal efficient technique ??? Issue I: how enough is enough in individual surgical experience !!!! Becomes even more rare in GUCHs due to antenatal diagnosis and earlier surgical management But, still a surgical dilemma in developing countries Issue II: an universal efficient technique ???

9. Knowledge of the long-term outcome in unoperated adult patients with Ebstein anomaly is limited, and the therapeutic approach is still controversial

10. Ebstein’s Anomaly in GUCHs: Surgical Imperatives Preservation of RV function: Avoiding diastolic dysfunction (adequate size) Relieving intra-RV obstruction ( functional TS ) Avoiding worsening systolic function (sub-valve apparatus /fibrous bands/ coronary entrapement)

11. Atrialized Portion Indications for plication are contraversial ?) source of dyskenisia ?) Increased thrombo-embolic risk ?) providing better RV size / enhanced diastolic function ?) Contributive to RV systolic function ?) Implications on LV function

12. Surgical considerations Primary Htpx Total Cavo-pulmonary Connections 1+ ½ RV (Glenn shunt) Corrective Surgery Functional RV status mandates surgical strategy

13. Surgical Strategy Functional RV Monitoring for RV failure Rescue 1+ ½ RV (Glenn shunt) Corrective Surgery Adequate Inadequate Secondary Htpx Corrective Surgery + 1+1/2 RV Secondary 1+1/2 RV Primary Htpx Corrective Surgery Follow-Up: Total Cavo-pulmonary Connections

14. Surgical correction: Current Techniques Regardless of TV repair or replacement, Surgical Procedure is classified: Supression of Atrialized Chamber Preservation of Atrialized Chamber

15. Corrective Surgery by TV Replacement Suppressing Atrialized Chamber Transverse Plication of the atrialized chamber Tricuspid Valve Replacement Preserving Sub-valve apparatus Coronary Sinus left on RA side Closure of ASD Risk of coronary entrapment CS

16. Corrective Surgery by TV Replacement Preserving Atrialized Chamber Respecting the atrialized chamber Tricuspid Valve Replacemment Coronary Sinus left on RV side Closure of ASD CS on RV

17. Corrective Surgery by TV Repair Jatene / Danielson Transverse Plicating the atrialized chamber Tricuspid Valve Repairing Segmental Annuloplasty Closure of ASD Risk of coronary entrapment

18. Corrective Surgery by TV Repair Carpentier Longitudinal Plicating the atrialized chamber Tricuspid Valve Repairing / Detaching + Sliding Anterior leaflet Ring Annuloplasty Closure of ASD Risk of Coronary Entrapment

19. Ebstein’s Anomaly: Valved-Skirt Technique / Case Male 23 y.o / Exercise intolerance / NYHA III / AF Echo: Severe RA + RV enlargement Apical Displacement of Septal Leaflet ≈ 1.8 cm Tethered Anterior Leaflet Atrio-Ventricular Junction : 8 cm in diameter Free TR

20. Valved-Skrit Technique / Case Male 23 y.o and female 14 y.o / Exercise intoleranc / NYHA III / AF MRI: Significant Anterior Leaflet Tethering Functional / Anatomical RV : 33% Functional RV : EF : 27% EDVI: 150 ml/m2 Anatomical RV: EF: 20% EDVI: 424 ml/m2 Atrio-Ventricular Junction : 8 cm in diameter LVEF: 40% / LVEDVI: 64 ml / m2

21. Ebstein’s Anomaly: Valved-Skirt Technique Home-made Valved-Skirt Using a Gore-Tex Patch and a Saint-Jude Bi- leaflet 33 mm Valve Prosthesis & Hancock biologic valve 27 mm

22. Valved-Skrit Technique Dilated RA RA Artrialized Dilated Atrialized Portion

23. Ebstein’s Anomaly: Valved-Skirt Technique Securing the Valved-Skirt at the Anatomical Atrio- Ventricular Junction Without any Anatomical changes on TV apparatus (securing the edge of Anterior Leaflet to free RV Wall if Sail type) Coronary Sinus left at RA side ASD / PFO closure

24. Valved-Skirt Technique Securing the Valved-Skirt at the Anatomical Atrio-Ventricular Junction Without any Anatomical changes on TV apparatus (securing the edge of Anterior Leaflet to free RV Wall if Sail type) Coronary Sinus left at RA side

25. Ebstein’s Anomaly: Valved-Skirt Technique Reductional RA-Plasty HLM: 60 min / Ao Clampage: 35 min

26. Ebstein’s Anomaly: Valved-Skrit Technique / Outcome Extubation < 8h ; no Inotrope / Sao2: 100% Uneventful Smooth Post-Operative Course Dramatic Improvement in Exertional Dyspnae ; NYHA I Reoperation at 21th P.O.D for Pericardial Effusion Remain in NYHA I at 60 th P.O.D

27. Ebstein’s Anomaly: Valved-Skrit Technique / Outcome Post-Op Echo @ 2 m P.O: Severe RA + RV enlargement Severe RV dysfunction Normal Prosthesis functioning : G max = 3.6 mmHg Post-Op MRI Pending

29. Ebstein’s Anomaly: Valved-Skirt Technique / Conclusions AA promising / every Surgeon procedure  Simple, Efficient, & Expeditive Corrective Procedure BBetter dealing with conduction pathway and the Coronary Sinus DDo not change the native anatomy: better preservation of RV function ??? PPossibility of later conversion to TV Repairing DDrawback: long-life anti coagulation / Prosthesis Dysfunction NNeeds further validation

30. Thanks for your patience