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EBSTEIN’S ANOMALI
Dr.Tanvir RahmanMS(CTS) final part student
NHFH & RI
Ebstein’s anomaly is a congenital malformation of the heart that is characterized by
• Delamination failure of TV leaflets(adherence of tricuspid leaflet to underlying myocardium)
• Apical displacement of functional annulus(septal>anterior>posterior)
• Atrialization & Dilatation of atrialized portion of RV
• Redundency fenestration and tethering of anterior leaflet
• Dilatation of true tricuspid annulus• Variable ventricular myocardial
dysfunction
History and background
• Wilhelm Ebstein first described a patient with cardiac defects typical of Ebstein anomaly in 1866.
• In 1927, Alfred Arnstein suggested the name Ebstein's anomaly for these defects.
• It presented an ongoing challenge since its initial repair attempts in 1958
• First successful replacement in 1963 by Barnard and Schrire
epidemiology
The natural course of the disease varies according to the severity of tricuspid valve displacement.
Patients presenting in infancy generally have severe disease and unfavorable prognosis.
Mean age of presentation is in the middle teenage years.
approximately 5% of these patients survive beyond age 50 years.
The oldest recorded patient lived to age 85 years.
pathophysiologyThe ultimate hemodynamic consequences of Ebstein’s anomaly is heart failure due to•malformed tricuspid leaflets leading to regurgitation(The severity of regurgitation depends on the extent of leaflet displacement)•The atrialized portion of the right ventricle(although anatomically part of the right atrium) contracts paradoxicallyLeads to stagnation of blood in RA during RV relaxation and causes a backward flow of blood into the right atrium during RV systoleAnd deformed TV leaflet may lead to RVOT obstruction and cyanosis
presentation• Patients can have a variety of symptoms related
to the anatomical abnormalities of Ebstein’s anomaly and their hemodynamic effects or associated structural and conduction system disease.
SOB on exertion Occasional palpitation fatigue Features of heart failure
Physical examination findings• Cyanosis• JVP- normal or large V wave• Liver palpable but not pulsatile• Ascitis & Peripheral oedema at advanced stage• Apex beat shifted to left• Left parasternal heave• Wide splitting of both 1st and 2nd heart sound• Soft Systolic murmur over left parasternal area due to TR
investigations
ECG
ECG-P pulmonale,RBBB,SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, ventricular tachycardia
Chest Xray
• Globular shaped heart (due to RA hypertrophy and outward and upward displacement of RVOT)
• Oligemic lung field
ECHO
Echocardiogram(2D/3D) TV anatomy
(annulus,leaflets,leaflet attachments,coaptation,jet flow)
RVOT PV anatomy ASD/VSD size and flow
direction all chamber anatomy and size
measurement RV and LV function
Cardiac cath (haemodynamic cath)
done in selective cases particularly in LV dysfunction, and when BDCP shunt is planned to see LV and RV pressure
Other investigations
CT angiogramMRI-quantitive
measurement of LV and RV size
Intra operative TEEInvasive ECG
classification
• Based on morphology of RV and TV (Carpentier’s classification-1988)
Risk Assessment Great Ormond Street Echo Score
Area of (RA + aRV) Area of (RV + LV + LA)
1 <0.5 8% 2 0.5-1.0 8% 3 (acyanotic) 1.1-1.4 10% E, 45% L 3 (cyanotic) 1.1-1.4 100% 4 >1.5 100%
GOSE Ratio MortalityScore
Treatment options
• Mostly Surgical management
biventricular repair approach Univentricular /RV exclusion approach Heart lung transplant Medical treatment can be given for symptom
alleviation and control of heart failure
Indication of surgical management
• Severe symptomatic• NYHA Class III/IV• Severe cyanotic• Paradoxical embolism• Cardiomegaly• Systolic dysfunction
Relative contra indication of surgery
• Relative Contra indications:• Older age(>50 years)• Moderate pulmonary hypertension• LVEF <30%• Complete failure /poor delamination of leaflets• Severe RV enlargement• Severe dilatation of true tricuspid annulus
Surgical Procedures• Danielson (valvuloplasty by annuloplasty with/without
annuloplasty ring +horizontal plication of non functional/atrialized portion of RV)
• Modified Danielson( annular remodeling)• Carpentier ( rotation valvuloplasty –annuloplasty)• Cone procedure• Bichell procedure• TVR (without plication)• Starnes procedure (single ventricle palliation strategy)
Cone procedure of TV repair -the latest option
• Surgical Delamination Of Fibrous & Muscular Attachments
• Clockwise rotation of the leaflets and Suturing margin of PL to SL to form a cone
• Vertical Plication of Large Atrialized RV
• Annular Reduction and Re suturing of leaflets
• Complete reconstruction with partial closure of ASD/PFO
Clockwise rotation of the leaflets and Suturing margin of PL to SL to form a cone
Plication of Large Atrialized RV
• Decreases tension at annulus• Beware of coronary arteries!!
Annularreduction
Annular Reduction and Re suturing of leaflets
Advantage of Cone repairLeaflet to leaflet coaptationRe constracted TV reattached to true
annulusHinge part of valve is in normal anatomical
positionPlication of thin transparent atriaalized RV
eliminates chance of dyskinesiaExcision of redundant RAVertical plication allow mentainance of near
normal ventricular anatomy
Heart transplantation
Indications:•Severe biventricular dysfunction(RV dilatation and dysfunction with severe LV dysfunction LVEF <25%)•Left ventricular dilatation and dysfunction with MR
THANK YOU
Univentricular/RV exclusion approach (Starnes and Colleagues)
• Patch closure of TV (4-5 mm fenestrated patch for RV decompression as it progressively fills with thebesian venous return)
• Enlargement of interatrial connection
• Placement of systemic to pulmonary arterial shunt
• RA reduction
• Ligation of MPA (if there is incompetent PV with patent RVOT
Modified Starnes/total RV exclusion(Sano and Associates)
Resection of Free wall of RV followed by
primary closure
PTFE closure