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Jose Pedro da Silva, MDHospital Beneficencia Portuguesa of Sao Paulo, Brazil
Ebstein’s anomaly
AATS SPECIAL INVITED LECTURE
Wilhelm Ebstein : “Concerning a very rare case of
insufficiency of the tricuspid valve caused by a congenital
malformation.” Arch Anat Physiol 1866.
Ebstein’s anomaly first report
•Downward displacement of theseptal and posterior leaflets of thetricuspid valve
•The anterior leaflet is redundant andpresents a sail like format
•Dilation of the true tricuspid annulus
TV regurgitation and dilation of theRA and RV
Ebstein’s anomaly
Ebstein’s anomaly:
Various degrees of failure in
tricuspid valve leaflets
delamination, resulting in a
more distal attachment of them
in the RV
Van Mierop & Gessner, Prog Card Dis,1972;15:67-85
EBSTEIN’S ANOMALY – EMBRYOLOGICAL ASPECTS
Tricuspid
leaflet
formation
in Ebstein’s
anomaly
“The presence of a free
leading edge is important for a
successful, durable repair.”G. K. Danielson
Linear attachment of the anterior leafletFree leading edge
EBSTEIN´S ANOMALY – ANATOMICAL VARIATIONS
No RV dilation, mild TR, mobile AL Enlarged RV, grade 3 TR, restricted AL
EBSTEIN’S ANOMALY – THE CARPENTIER’S CLASSIFICATION
Carpentier et alJ T C S 1988; 96:92-101
Type A: volume of true RV is adequate,
mobile AL
Type B: large atrialized RV, mobile AL
Type C: AL restricted, + RVOTO
Type D: complete atrialization of RV
Varieties of Surgical Repairs for EA
Sebening
Knott-Craig
Quaegebeur
HetzerStarnes
Kaushal
Ullmann Wu
Meisner
Chauvaud
Danielson
Vargas
Friesen
Schmid
Ross
Kaneko Sano
Hardy
Procedure Patients (N) Age Mean (range)
Hospital Death
TV repair 52 (28.3%) 7.1± 3.9 years ( 5 m to 12y) 3(5.8%)
TV replacement 117 (63.6%) -
Other 17
Tricuspid Valve Repair for Ebstein’s Anomaly inYoung Children: A 30-Year Experience(N=184)
(MAYO CLINIC)
Boston, U et al: Ann Thorac Surg 2006;81:690–6
J Thorac Cardiovasc Surg 2008;135:1120-36
EA patients than 12y EA patients age equal or greater than 12y
Ebstein’s Anomaly: the Techniques of Carpentier andQuaegebeur
Carpentier J T C S 1988;96:92-101, Quaegebeur J ACC 1991;17:722-8.
Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association
Sarris G. E. et al.; JTCVS 2006;132
Surgical procedures
valve replacement
valve repair 1 1/2 ventricle repair
palliative shunt
other complex procedures
(n= 179) 60 (33.5%) 49 (27.3%) 46 (25.6%) 13 (7.26%) 11 (6.14%)
Hospital Mortality
9.2% 7.1% 16.6% 75% 25%
179 patients from 13 centers Ages ranged from 1 day to 48.3 years (mean 9.5 ± 10.2 y , median 6 years).
Ebstein’s anomaly - The Cone Procedure
1993- We have developed an operation intended to be a single
strategy to approach the wide variety of anatomical presentations of
Ebstein’s anomaly. This operation main surgical concepts are:
Mobilization of the tricuspid valve by cutting all its abnormal
attachments to the right ventricle
Creation of a cone-like structure from all available leaflet tissue.
This technique allows the TV to close by leaflet to leaflet coaptation
and contrasts with the previous procedures that result in a monocusp
valve coapting with the ventricular septum.
Arq Bras Cardiol 2004;82:2 J T C S. 2007;133:215-23 1
Anatomy of the Tricuspid Valve
Image courtesy ofProf Vera Aiello ,INCOR
McCarthy .Operative Techniques in TCVS 2011; 16:97-111 (
POSTOP
.
CIA
PREOP
Modified from Dearani J, Bacha E, Da SilvaJP. Operative Techniques CVS 2008
EBSTEIN’S ANOMALY – THE CONE REPAIR
Small septal leaflet and linear attachment of the posterior leaflet
EBSTEIN’S ANOMALY – THE CONE REPAIR
Dearani J, Bacha E, Da SilvaJP. Operative Techniques CVS 2008
EBSTEIN’S ANOMALY – THE CONE REPAIR
Small septal leaflet is combined wit the posterior leaflet
EVENTS NUMBER
Significant findings 29 (69%)
Free of significant findings 13
Catheter ablation during EPS 17
EPS guided one or more intraoperativerhythm interventions
12
Preoperative Electrophysiologic Studies in 42 Ebstein's anomaly
patients out of 74 underwent the Cone procedure Boston Children's Hospital , Dec 2006 –Sep 2012
Shivapour JK et al.Heart Rhythm. 2014 Feb;11(2):182-6
Ebstein’ anomaly: Associated conduction system abnormalities
Ho Sy et al, heart 2000Image courtesy ofProf Vera Aiello ,INCOR
Ebstein’s Anomaly: Bidirectional Glenn
• reduces venous return to enlarged,
dysfunctional RV by ~ 35-45%
• optimizes preload to LV
MPAP < 18-20 mmHg
Postoperative Echocardiogram
BV 5 YOG, three years after the cone operation
Images courtesy of Lilian Lopes, MD
Ebstein’s Anomaly: Variations in Tricuspid Valve Opening Plan
Image courtesy of J Dearani TV opens toward the RVOT
The echocardiographer attempted to predict the likelihood of successful surgical valve repair in 284 cases.
Tricuspid Repair for Ebstein’s: DanielsonPredictive Value of Preoperative Echocardiography
Sensitivity was 59%, Specificity was 92%,Positive predictive value was 65%, Negative predictive value was 90%.
“Favorable echocardiographic criteria for TV repair include both the valveleaflet location and morphology and the papillary muscle location andattachments. Valves that have severe leaflet displacement into the RV apex
or those anteriorly rotated into the RVOT are generally notsuitable for the traditional monocusp repair.”
Brown ML et alJ Thorac Cardiovasc Surg 2008;135:1120-36
Carpentier´s type D anatomy
EBSTEIN’S ANOMALY – THE CONE REPAIR
Type D: complete atrialization of RV
Procedure Number of patients Hospital mortality
Cone Repair 174 5 (2.9%)*
Neonate Cone repair 8 2 (25%)
TV Replacement 1 0
Total 184 6 (3.8%)
EBSTEIN’S ANOMALY: BIVENTRICULAR SURGICAL TREATMENT184 patients – 1993- November 2015
*The later 60 consecutive patients without hospital mortality.
EARLY AND LONG TERM RESULTS - 174 pts (November 1993 – November 2015)
EVENTS NUMBER CAUSES
Hospital deaths 5 (2.9%) Low cardiac output 4 Acute dehiscence of TV 1
Late deaths 5 (2.9%) Endocardites 1Heart failure +arrhythmia 2 Sudden death (arrhythmia?) 1Swimming Pool accident 1
TV re-repair 5 (2.9%) Increased regurgitation
A-V block 2 (1.1%) β blocker and amiodarone use, one year after operation
EBSTEIN’S ANOMALY – THE CONE REPAIR
EA: Preoperative X Postoperative TV function
Tricuspid regurgitationMean ± Confidence Interval of 95%
Da Silva et al, Arq Bras Cardiol 2011;97:199-208
Cardiothoracic Ratio Index
Comparison between preop and long term follow-up values. Mean ± Confidence Interval of 95%
MICHIGAN UNIVERSITY Shinkawa T,…Bove EL, Ohye,R; J Thorac Cardiovasc Surg 2010
Neonatal Ebstein’s Anomaly
CONE REPAIR FOR EBSTEIN’S ANOMALY
After previous operation done elsewhere
Previous procedure
Danielson’s repair
Carpentier’s repair
TV replacement
(n= 4) 1 2 1
Hospital Mortality
0 0 0
CONE REPAIR FOR EBSTEIN’S ANOMALY
After previous TV replacement
FA, 17yom - Preoperative MRI FA, 17yom – Postoperative echo
Procedure Patients (N) Age Median (range)
Hospital Death
TV repair 89 19 years (19 d – 68y) 1 (1.1%)
TV replacementAt same hospitalization
6 (6.7%) -
Strategies for Tricuspid Re-Repair in Ebstein MalformationUsing the Cone Technique
Dearani J et alAnn. TS 96; 202-210; 2013
Strategies for Tricuspid Re-Repair in Ebstein MalformationUsing the Cone Technique (N=25)
Dearani J et alThe Annals of Thoracic Surgery 2013; 96:202-210
EBSTEIN’S ANOMALY – THE CONE REPAIRResults: Children X older than 12 years patients
Kaplan-Meier survival comparing patients under 12 years of age with patients older than 12 years. CI=95%.
Actuarial suvival curvesGehan-Breslow-Wilcoxon Test
Pe
rce
nt
su
rviv
al
0 5 10 15 20 250
50
100 >12 anos
<=12 anos
Patients at risk
Baseline 5 years 10 years 15 years 20years
>12 yo 89 47 24 6 1
= 12yo 81 41 18 6 4
p = 0,0454
Indications for operation have included symptoms, deterioratingexercise capacity, New York Heart Association functionalclass III, IV heart failure, cyanosis (oxygen saturation90%), paradoxical embolism, progressive cardiomegaly onchest X-ray (computed tomographic ratio0.6), progressiveright ventricular enlargement on echocardiography, and onsetor progression of atrial of ventricular arrhythmias. Observationhas been recommended for asymptomatic patientswith low normal exercise tolerance, no right-to-left shunting,and only mild cardiomegaly. With the introduction of thecone repair and its excellent early to mid-term results, consideration to earlier operative intervention may be given because this procedure can be can be performed with low riskand provides a near anatomic repair.” Joseph Dearani, MD, Mayo Clinic .
Dearani J, Bacha E and da Silva J.Operative Techniques in Thoracic and Cardiovascular Surgery 2008; 13:109-125
Changes in Surgical Indications in Ebstein’s anomaly After the Cone Procedure
Ebstein’s Anomaly: Currently Surgical indications
1. Symptoms or reduction in exercise tolerance2. Cyanosis if ASD or FOP are present3. Arrhythmias: New onset or worsening.4. Progressive RV dilation or dysfunction5. Surgical repair be performed between 2 and 5
years of age when the TV anatomical changes are typical of Ebstein’s anomaly
The cone procedure: Summary 1
Ebstein’s anomaly is a complex congenital heart defect involving the tricuspid valve (TV) and the right ventricle (RV).
The vast variety of anatomical and pathophysiological presentations of Ebstein’s anomaly has made it difficult to achieve uniform results with surgical repair.
Numerous repair techniques have been described, but they either fail to effectively treat tricuspid insufficiency in subsets of anatomic variations or have substantial recurrence of TV regurgitation and thus valve replacement has been necessary in many cases.
The cone procedure: Summary 2
The cone procedure for TV reconstruction in Ebstein’s malformation results in a central flow through the tricuspid orifice and full coaptation of the leaflets. It can be performed in nearly all anatomical variations of Ebstein’s anomaly, with low mortality and morbidity.
The tricuspid regurgitation repair achieved using the cone technique is effective and durable for the majority of patients, resulting in clinical improvement and good long-term clinical outcome with rare need for valve replacement.
The procedure restores RV functional volume and may lead to reverse remodeling of the heart in most patients.
Our data suggest that the cone procedure may result in better clinical outcome when performed at a young age.
1. Danielson: transverse plication of RV, repairs the tricuspid valve at the level it exists in the RV.
2. Carpentier: vertical plication of RV, bring the TV to the true tricuspid annulus.
MAIN OPERATIONS AVAILABLE IN 1989
1- Mayo Clin Proc 1979;54:185-192
2- J T C S 1988; 96:92-101
The anterior and posterior TV leaflets are mobilized from their anomalous attachment in the RV.
EBSTEIN’S ANOMALY – THE CONE PROCEDURE
Da Silva JP et al.Journal Thorac Cardiovasc Surg, 2007;133:215-23
The free edge of this complex is rotated clockwise to be sutured to the septal border of the anterior leaflet, creating a cone.
Plication of the tricuspid annulus at the AV junction level and suture the base of the cone.
Valved closure of the atrial septal defect
EBSTEIN’S ANOMALY – THE CONE PROCEDURE
Da Silva JP et al.Journal Thorac Cardiovasc Surg, 2007;133:215-23
1953- Systemic-pulmonary anastomosis
(Blalock-Taussig and Potts-Smith shunts) and
ASD closure
1960- Weinberg et al. reported the first
successful Glenn operation for Ebstein’s
anomaly
The Initial Surgical Palliations for Ebstein’s Anomaly
Goodwin JF et al
Am Heart J.1953; 45(1):144-58.
Weinberg M Jr et alJTCS.1960;40:310-20
The Evolution of Biventricular Ebstein’s Anomaly Correction
• 1956- Hunter and Lillehei designed a repair technique based in the ARV exclusion by transverse plicature.
• 1962- Barnard and Schrire (13) reported valve replacement in a patient with Ebstein’s anomaly who was the first survivor of biventricular EA correction.
• 1964 – Hardy et al reported the first successful tricuspid valve repair with transverse plicature of the RV atrialized portion. They used the same technique described by Hunter and Lillehei, which was reportedin 1958.
Hunter SW. Dis Chest.1958;33:297-304
Barnard CN, Schrire V. Surgery. 1963;54:302-8.
Hardy KL. J Thorac Cardiovasc Surg. 1964;48:927-49
CASO CLÍNICO: Implante de ECMO
Em 18/09/2014:
Devido ao quadro persistente de baixo débito cardíaco, com falência do VD, optou-se pela realização da derivação cavo-pulmonar parcial. Como persistiu o baixo débito, realizou-se o implante de ECMO.
Ebstein’s Anomaly: Variations in Tricuspid Valve Opening Plan
Image courtesy of J Dearani TV opens toward the RVOT