TV annulus• The tricuspid valve is the most apically (orcaudally) placed valve with the largest orificeamong the four valves.• The tricuspid annulus is oval-shaped and whendilated becomes more circular.• 20% larger than MV annulus .• Normal TV annulus= 3.0 3.5 cm
Leaflets The tricuspid valve has three distinct leafletsdescribed as septal, anterior, and posterior. The septal and the anterior leaflets are larger. The posterior leaflet is smaller and appears to beof lesser functional significance since it may beimbricated without impairment of valve function.
Leaflets The septal leaflet is in immediate proximity of themembranous ventricular septum, and itsextension provides a basis for spontaneousclosure of the perimembranous ventricular septaldefect. The anterior leaflet is attached to the anterolateralmargin of the annulus and is often voluminousand sail-like in Ebstein’s anomaly.
Papillary Muscles & Chordae There are three sets of small papillary muscles,each set being composed of up to three muscles. The chordae tendinae arising from each set areinserted into two adjacent leaflets. The anterior set chordae insert into half of theseptal and half of the anterior leaflets. The medial and posterior sets are similarly relatedto adjacent valve leaflets.
Embryologic aspect The leaflets and tensile apparatus of the atrioventricular valves seem to be formed by a process ofdelamination of the inner layers of the inlet zone ofthe ventricles. Delamination of the right ventricular freewall, continues to the level of AV junction. In EA, delamination of these leaflets may have failedto occur due to an incompletely understoodmechanism Process of delamination is incomplete and falls shortof reaching the level of the AV junction. The apical portions of the valve tissue fail to resorbcompletely. Distortion and displacement of the tricuspid valveleaflets, and a part of the right ventricle becomes
DEFINITION Congenital defect Origins of septal or posteriorleaflets, or both, are displaceddownward into RV Leaflets are variably deformed Atrialization of right ventricle Anterior leaflet is enlarged and sail-
Pathologic anatomy of TV Origin of TV from AV ring &its chordal attachments aremalpositioned Leaflets aremalformed, dysplastic(thickened &distorted), enlarged orreduced in size Septal leaflet alwaysaffected, posterior leafletnearly always, and anteriorleaflet seldom Septal & posterior leaflets
Top, Normal tricuspid valve with anterior,posterior,and septal leaflets in 1 plane.Middle, Tricuspid valve in right sidedEbstein’s anomaly showing displacementof posterior andseptal leaflets; maximal displacement is atthe crux of the posterior and septal leaflets.Bottom, Tricuspid valve in left-sidedEbstein’s anomaly; the displacement ofleaflets is similar to that in the right-sidedanomaly.From Anderson et al.16 Used withpermission of the Mayo Foundation forMedical Education and Research.
Pathologic anatomy ofRV Proximal Atrialized in one fourth of heartsdilated When thin moves paradoxically during systole Electrical potentials are ventricular, but pressure pulse is atrialcontoured Distal Smaller than normal RV RV dilatation Functional portion is infundibulum, trabeculated apex, portionbeneath anterior cusp Thinner walled with fewer muscles
Severe Ebstein’smalformation of tricuspidvalve (4-chamber view)showing marked downwarddisplacement of shelf-likeposterior leaflet withattachment to underlyingfree wall by numerousmuscular stumps(arrows), markedly dilatedatrialized portion of rightventricle (ARV), smallfunctional portion of rightventricle (RV), leftwardbowing of ventricularseptum, and markeddilatation of right atrium(RA). LA indicates leftatrium; LV, left ventricle
Epidemiology Ebstein’s anomaly occurs in 0.3-0.8% of allcongenital heart diseases 1 in 20,000-50,000 live births Equal male: female occurrence Mortality in children presenting in the neonatalperiod is 30-50% Mortality at all ages is 12.5%Arch Anat Physiol 1866:238–25
Associated defects Commonly associated with: ASD or PFO (90%) VSD, AV canal defect Pulmonary stenosis/atresia (20-25%) Wolff-Parkinson-White Syndromes: Down, Marfan, Noonansumner RG, Jacoby WJ Jr, Tucker DH.Ebstein’s anomaly associated with Car-diomyopathy and Pulmonary Hypertension.Circulation 1964;30:578–587.
Etiology Congenital disease of often uncertaincause. Environmental factors Maternal ingestion of lithium in firsttrimester Maternal benzodiazepine use Maternal exposure to varnishingsubstances Maternal history of previous fetal loss Risk is higher in whites than in otherraces.
Genetic factors Rare cases of cardiac transcription factor NKX2.5mutations, 10p13-p14 deletion, and1p34.3-p36.11 deletion have been described inthe anomaly . Recently, Postma et al. reported the results of amutational analysis in a cohort of 141 unrelatedprobands with Ebstein anomaly. Eight were found to have a mutation in the geneMYH7 and six of the eight patients also had leftventricular noncompaction. This may warrant genetic testing and familyevaluation in this subsetemanuel R, O’Brien K, Ng R. Ebstein’sanomaly: genetic study of 26 fami-lies. BrHeart J 1976;38:5–7.
Origin 1866 A.D. Hypothesis on single autopsy specimen He even did not see the patient, when later wasalive Word used in 1927 A.D.
A pencil sketchof WilhelmEbsteinpublished in theFestschriftcelebratingEbstein’s 70thbirthday.Published bypermission ofthe Mayo ClinicProceedings,where it waspublished byMann RJ, Lie JT.The life storyof WilhelmEbstein (1836–1912) and hisalmostoverlookeddescription of acongenital heart
Presentation at various stages oflife Fetal life: Diagnosed incidentally byechocardiography. Neonatal life and infancy: Cyanosis and/or severeheart failure Improve as pulmonaryvascular resistancedecreases. Adult life: Fatigue, exertionaldyspnea, cyanosis, tricuspid regurgitation and/orright heart failure, and
Neonatal presentation Congestive heart failure Due to TR and RV dysfunction Cyanosis Decreased pulmonary blood flow due to R Lshunt through ASD or PFO Increased pulmonary vascular resistance in theneonatal period compounds this problem Murmur
Later presentation Cyanosis Due to R L shunt at atrial level Fatigue and dyspnea Secondary to RV failure and decreased LV ejectionfraction Palpitations and sudden cardiac death Incidental murmur Paradoxical embolism
Cyanosis Fairly common Right-to-left shunt at the atrial level and/or severe heartfailure Transient in neonatal life with recurrence in adult life May appear for the first time in adult life Transient appearance/worsening of cyanosis in adult lifedue to paroxysmal arrhythmias
Fatigue and dyspneaPoor cardiac output secondary to rightventricular failure and decreased leftventricular ejection fraction. Palpitations and sudden cardiac deathSVT in as many as one third of patients Fatal ventricular arrhythmiasaccessory pathways
Arrhythmias Due to right atrial enlargement and highprevalence of accessory pathways 30-50% have evidence of WPW secondary to theatrialized RV tissue Mapping and ablation are difficult Atrial dilation disrupts anatomic landmarks Accessory pathways are often multiple
Electrophysiologic studies 25-30% have accessory pathways 5-25% have evidence of preexcitation on the surface ECG. Right-sided pathways are more common. Fifty percent of the patients have multiple pathways. Guide ablative therapy.
Pregnancy & EbsteinsAnomaly Pregnancy seems to be well tolerated with adequatesupervision.
Prenatal presentation Difficult to diagnose prenatally Fetal presentation is variable: possiblefeatures include cardiomegaly, RAenlargement, tricuspid regurgitation ordysplasia, arrhythmia, or fetal hydrops Prognosis for the fetus diagnosed in uterowith significant tricuspid valve disease is verypoor (20% survival) Progressive right heart dilatation Cardiac failure Lung hypoplasia Pulmonary stenosis/atresia
Cardiothoracic area ratio at 33 (A) and35 (B) weeks gestational age. Aindicates anterior; P, posterior; R, right;and L, left.Circulation September 23, 2003
Prognosis & management Prenatal prognosis could be significantlyinfluenced by the ability of foramen ovale todecompress the right atrium Arrythmia due to extreme dilatation of the RAmight be a cause of sudden intrauterine death Ebstein’s anomaly without cardiac failure orhydrops: vaginal delivery Cesarean section
Physical Examination Cyanosis and clubbing - Varying degrees ofcyanosis at various times in life and transientworsening with arrhythmias Precordial asymmetry Usually left parasternal prominence andoccasionally right parasternal prominence Absent left parasternal (ie, right ventricular) lift animportant negative sign Jugular venous pulse May be normal Large a and v waves late in the course of the
Physical Examination Arterial pulses Usually normal Diminished volume Heart sounds First heart sound widely split with loud tricuspid component Mitral component may be soft or absent in the presence of prolongedPR interval. Second heart sound usually is normal widely split when the pulmonary component is delayed due to RBBB.
Physical Examination Additional heart sounds and murmurs Third and fourth heart sounds commonly present, even in the absence of congestive heart failure(CHF). Summation of third and fourth heart sounds, especially with prolongedPR interval, can mimic an early diastolic murmur. The holosystolic murmur of tricuspid regurgitation At the lower left parasternal area and sometimes at the apex Murmur intensity and duration increase during inspiration.
12-lead ECG Rhythm Usually normal sinus findings IntermittentlySVT, paroxysmal SVT, atrialflutter, atrialfibrillation, ventriculartachycardia Abnormal P wavesconsistent with right atrialenlargement – “HimalayanP waves”• PR interval– Most commonly prolonged– May be normal or short inpatients with WPW (B)syndrome• QRS complex– RBBB• Low voltage in manypatients
Chest X-Ray Cardiomegaly( Rounded or Box-like contour ) Small aortic rootand mainpulmonary arteryshadow Decreasedpulmonaryvasculature
Echocardiogram Standard for diagnosis. M-mode Paradoxical septal motion Dilated right ventricle Delayed closure of tricuspid valve leaflets morethan 65 milliseconds after mitral valve closureRoberson DA, Silverman NH. Ebstein’sanomaly: echocardiographic and clinicalfeatures in the fetus and neonate. J AmColl Cardiol 1989;14:1300–1307.
Echocardiogram Two-dimensional Apical displacement of the septal leafletof greater than 8 mm/m2 – Abnormalities in morphology and septalattachment of the septal and anteriortricuspid leaflets Eccentric leaflet coaptation Dilated right atrium Dilated right ventricle with decreasedcontractile performance Various left heart structural abnormalities
Echocardiogram Assessment of severity and surgical options Functional right ventricular area less than 35% of total right ventriculararea or an atrialized to functional right ventricular ratio greater than 0.5associated with unfavorable prognosis Functional right ventricular size Degree of septal leaflet displacement Amount of leaflet tethering Magnitude of leaflet deformity and dysplasia Aneurysmal dilatation of right ventricular outflow tract (right ventricularoutflow tract-to-aortic root ratio of >2:1 on parasternal short axis view) Moderate-to-severe tricuspid regurgitation
Cardiac MRI CMR imaging has emerged as another tool forevaluation of Ebstein’s patients It provides quantitative measurement of rightatrial and ventricular size and systolic functioneven in the presence of significant distortion ofright ventricular anatomy.Yalonetsky S, Tobler D, Greutmann M, etal. Cardiac magnetic resonance imagingand the assessment of Ebstein anomaly inadults. Am J Cardiol 2011;107:767–773.
Cardiac Catheterization No longer required to make/confirm the diagnosis The most diagnostic characteristic- Typicalatrial pressure & ventricular intracardiac ECG inthe atrialized portion of the RV Elevated RAP R-L atrial shunting with systemic desaturation Elevated RVEDP
Natural history It is not uncommon for Ebstein’s anomaly to beundiagnosed until adulthood. However, late diagnosis is associated withreduced survival. The mean age of diagnosis in a study of thenatural history of 72 unoperated patients, was23.9 ± 10.4 years. In this group of patients, arrhythmias were themost common clinical presentation (51%) . The estimated cumulative overall survival rateswere 89%, 76%, 53%, and 41% at 1, 10, 15, and20 years of follow-up, respectively.
Natural history Predictors of cardiac-related death on univariateanalysis included: Cardiothoracic ratio of ≥ 0.65 Increasing severity of TV displacement onechocardiography New York Heart Association (NYHA) class III or IV Cyanosis Severe TR Younger age at diagnosis In a multivariate model, younger age atdiagnosis, male sex, cardiothoracic ratio of ≥0.65, and the severity of TV leaflet displacement onechocardiography were predictors of late cardiacmortality.
Management Severity Assessment Guideline/Indications Medical management Surgical management Post operative functional status
Celermajer Index Celermajer et al. described an echocardiographicgrading score for neonates with Ebstein’sanomaly, the Great Ormond Street Echocardiography(GOSE) score, with grades 1 to 4. The ratio of the combined area of the RA andatrialized RV is compared to the functional RV and leftheart. This classification is particularly helpful withneonatal Ebstein’s anomaly. Grade 1: ratio <0.5 Grade 2: ratio of 0.5 to 0.99 Grade 3: ratio of 1.0 to 1.49 Grade 4: ratio ≥ 1.5 Celermajer DS, Bull C, Till JA, et al.Ebstein’s anomaly: presentation andoutcome from fetus to adult J Am Coll
Carpentier’s classification In 1988, Carpentier et al. proposed the followingclassification of Ebstein’s anomaly - Type A: The volume of the true RV is adequate Type B: A large atrialized component of the RVexists, but the anterior leaflet of the TV movesfreely Type C: The anterior leaflet is severely restrictedin its movement and may cause significantobstruction of the RVOT Type D: Almost complete atrialization of theventricle except for a small infundibularcomponent.
EBSTEIN’S ANOMALY(Carpentier’s classification)TYPE A (M+ C+) TYPE B (M+ C -)TYPE С (M- C -) TYPE D (“tricuspid suck”)M – mobility C - contractility
CANADIAN CARDIOVASCULARSOCIETYCANADIAN CARDIOVASCULAR SOCIETY2009 CONSENSUS CONFERENCEUPDATE ON THE GUIDELINES FOR THEMANAGEMENT OF ADULTS WITHCONGENITAL HEART DISEASE
EBSTEIN ANOMALYClass IThe following situations warrant intervention: Limited exercise capacity (New York Heart Association class greaterthan II) (Level of Evidence: B) Increasing heart size (cardiothoracic ratio greater than 65%) (Levelof Evidence: B) Important cyanosis (resting oxygen saturations < 90%) (Level ofEvidence: B) Severe tricuspid regurgitation with symptoms (Level of Evidence: B) Transient ischemic attack or stroke (Level of Evidence: B)Patients who require operation for Ebstein anomaly should beoperated on by congenital heart surgeons who have substantialspecific experience and success with this operation. Every effortshould be made to preserve the native TV. (Level of Evidence: C)Presentation at Annual CCS Meeting in Edmonton 2009
Initial management Prostaglandin infusion Placement of umbilical catheters Initiation of mechanical ventilation Minimum possible mean airway pressure Tidal volumes of 10-15 ml/kg to overcomeatelectasis Management of pulmonary hypertension Diuretics for CHF
Management of pulmonaryhypertension Nitric oxide Reduces after load of right ventricle Sedation Other pulmonary vasodilators
The surgical options include-(a) Biventricular repair (Knott-Craig approach)(b) Single ventricle pathway with right ventricularexclusion (Starnes’ approach)(c) Cardiac transplantation.
Biventricular Repair (Knott-CraigApproach) TV is repaired and the atrial septum is partiallyclosed. This repair is typically a mono cusp type basedon a satisfactory anterior leaflet Routine right atrial reduction is important toreduce the size of the markedly enlarged heart toallow room for the lungs. Although early mortality is high (about 25%), theintermediate outcome appears to be promising. Survival to hospital dismissal was 74% with nolatemortality. Ann Thorac Surg 2002;73:1786–1
Right Ventricular ExclusionStarnes Approach Starnes et al. pioneered the right ventricularexclusion approach, which involves:(a) fenestrated patch closure of the TV orifce,(b) enlarging the interatrial communication,(c) right atrial reduction, and(d) placing a systemic-to-pulmonary artery shunt. Particularly useful when there is anatomic RVOTobstruction. Right ventricular decompression is required as itpassively fills from thebesian venous drainage, this isusually accomplished with a 4- to 5-mm punchfenestration in the TV patchstarnes VA, Pitlick PT, Bernstein D, et al.Ebstein’s anomaly appear-ing in theneonate. A new surgical approach. JThorac Cardiovasc Surg 1991;101:1082–
Modified Starnes Repair (Total VentricularExclusion) Sano et al. modified the Starnes single-ventricleapproach by performing a total right ventricularexclusion in which the free wall of the RV isresected and closed primarily or with a polytetrafluoroethylene patch . This simulates a large right ventricularplication, which may improve the left ventricularfilling and provide adequate decompression to thelungs and LV.
Cardiac Transplantation With the improved results of the biventricular andsingle ventricle approaches, transplantation rarelyis performed in the current era. Cardiac transplantation remains an option in themost severe forms of Ebstein’s anomaly,particularly when there is significant leftventricular dysfunction.
Children and Adults Medical Bacterial endocarditis prophylaxis may berequired in the presence of prosthetic materials orpatches that were used for the repair. In mild Ebstein’s anomaly, with nearly normalheart size, and in absence of arrhythmias,athletes can participate in all sports. However, in severe Ebstein’s anomaly, activity isrestricted unless it has been optimally repairedwith near normal heart size and no arrhythmias.
Surgery INDICATIONS FOR SURGERY Presence of symptoms, Cyanosis, Paradoxical embolization. Patients who have decreased exerciseperformance Progressive increase in cardiothoracic ratio Progressive right ventricular dilatation anddysfunction Onset or progression of arrhythmias Presence of class III or IV NYHA or significantsymptoms
PRINCIPLES OF SURGERY FOREBSTEIN’S ANOMALY The following principles are the goals of surgery:(a) Closure of any intra cardiac communications(b) TV repair or replacement(c) Ablation of arrhythmias(d) Selective plication of the atrialized RV fromapex to base,(e) Reduction right atrioplasty(f) Repair of associated defects (e.g., closureVSD).
Tricuspid Valve RepairThe goal of operation is to obtain a competentTV, preserve right ventricular contractility, and todecrease the risk of late rhythm disturbances.
Danielson Repair This repair technique was reported in 1979 andwas based on the creation of a monocusp valveusing the anterior leaflet. This consisted of - Plication of the free wall of the atrialized RV Posterior tricuspid annuloplasty Right reduction atrioplastyDanielson GK, Maloney JD, Devloo RA.Surgical repair of Ebstein’s anom-aly. MayoClin Proc 1979;54:185–192.
Modified Danielson Repair Modifications involves - Bringing the anterior papillary muscle(s) toward theventricular septum, which facilitates coaptation of theleading edge of the anterior leaflet with the ventricularseptum. Generally, an antero posterior tricuspid purse stringor ringed annuloplasty is used, and atrialized rightventricular plication is performed selectively. Thisresults in a TV repair at the level of the functionalannulus, in contrast to the original repair, whichbrought the hinge point of the functional annulus up tothe true annulus. A more recent modification includes patchaugmentation of the mid-anterior leaflet with surgicaldelamination of attachments to the anterior and/orinferior leafets.Dearani JA, Danielson GK. Tricuspid valverepair for Ebstein’s anomaly. Oper TechThorac Cardiovasc Surg 2003;8:188–192.
THE BRAZIL EXPERIENCE (da SILVA APPROACH) The cone repair described by Dr. da Silva from Brazilwhen the anatomy allows, as this technique is themost anatomic of all the repair techniques described. Specially, some septal leaflet should be present,which facilitates this repair technique. The cone technique represents the most anatomicrepair by completion of the delamination process ofthe TV, providing 360 degrees of leafet tissue aroundthe AV junction with its hinge point at the AV groove(true annulus). Although not initially described with this technique,some do supplement the repair with a flexibleanterior annuloplasty band from anteroseptalcommissure to inferseptal commissure wheneverpossible.
Relative contraindications to thecone reconstruction technique Age >50 years Moderate pulmonary hypertension Significant left ventricular dysfunction: ejectionfraction <30% Complete failure of delamination of the septal andposterior leaflets with poor delamination of theanterior leaflet (<50%) Severe right ventricular enlargement Severe TV annular dilatation
THE VENTRICULIZATIONPROCEDURE Ullmann et al. published their results with theventriculization procedure in 2004. This is characterized by reintegration of theatrialized portion of the RV into the rightventricular cavity (ventricularization). This can be obtained by orthotopic transpositionof the detached septal and posterior leaflets ofthe TV. The reimplanted septal leaflet serves as anopposing structure for coaptation of thereconstructed AV valve.
Tricuspid Valve Replacement Every effort should be made to repair the TVrather than replacing it, but if TV repair is notfeasible, then porcine bioprosthetic valvereplacement remains a good alternative. Most prefer bioprostheses to mechanical valvesdue to the relatively good durability and the lackof need for anticoagulation .Kiziltan HT, Theodoro DA, Warnes CA, etal. Late results of biopros-thetic tricuspidvalve replacement in Ebstein’s anomaly.Ann Thorac Surg 1998;66:1539–1545.
One and Half Ventricle Repair The BDCPA does two important things in thesetting of Ebstein’s anomaly. First, it reduces venous return to the enlarged,dysfunctional RV by approximately one-third Second, it provides sufficient preload to the LV tosustain adequate systemic perfusion when right-sided output is low. Surgens usually prefer it if the left ventricular end-diastolic pressure (LVEDP) is <12 mm Hg, thetranspulmonary gradient <10 mm Hg, and themean pulmonary arterial pressure <16 mm Hg,before considering a BDCPA.Kopf GS, Laks H, Stansel HC, et al. Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J ThoracCardiovasc Surg 1990;117:662–670.
Indications for the BDCPAinclude Severe RV enlargement and/or dysfunction Squashed LV (D-shaped LV) Moderate degree of TV stenosis (mean gradient>6 mm Hg) as a result of reduction in the valveorifice area after repair RA:LA pressure ratio >1.5, which indicates poorRV function. Preoperative cyanosis at rest or with exerciseChauvaud S, Fuzellier JF, Berrebi A et al.Bi-directional cavopulmonary shuntassociated with ventriculo andvalvuloplasty in Ebstein’s anomaly:benefits in high risk patients. Eur J
Heart Transplantation Heart transplantation rarely is necessary forEbstein’s anomaly. Indication for transplantation is usually thepresence of severe biventricular dysfunction (leftventricular ejection fraction <25%).
Arrythmia management The most common atrial tachyarrhythmias inEbstein’s anomaly are atrial fibrillation and flutter. Most surgens used successfully the right-sidedcut-and-sew lesions of Cox-maze III procedure inEbstein’s anomaly. With the availability of newer devices such asradiofrequency or cryoablation, the proceduretime for maze procedure is shortenedsignificantly. A biatrial maze procedure, performed particularlywhen there is chronic atrial fibrillation, left atrialdilation, or concomitant mitral regurgitation.