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Ebstein’s Anomaly
Steven H. Todman, M.D.
Assistant Professor
Pediatric Cardiology
LSUHSC-Shreveport
Goals and objectives
• The learner will
understand the
anatomy, pathology,
genetic factors,
associated cardiac
defects,
presentation, and
evaluation and
management in
children with
Ebstein’s Anomaly
Objectives
• Embryology
▫ Know the embryologic basis for Ebstein anomaly of
the tricuspid valve
• Anatomy
▫ Recognize pathologic features of Ebstein anomaly of
the tricuspid valve
▫ Recognize lesions commonly associated with Ebstein
anomaly of the tricuspid valve
• Physiology
▫ Know the spectrum of abnormalities in circulatory
physiology and oxygen delivery in Ebstein anomaly of
the tricuspid valve
Objectives
• Clinical findings
▫ Recognize Ebstein anomaly of the tricuspid valve
based on clinical findings
• Laboratory findings
▫ Recognize the typical radiologic features of Ebstein
anomaly of the tricuspid valve
▫ Diagnose Ebstein anomaly of the tricuspid valve by
echocardiography, and recognize important anatomic
features
▫ Recognize the typical ECG findings for Ebstein
anomaly of the tricuspid valve
Objectives
• Management, including complications
• Plan medical management of a neonate with Ebstein’s
anomaly of the tricuspid valve and severe hypoxemia
• Plan appropriate surgical and transcatheter therapy in a
patient with Ebstein’s anomaly of the tricuspid valve
• Understand ventilatory and metabolic consequences in a
severely hypoxemic patient with Ebstein’s anomaly of
the tricuspid valve
• Manage the surgical complications of Ebstein’s anomaly
of the tricuspid valve
Which of the following is false?
• (A) The tricuspid valve has three leaflets: anterior,
inferior (posterior), and septal.
• (B) The leaflets develop from the endocardial
cushions exclusively
• (C) Ebstein’s anomaly is characterized by adherence
of the septal and inferior leaflets to the underlying
myocardium.
• (D) There is redundancy, fenestrations, and
tethering of the anterior leaflet of the tricuspid
valve.
• (E) There is dilation of the right AV junction (true
tricuspid annulus)
Which of the following is false?
• (A) The tricuspid valve has three leaflets: anterior,
inferior (posterior), and septal.
• (B) The leaflets develop from the endocardial
cushions exclusively
• (C) Ebstein’s anomaly is characterized by adherence
of the septal and inferior leaflets to the underlying
myocardium.
• (D) There is redundancy, fenestrations, and
tethering of the anterior leaflet of the tricuspid
valve.
• (E) There is dilation of the right AV junction (true
tricuspid annulus)
Answer
• (B) is false. The leaflets of the tricuspid valve
develop equally from the endocardial cushion
tissues and the myocardium. Downward
dysplacement of the tricuspid valve is due to
failure of delamination of valve leaflets from
underlying myocardium.
Which of the following are false?
• (A) The anterior leaflet is usually small, and
attached to the tricuspid annulus
• (B) The anterior leaflet is generally redundant
and may have fenestrations.
• (C) Chordae tendineae are generally short and
poorly formed.
• (D) The anterior leaflet may form a sail-like
intracavitary curtain.
Which of the following are false?
• (A) The anterior leaflet is usually small,
and attached to the tricuspid annulus
• (B) The anterior leaflet is generally redundant
and may have fenestrations.
• (C) Chordae tendineae are generally short and
poorly formed.
• (D) The anterior leaflet may form a sail-like
intracavitary curtain.
Answer
• (A) is false. The anterior leaflet is usually the
largest leaflet, and it is attached to the tricuspid
valve annulus.
Which of the following are false?
• (A) In normal hearts, the downward displacement of
the septal and posterior leaflets in relation to the
anterior mitral valve leaflet is <8mm/m2 body
surface area.
• (B) There is usually marked dilation of the true TV
annulus which is not displaced.
• (C) The left coronary artery demarcates the level of
the true tricuspid valve annulus.
• (D) The right coronary artery is vulnerable to
kinking or distortion during RV plication,
annuloplasty procedure, or tricuspid valve
replacement.
Which of the following are false?
• (A) In normal hearts, the downward displacement of
the septal and posterior leaflets in relation to the
anterior mitral valve leaflet is <8mm/m2 body
surface area.
• (B) There is usually marked dilation of the true TV
annulus which is not displaced.
• (C) The left coronary artery demarcates the
level of the true tricuspid valve annulus.
• (D) The right coronary artery is vulnerable to
kinking or distortion during RV plication,
annuloplasty procedure, or tricuspid valve
replacement.
Answer
• (C) is false. The right coronary artery is
vulnerable to kinking or distortion during RV
plication, annuloplasty procedures, or tricuspid
valve replacement due to the thin nature of the
atrial and ventricular tissue at the level of the AV
groove.
Which of the following is false?
• (A) The dilation in Ebstein’s anomaly usually
involves the atrialized inlet portion of the RV
and the right ventricular apex and outflow tract.
• (B) The anomaly is more common in twins, in
those with a family history of congenital heart
defects, and those with a maternal exposure to
benzodiazepines.
• (C) Maternal lithium exposure is a frequent
association with Ebstein’s anomaly.
Which of the following is false?
• (A) The dilation in Ebstein’s anomaly usually
involves the atrialized inlet portion of the RV
and the right ventricular apex and outflow tract.
• (B) The anomaly is more common in twins, in
those with a family history of congenital heart
defects, and those with a maternal exposure to
benzodiazepines.
• (C) Maternal lithium exposure is a
frequent association with Ebstein’s
anomaly.
Answer
• (C) Is false. Maternal lithium exposure is not a
frequent association with Ebstein’s anomaly.
Which of the following are false?
• (A) A PFO or ASD is present in 80-94% of patient’s
with Ebstein’s Anomaly.
• (B) A VSD is commonly present with or without
pulmonary atresia
• (C) RVOT obstruction and PDA are commonly seen.
• (D) Left sided heart lesions and coarctation of the
aorta are often seen.
• (E) Accessory conduction pathways are present in
15-20% of patient’s, predisposing them to
arrhytmias.
• (F) RV noncompaction is frequently seen.
Which of the following are false?
• (A) A PFO or ASD is present in 80-94% of patient’s
with Ebstein’s Anomaly.
• (B) A VSD is commonly present with or without
pulmonary atresia
• (C) RVOT obstruction and PDA are commonly seen.
• (D) Left sided heart lesions and coarctation of the
aorta are often seen.
• (E) Accessory conduction pathways are present in
15-20% of patient’s, predisposing them to
arrhytmias.
• (F) RV noncompaction is frequently seen.
Answer
• (F) RV noncompaction is not frequently seen.
39% of 18% of patients had left ventricular
dysplasia resembling noncompaction.
Which of the following is false?
• (A) The functional impairment of the RV and TV
regurgitation retards forward flow of blood through
the right side of the heart.
• (B) Ebstein’s anomaly can be a ductal dependent
lesion
• (C) Patients with severe disease will have elevated
RA pressure, and significant right to left interatrial
shunting, with arterial desaturation.
• (D) Patients may present with cyanosis that may
worsen as pulmonary vascular resistance decreases.
• (E) Patients are often at risk for paradoxical
embolization, brain abscesses, and sudden death.
Which of the following is false?
• (A) The functional impairment of the RV and TV
regurgitation retards forward flow of blood through the
right side of the heart.
• (B) Ebstein’s anomaly can be a ductal dependent lesion
• (C) Patients with severe disease will have elevated RA
pressure, and significant right to left interatrial shunting,
with arterial desaturation.
• (D) Patients may present with cyanosis that may
worsen as pulmonary vascular resistance
decreases.
• (E) Patients are often at risk for paradoxical
embolization, brain abscesses, and sudden death.
Answer
• Patients may present with cyanosis that may
improve as pulmonary vascular resistance
decreases.
Which of the following are not frequently seen as
part of the physical exam of Ebstein’s Anomaly?
• (A) Murmur and click
• (B) Cyanosis
• (C) Prominent “v” wave in the distended jugular
veins
• (D) Hepatomegaly
• (E) Widely split first and second heart sounds
• (F) A prominent S3 and/or loud S4
• A systolic murmur at the left lower sternal
border that increases with inspiration.
• (G) A mid-diastolic murmur
Which of the following are not frequently seen as
part of the physical exam of Ebstein’s Anomaly?
• (A) Murmur and click
• (B) Cyanosis
• (C) Prominent “v” wave in the distended
jugular veins
• (D) Hepatomegaly
• (E) Widely split first and second heart sounds
• (F) A prominent S3 and/or loud S4
• A systolic murmur at the left lower sternal
border that increases with inspiration.
• (G) A mid-diastolic murmur
Answer
• Prominent “v” wave in the distended jugular
veins are usually not present, despite severe
regurgitation of the tricuspid valve because the
large RA engulfs the increased volume.
• Prominent “a” waves, however are seen in the
distended jugular veins.
Which of the following are false?
• (A) First degree AV block rarely occurs.
• (B) Chest radiography shows can show an enlarged
globe-shaped heart with a narrow waist, similar to
that seen with a pericardial effusion.
• (C) Outcome is worse when the cardiothoracic ratio
is >0.65 on chest x-ray.
• (D) Intra-atrial conduction disturbance including
PR interval prolongation and tall P waves can be
seen.
• (E) A right bundle branch block can be seen.
Which of the following are false?
• (A) First degree AV block rarely occurs.
• (B) Chest radiography shows can show an enlarged
globe-shaped heart with a narrow waist, similar to
that seen with a pericardial effusion.
• (C) Outcome is worse when the cardiothoracic ratio
is >0.65 on chest x-ray.
• (D) Intra-atrial conduction disturbance including
PR interval prolongation and tall P waves can be
seen.
• (E) A right bundle branch block can be seen.
Answer
• First degree AV block frequently occurs.
Which of the following is false?
• (A) Apical displacement of the septal leaflet by at
least 8 mm/m2 BSA is considered a diagnostic
feature of Ebstein’s anomaly.
• (B) Important features that can be determined
echocardiographically and that can predict
outcome in neonates include patency of the
RVOT.
• (C) Predictors of cardiac-related death include
NYHA class III or IV, cyanosis, severe TR and
younger age at diagnosis.
Which of the following is false?
• (A) Apical displacement of the septal leaflet by at
least 8 mm/m2 BSA is considered a diagnostic
feature of Ebstein’s anomaly.
• (B) Important features that can be determined
echocardiographically and that can predict
outcome in neonates include patency of the
RVOT.
• (C) Predictors of cardiac-related death include
NYHA class III or IV, cyanosis, severe TR and
younger age at diagnosis.
Answer
• All are true.
Which of the following is false?
• (A) Biventricular repair (Knott-Craig Approach)
involves a repair of the tricuspid valve, and partial
closure of the atrial septum.
• (B) Right ventricular exclusion (Starnes Approach)
involves fenestrated patch closure of the tricuspid
orifice, enlargement of the interatrial
communication, right atrial reduction, and
placement of a systemic to pulmonary artery shunt.
• (C) The RV exclusion procedure is useful for
patients with anatomic RVOT obstruction.
• (D) Cardiac transplantation is most often utilized
when there is significant LV dysfunction.
Which of the following is false?
• (A) Biventricular repair (Knott-Craig Approach)
involves a repair of the tricuspid valve, and partial
closure of the atrial septum.
• (B) Right ventricular exclusion (Starnes Approach)
involves fenestrated patch closure of the tricuspid
orifice, enlargement of the interatrial
communication, right atrial reduction, and
placement of a systemic to pulmonary artery shunt.
• (C) The RV exclusion procedure is useful for
patients with anatomic RVOT obstruction.
• (D) Cardiac transplantation is most often utilized
when there is significant LV dysfunction.
Answer
• All are true.
Which of the following are false?
• (A) In mild Ebstein’s anomaly, with nearly
normal heart size, and absence of arrhythmias,
athletes can participate in all sports.
• (B) ACE inhibitors have unproven efficacy in
right-sided failure, but they are used frequently
as part of a heart failure regimen.
• (C) Patients with tachyarrhythmias should
undergo EP evaluation and ablation.
• (D) Success rate of catheter ablation is equal to
those with structurally normal hearts.
Which of the following are false?
• (A) In mild Ebstein’s anomaly, with nearly normal
heart size, and absence of arrhythmias, athletes can
participate in all sports.
• (B) ACE inhibitors have unproven efficacy in rightsided failure, but they are used frequently as part of
a heart failure regimen.
• (C) Patients with tachyarrhythmias should undergo
EP evaluation and ablation.
• (D) Success rate of catheter ablation is equal
to those with structurally normal hearts.
Answer
• Success rate of catheter ablation is lower than
those with structurally normal hearts.
Which of the following is false?
• (A) Indications for surgery includes the presence of
symptoms, cyanosis, and paradoxical embolization.
• (B) In the presence of class III or IV NYHA or
significant symptoms, medical treatment has little to
offer and the surgery will be the best chance for
improvement.
• (C) If TV repair is not feasable, porcine bioprosthetic
valve replacement is a good alternative over
mechanical valves due to the lack of anticoagulation.
• (D) The most common atrial tachyarrhytmias in
Ebstein’s anomaly are atrial fibrillation and flutter.
Which of the following is false?
• (A) Indications for surgery includes the presence of
symptoms, cyanosis, and paradoxical embolization.
• (B) In the presence of class III or IV NYHA or
significant symptoms, medical treatment has little to
offer and the surgery will be the best chance for
improvement.
• (C) If TV repair is not feasable, porcine bioprosthetic
valve replacement is a good alternative over
mechanical valves due to the lack of anticoagulation,
despite increased risk of thrombosis.
• (D) The most common atrial tachyarrhytmias in
Ebstein’s anomaly are atrial fibrillation and flutter.
Answer
• All are true
Which of the following are false?
• (A) Optimal timing for surgical repair is before the
onset of RV dysfunction even in asymptomatic
patients.
• (B) When a mechanical valve is used, the target INR
is 1.5 to 2.5, in addition to aspirin 81 mg daily.
• (C) Uhl’s anomaly is an absence of the myocardial
layer of the RV, and generally results in CHF,
peripheral edema, and pleural effusion.
• (D) Arrhythmias are not common in Uhl’s anomaly.
• (E) Uhl’s anomaly is associated with PA/IVS.
Which of the following are false?
• (A) Optimal timing for surgical repair is before the
onset of RV dysfunction even in asymptomatic
patients.
• (B) When a mechanical valve is used, the
target INR is 1.5 to 2.5, in addition to aspirin
81 mg daily.
• (C) Uhl’s anomaly is an absence of the myocardial
layer of the RV, and generally results in CHF,
peripheral edema, and pleural effusion.
• (D) Arrhythmias are not common in Uhl’s anomaly.
• (E) Uhl’s anomaly is associated with PA/IVS.
Answer
• When a mechanical valve is used, the target INR
is 3 to 3.5, in addition to aspirin 81 mg daily.