Surgical Management of Ebstein’s Anomaly

By Ayman KhalifaResident of CTS

Atfal Masr Hospital (HIO)

Ebstein’s anomaly is known as:

-Apical displacement of septal and / or posterior leaflet from the insertion of anterior mitral leaflet by >8 mm/m² BSA-anterior leaflet is large and sail like-Atrialized portion of RV-ASD or PFO

Carpenteir classificationType A (minimal disease) Type B (intermediate

disease)small atrialized RV large true RVmoderate displacement of septal and /or posterior leafletanterior leaflet is large and mobile

large atrialized RVsmall true RVanterior leaflet is large and mobile

Type C (sever disease) Type D (tricusped sac lesion)

anterior leaflet is restricted in motion causing significant RVOT obstruction

RV is completely atrialized except small infundibular area

PathophysiologyThe sevsrity depend on

Degree of displacement Relation ( ) the anterior leaflet and RVOT Presence of associated cardiac anomaliesAt birth, PVR still near systemic resistance so, if there is

ASD there is right to left shunt leading to cyanosisIn patient with significant atrialization of RV and / or

RVOT obstruction,there is high incidence of cyanosis and congestive HF

Patient with sever cardiomegally and cardiothoracic ratio > ,65 usually don't tolerate to medical treatment

Surgical management

1-Valve replacement

(1) With transverse plication-Hardy

-Danielson

2-Valve repair

(2) With longitudinal plication

-Carpenteir-Quaegebeur

(4) cone reconstruction

3-Palliation-Starnes

(3) Without plication-Hetzer

1-Valve replacement -Usually prosthetic with a valve of diameter 31-33 mm-Leaflets and chordal attachment are left in place to preserve RV function-It is done without plication of the atrialized RV-plication is done in case of dilated aRV with normal tRV

Modified technique-The suture line is placed posterior to the AVN and around coronary sinus to avoid injury to the conduction system so, coronary sinus drain directly into RV

-aRV is not plicated but,if plicated it should be done with separate sutures away from the sutures of the replacement to avoid obstruction of coronary sinus

2-Valve repair

(1) With transverse plication-Hardy

-Danielson

(2) With longitudinal

plication-Carpenteir

-Quaegebeur(4) cone reconstruction

(3) Without plication

-Hetzer

Hardy technique Transverse plication of the aRV

Elevation of the septal and posterior leaflets to the level of true tricusped annulus

Daneilson ( modified hardy )

Posterior annuloplasty to reduce the diameter of true tricusped annulus

It is done at the level of coronary sinus to avoid injury to the conduction bundle

Carpenteir technique

Anterior and posterior leaflets are detached from the annulus Longitudinal plication of the aRV The leaflets are rotated in clockwise direction then reattached to the new small annulus Reinforcement of the annulus by ring

Quaegebeur (modified carpenteir )

Posterior annuloplasty without using ring

Hetzer Division of the true annulus into 2 orifices by a suture between anterior leaflet and opposite side in the septumThe anterior one will be the tricusped annulus and should be at least 2.5 cm in diameterPosterior annulus will be obliterated with sutures

N.BIf there is rugurge after testing we may narrow the annulus by suture at anteroseptal commissure

Cone reconstruction•Leaflet reconstruction•RV reduction by plication or resection•RA reduction•Creation of atrial lesions to prevent arrhythmia

Steps:*Detachement of leaflet : Started in anterior leaflet in clockwise and counter clockwise direction*Division of secondary chordal attachment and muscle bundle primary chordal attachment should be preserved *Leaflet lenghening the cut edges of posterior and septal leaflets are attached to each other*Annular and ventricular remodeling -the thin dilated part of RV is excised or plicated to remove the akinetic or dyskinetic part leading to increase RV efficiency -the annulus is reduced to match the size of new TV -reinforcement of the plicated annulus with ring

* Leaflet reattachement attachement of the reconstructed leaflet to the true annulus* Cryoablation for prevention and treatment of arrhythmia* ASD closure

3- palliation-Used in patient with functional PA due to : *Increase PVR *Sever TR-These patient are duct dependant-Palliation is done by central shunt (MBT) or peripheral shunt (BDG)-The results are unfavorable due to persistent TR Recently, creation of true TA has better results and the operation include (shunt + TA + ASD enlargement)

Thank you