Ebstein`s Repair in the Adult Patient

Surgical Strategies in Adult
Ebstein
Joseph A. Dearani, MD
AATS Toronto, April 2013
No Disclosures
Adult Ebstein FAQ’s
• Tricuspid - repair or replace
• Atrial arrhythmia
• Coronary disease
• Role of bidirectional Glenn
• PFO closure
Mayo Ebstein Experience
• Overall n=990
(~45% adults)
• Cone n=184
(oldest 70 years)
• Early mortality <2%
• Age 4 days – 79 years
• TV repair rate
• Children
• Adult
• BDCPA
>98%
>85%
~20%
Brown et al JTCVS 2008
Dearani et al ATS 2012
Dearani et al ATS 2013
Risk Factors for Mortality in
Adult Ebstein
Renal insufficiency
0.01
NYHA class IV
0.01
Mitral regurgitation
0.001
LV dysfunction
0.001
Brown, Dearani, et al. JTCVS 2008
The LV and Mitral Valve
• Mechanism of MR – structural ?
• Valve repairable ?
• LV function and size ?
• Coronary disease ?
Adult Ebstein
Diagnostic Studies
Echo – valve anatomy
MRI – ventricular size & function
Cath – hemodynamics, coronaries
Minimizing ischemic time
XC required
XC not required
(60-70 min)
(7 min)
• TV
• leaflet
delamination
augmentation
• septal reattach
• annular reattachment
• RV plication
• ring placement
• ASD closure
• BDCPA
• valve testing
• TV replacement
• reduction atrioplasty
Surgical Delamination
RV Plication
AL
SL
Mayo Cone Modifications
Reinforce inferior annulus
Severe RV & Annular
Dilatation
Mayo Cone Modifications
Patch augmentation + Sebening stitch
TV Replacement
•
•
•
•
Low threshold when > 60 years old
Elevated pulmonary artery pressures
Severe RV, TV annular enlargement
Bioprosthesis - porcine – not pericardial
• Coumadin for 3 months, ASA forever
• Mechanical – rarely; avoid with poor RV function,
target INR 3-4
TV Replacement for Ebstein
Porcine
CS
Suture line on atrial side of annulus
Maze Lesions
• Atrial flutter – cavotricuspid isthmus
• Paroxysmal Afib – right-sided
• Continuous Afib - biatrial
Coronary Disease in Ebstein
• If significant cardiomegaly, then…
• LAD disease – LIMA
• Circumflex and right coronary
preop PCI
©2011 MFMER | slide-18
Survival by LIMA Graft in ACHD
Survival (%)
Yes
No
p=0.006
Follow-up time (year)
Stulak et al Ann Thorac Surg 2009
Ebstein - Bidirectional Glenn
 venous return to RV
•
•
LV preload
LVEDP
< 12 mmHg
TPG
< 10 mmHg
MPAP
< 18 mmHg
BD Glenn in Ebstein
• Severe
RV enlargement
• CT ratio > 0.7
• RV EF < 25%
• n=62; oldest = 57 years
• Low LV EF (n=10; none LV dilatation)
• EF normalized at follow-up
Raju, et al, Ann Thorac Surg 2014 in press
Role of Atrial Septal Fenestration
• R  L shunt provides LV preload
• No significant RV unloading
• penalty is cyanosis (indication for op)
• neonate routinely
• adult rarely (paradoxical embolism)
• adults,  LV EF – cath LVEDP & PAP
Operative Strategies
• Epi-milrinone; Norepi-NTG – slow wean
• Cautious volume administration
• Faster HR (>100-120; A-pace)
• ACE inhibitor, + sildenafil x 3 mo
• β-blocker vs amio for 3 mo (plication)
• Arrhythmia surveillance
Adult Ebstein FAQ’s
• Lower threshold to replace valve
• Maze frequent, ? prophylactic
• Coronary disease - hybrid
• BD Glenn selectively
• PFO closure routinely