Ebstein’s anomaly

Department of cardiovascular and thoracic surgery

Sarit Levinsky
Group M1656
 • It is a rare congenital heart defect
Definition associated with malformed tricuspid
valve leaflets (septal and posterior
ones) that are displaced into the
right ventricle with subsequent
tricuspid valve regurgitation and
right heart enlargement.
• <1% of all CHD cases.
• Classified as critical congenital
heart defect.
• Dr Wilhelm Ebstein first described this
anomaly in 1866
Normal
anatomy of the
tricuspid valve
 Etiology
• Congenital diseases are often of uncertain cause
• Multifactorial (most cases are sporadic)
• Environmental factors: maternal ingestion of
lithium, maternal BZD use, maternal history of
previous fetal loss
• Isolated genetic defects (such as in MYH7,
which encodes beta myosin heavy chain)
Hey there… just a quick explanation:
• What does atrialization mean?
It occurs when the abnormal position of the tricuspid valve causes part of the right
ventricle to become functionally part of the right atrium. It occurs in ebstein anomaly.
• So, in conclusion, what does ebstein’s anomaly mean?
In Ebstein's Anomaly of the Tricuspid Valve, the valve forms abnormally and is lower
than usual in the heart, which may then involve this valve’s insufficiency and right
atrium enlargement
Okay now, are you ready to pass on to the pathophysiology?
Pathophysiology
Ebstein’s anomaly is a malformation of the tricuspid valve and
right ventricle characterized by:
(1) adherence of the septal and posterior leaflets to the
underlying myocardium (failure of delamination, namely
splitting of the tissue by detachment of the inner layer
during embryologic development)
(2) downward (apical) displacement of the functional annulus
(septal>posterior>anterior)
(3) dilation of the “atrialized” portion of the right ventricle,
with various degrees of hypertrophy and thinning of the
wall
(4) redundancy, fenestrations, and tethering of the anterior
leaflet
(5) and dilation of the right atrioventricular junction (true
tricuspid annulus)
One last
picture, to
make sure
everything is
clear…
Normal
VS
Anomaly
Classification
GOSE
score with
mortality
rates
Carpentier
Classification:
How it looks
like
 • The cardinal symptoms in ebstein’s
anomaly are cyanosis, right sided heart
failure, arrythmias and sudden cardiac
death.
• Fetuses may present abnormal routine

Clinical prenatal scan (86%)

• Neonates: cyanosis (76%)
• Infants: heart failure (43%)
features • Children: incident murmur (63%)
• Children >10 years old and adults:
arrhythmias (42%), decreased exercise
tolerance, fatigue or right sided heart
failure.
 • Exertional dyspnea

Other • Palpitations
• Severe cardiomegaly
Features • Respiratory distress
• Wolff – Parkinson white syndrome
Include… • Paradoxical embolism
 Atrial septal defect
Patent foramen ovale
bicuspid or atretic aortic valves
Associated pulmonary atresia or hypoplastic pulmonary artery
Cardiac subaortic stenosis or coarctation

Malformations mitral valve prolapse, accessory mitral valve tissue

pulmonary stenosis
Left heart abnormalities such as left ventricular dysplasia
 Rem
Diagnosis e
Oxyg mber!
satur en
a
will tion rate
be lo
w
:Physical examination
Skin and nails inspection
Cyanosis •
Distention of jugular veins •
Digital clubbing depends on the degree of severity •
Heart and vessels auscultation
widely split S1, S2 •
Prominent S3, S4 •
Systolic murmur •
Prominent “a” wave in distended jugular veins •
Diagnosis –
echocardiography is
the test of choice!

• Example of an
echocardiogram of a patient
with severe Ebstein’s
anomaly showing a grossly
displaced septal leaflet
(arrow). The anterior leaflet is
severely tethered and nearly
immobile. The functional
right ventricle (RV) is small.
Diagnosis – Imaging
• Chest X ray: Ebstein’s anomaly configuration consisting of a globe-
shaped heart with a narrow waist like that seen with pericardial
effusion. Vascularity of the pulmonary fields is either normal or
decreased, pay attention to the cardio thoracic ratio! (>0.65 may indicate
a poor prognosis).
Are you curious about how ebstein’s anomaly
looks on ECG?
• Right bundle branch block
• Supraventricular tachycardia
• WPW
• Atrial flutter or fibrillation
• First degree heart block
• Deep Q waves in leads V1-4 and the inferior leads
 • ECG of a patient with severe Ebstein’s anomaly showing the
typical changes, with prolongation of the PR interval, right
ECG bundle-branch block, and a probable bizarre configuration of the
QRS complex.
Diagnosis -
Imaging
• Cardiac catheterization
is rarely necessary but
can be done before
coronary angiography.
• MRI
 • First, let’s think about the patient – the grade of severity
Before of his anomaly, does he suffer from heart failure?
If he does – we can administer diuretics, ACE inhibitors,
proceeding to digoxin (but make sure the patient doesn’t have heart
block).
treatment, let’s • Next – do you consider that the patient must undergo

ask ourselves surgery?

If yes, let’s think (before naming the procedures), what
how we can surgery can we do for the patient. So, if the problem is in
the tricuspid valve – of course we would like to repair the
help this tricuspid, right? Maybe we can even replace the tricuspid
(but let’s start first with repairing, it’s preferable). Oh, and
patient… by the way, does the patient have ASD? Yes? Okay then,
let’s close this defect in surgery.
 Prostaglandin infusion

Placement of umbilical catheters

Initiation of mechanical ventilation

Treatment –
Management of pulmonary hypertension with nitric oxide,
Neonates sildenafil

Diuretics

Indications Heart failure

for
:surgery Profound cyanosis
Surgical • Biventricular repair (knott – craig
Options approach)
• Single ventricle pathway with
Include… right ventricular exclusion
(stranes’ approach)
• Modified starnes’ approach with
total ventricular exclusion
• Cardiac transplantation
Treatment – children and adults

PROCAINAMIDE FOR DIURETICS, ACE RADIOFREQUENCY

ARRHYTHMIAS INHIBITORS, DIGOXIN ABLATION
FOR HEART FAILURE
Indications for surgery
• Severe tricuspid insufficiency with aggravation of symptoms
• Cyanosis (resting oxygen saturation rate <90%)
• Paradoxical embolism
• Limited exercise capacity (NYHA 3-4)
• Increased heart size (cardiothoracic ratio >65%)
• Transient ischemic attack or stroke
 • Da silva’s Cone repair – (newer
Surgical method)
• Tricuspid valve repair (Danielson
Options repair or modified Danielson repair)
• Tricuspid valve replacement
• Ventriculization procedure (reintegration of
the atrialized chamber into the right ventricular cavity)
Surgical procedures for arrhythmias

Ablation of the accessory ways

Maze procedure
Short explanation about
the Maze procedure…
• In this procedure, the surgeon makes small
incisions in the upper chambers of the patient’s
heart to create a pattern or maze of scar tissue.
Because scar tissue doesn't conduct electricity,
it interferes with stray electrical impulses that
cause some types of fast heart rhythms. Extreme
cold (cryotherapy) or radiofrequency energy
also can be used to create the scars.
 Last resort? Or the
beginning of a new life?
Cardiac transplantation
Thank you!
Bibliography
• https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.106.619338
• https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/diagnosis-treatment/d
rc-20352132
• https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-d
efects/ebsteins-anomaly
• https://www.ssmhealth.com/cardinal-glennon/fetal-care-institute/heart/ebsteins-anomaly
• https://emedicine.medscape.com/article/154447-overview
• https://www.amboss.com/us/knowledge/Cyanotic_congenital_heart_defects