ebstein's anomali

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  • EBSTEINS ANOMALIDr.Tanvir RahmanMS(CTS) final part studentNHFH & RI

  • Ebsteins 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 RVRedundency fenestration and tethering of anterior leafletDilatation of true tricuspid annulusVariable ventricular myocardial dysfunction

  • History and backgroundWilhelm 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 1958First successful replacement in 1963 by Barnard and Schrire

  • epidemiologyThe 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 Ebsteins anomaly is heart failure due tomalformed 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 paradoxically

    Leads 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

  • presentationPatients can have a variety of symptoms related to the anatomical abnormalities of Ebsteins anomaly and their hemodynamic effects or associated structural and conduction system disease.

    SOB on exertion Occasional palpitation fatigue Features of heart failure

  • Physical examination findingsCyanosisJVP- normal or large V waveLiver palpable but not pulsatileAscitis & Peripheral oedema at advanced stageApex beat shifted to leftLeft parasternal heaveWide splitting of both 1st and 2nd heart soundSoft Systolic murmur over left parasternal area due to TR

  • investigations

  • ECGECG-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 investigationsCT angiogramMRI-quantitive measurement of LV and RV sizeIntra operative TEEInvasive ECG

  • classificationBased on morphology of RV and TV (Carpentiers classification-1988)

  • Risk Assessment Great Ormond Street Echo ScoreArea of (RA + aRV) Area of (RV + LV + LA) 11.5100%GOSERatioMortalityScore

  • Treatment optionsMostly 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 managementSevere symptomaticNYHA Class III/IVSevere cyanoticParadoxical embolismCardiomegalySystolic dysfunction

  • Relative contra indication of surgeryRelative Contra indications:Older age(>50 years)Moderate pulmonary hypertensionLVEF
  • Surgical ProceduresDanielson (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 procedureBichell procedureTVR (without plication)Starnes procedure (single ventricle palliation strategy)

  • Cone procedure of TV repair -the latest optionSurgical 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!!

  • AnnularreductionAnnular 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 transplantationIndications:Severe biventricular dysfunction(RV dilatation and dysfunction with severe LV dysfunction LVEF

  • 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