test 5 Ebstein's Malformation/Anomaly

Question 1

Ebstein’s Malformation /Anomaly

Answer 1

• “Atrialized RV” (tricuspid valve lower than normal = leaflet problems)
   Rare congenital anomaly
   0.5% of all Congenital Heart Diseases
   Cyanotic Legion
  - Because is just pumps right back into the atrium and very little goes into pulmonary artery
   Tricuspid valve leaflets placed below annular ring
   Have an enlarged sail-like anterior leaflet

Question 2

Ebstein’s Anomaly orientation

Answer 2

 Orientation of the valve divides the RV into 2 parts
 Proximal atrialized RV
 Portion of the RV on the atrial side of inferior displaced tricuspid valve
 Thin walls
 Distal functional RV
 Small
 PFO/ ASD is common

Question 3

Ebstein’s Anomaly - Symptoms (Clinical presentation)

Answer 3

 Anatomic severity is variable
 TV Insufficiency
         Possibly combined with stenosis
 RV and RA dysfunction
         Results in cyanosis
         RV failure
 Wide range of symptoms
         Dyspnea, Cyanosis, Clubbing
 Arrhythmias are common
         Cause of sudden death

Question 4

Ebstein’s Anomaly – In Neonate

Answer 4

 Neonatal presentation
 Cyanosis due to RV dysfunction
 Requires PDA patency for pulmonary blood flow
 PV does not open (normal formation) due to inability of RV to generate pressure in excess of PA pressure
 Functional pulmonary valve atresia
 Venous return to the heart goes thru an ASD/PFO to the LA.

Question 5

Ebstein’s Malformation/ Anomaly repair

Answer 5

 Surgery is indicated with symptoms
 Repair:
 Ideally – want to create normal functioning tricuspid valve and close the atrial communications.
 ie. Create complete separation of pulmonary and systemic circulations

Question 6

Postnatal correction of Ebstein’s

Answer 6

 Repair (Bi-ventricular)
 Plicate the atrialized portion of the RV
 Reconstruct the Tricuspid valve annulus
 Close the ASD
 Resect the redundant atrial wall.

Question 7

Neonatal correction of Ebstein’s

Answer 7

 Repair described by Starns, et al. (CHLA) (Univentricular)
 Tricuspid valve orifice is closed with a patch
 Patch is often fenestrated
 Careful of the conduction pathways
 Create unrestricted flow across the ASD
 Resect the septum
 Plicate the redundant atrialized RV tissue
 Divide the PDA
 Pulmonary blood flow provided via systemic to PA shunt
 Bidirectional Glenn shunt and eventually and Fontan completion