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