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Jose Pedro da Silva, MDHospital Beneficencia Portuguesa of Sao Paulo, Brazil

Ebstein’s anomaly

AATS SPECIAL INVITED LECTURE

Presenter DisclosureJose Pedro da Silva, MD

No Relationships to Disclose

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

Images courtesy ofProf Vera Aiello ,INCOR

EBSTEIN’S ANOMALY – ECHOCARDIOGRAPHIC FEATURES

Drawing courtesy of J Dearani

EBSTEIN´S ANOMALY – ANATOMICAL VARIATIONS

No RV dilation, mild TR, mobile AL Enlarged RV, grade 3 TR, restricted AL

MRI - size & function of RV

RV with good functionRV : thin wall, bad dysfunction

Chest XR in Ebstein’s anomaly

Typical configuration: globular-shaped heart with a narrow waist

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

Tricuspid Repair for Ebstein’s: Danielson

True annulus

AL

Functionalannulus

G. K. Danielson

RV plication part of repair Posterior annuloplasty

Tricuspid Repair for Ebstein’s: Danielson

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

RA, 19 YOF


Ebstein’s Anomaly

Right

Coronary

Artery

Small septal leaflet and linear attachment of the posterior leaflet


Dearani J, Bacha E, Da SilvaJP. Operative Techniques CVS 2008


Small septal leaflet is combined wit the posterior leaflet

Dearani J, Bacha E, Da Silva JP. Operative Techniques CVS 2008


One year postoperative echocardiogram


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

Cone Attachment to the septal annulus of the tricuspid valve

ASD Closure in Ebstein’s anomaly repair

Ebstein’s Anomaly: Bidirectional Glenn

• reduces venous return to enlarged,

dysfunctional RV by ~ 35-45%

• optimizes preload to LV

MPAP < 18-20 mmHg

BV, 2yo girl

EBSTEIN’S ANOMALY – THE CONE PROCEDURE


BV, 2yo girl

Postoperative 3D Echocardiogram

BV 5 YOG, three years after the cone operation

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

17 Y, O, M

Preoperative echocardiogram

The cone repair for Ebstein’s anomaly

17 y o m

EA with TV opening towards the RVOT

Your text here

Postoperative echo, 3 years

Carpentier´s type D anatomy


Type D: complete atrialization of RV

Carpentier´s type D anatomy:

postoperative MRI

The Cone Operation: complete leaflet mobilization

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


EA: Preoperative X Postoperative TV function

Tricuspid regurgitationMean ± Confidence Interval of 95%

Da Silva et al, Arq Bras Cardiol 2011;97:199-208


The first case

TD, 31yof, 19 years after the cone operation

M E, 13yog, 9 years after the cone repair


Eighteen months postoperative echocardiogram


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

Neonatal Ebstein´s Anomaly Cone Procedure (N=8)

Fetal echocardiogram

The Cone Operation in NeonatalEbstein´s Anomaly repair


Postoperative echo at 6 and 36 months

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


After previous TV repair operation

AK, 9yo, girl

AK, 9yo, girl


After previous TV repair operation

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.

THANK YOU

HOSPITAL BENEFICENCIA PORTUGUESA OF SAO PAULOhttp://www.beneficencia.org.br/

THANK YOU

EBSTEIN’S ANOMALY – ECHOCARDIOGRAPHIC FEATURES

17 Y, O, M

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

Tricuspid valve with multiple holes


The anterior and posterior TV leaflets are mobilized from their anomalous attachment in the RV.


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


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

Da Silva et al

JTCVS, 2006

The Cone Reconstruction Of TV in Ebstein’s Anomaly

MIDTERM RESULTS

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

RM PRE-OPERATORIA

VES, F, 13anos

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.

CASO CLÍNICO – RMI 5 MESES PO

VE, Fevereiro 2015

Ebstein’s Anomaly: Variations in Tricuspid Valve Opening Plan

Image courtesy of J Dearani TV opens toward the RVOT

Ebstein’s Anomaly

**AV node

Membranous

septum

Coronary

sinus

The Cone Operation in Neonatal Ebstein´s Anomaly repair


PREOP POSTOP

Radiographic follow-up S.G. 10 year old girl

Preop

CTI = 0,97

10º postop day

CTI =0,72

38º postop month

CTI = 0,57

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