A lecture on the echocardiographic evaluation of hypertrophic cardiomyopathy. Starts with an overview of the topic then a systematic approach to diagnosis and then a differential diagnosis followed by take-home messages and conclusion.
Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease. This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.” The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale. By 1950, only 3 cases of this anomaly had been published.
In cases of right atrial enlargement the duration of the P wave hardly changes, but the P-R interval increases, so that the P--R segment ratio falls below the normal range.Left atrial enlargement, on the other hand,does not affect the P-R interval, but the P wave lengthens at the expense of the P-R segment.The result is a- ratio above P-R segment the normal maximal limit of 1.6.In combined atrial enlargement, both P-R interval and P wave are prolonged. It follows that in such cases the ratio may P-R segment
be normal.
Ventricular septal defects occur either as an isolated defect or as a component of a more complex lesion
It occurs in 50 percent of all children with CHD and in 20 to 30 percent as an isolated lesion
Most common congenital cardiac anomaly in children
Second most common congenital abnormality in adults, second only to bicuspid aortic valves
They are more common in premature infants and those born with low weight
VSDs are slightly more common in females (56%)
A lecture on the echocardiographic evaluation of hypertrophic cardiomyopathy. Starts with an overview of the topic then a systematic approach to diagnosis and then a differential diagnosis followed by take-home messages and conclusion.
Ebstein’s anomaly is a rare congenital heart disorder occurring in ≈1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease. This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.” The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen ovale. By 1950, only 3 cases of this anomaly had been published.
In cases of right atrial enlargement the duration of the P wave hardly changes, but the P-R interval increases, so that the P--R segment ratio falls below the normal range.Left atrial enlargement, on the other hand,does not affect the P-R interval, but the P wave lengthens at the expense of the P-R segment.The result is a- ratio above P-R segment the normal maximal limit of 1.6.In combined atrial enlargement, both P-R interval and P wave are prolonged. It follows that in such cases the ratio may P-R segment
be normal.
Ventricular septal defects occur either as an isolated defect or as a component of a more complex lesion
It occurs in 50 percent of all children with CHD and in 20 to 30 percent as an isolated lesion
Most common congenital cardiac anomaly in children
Second most common congenital abnormality in adults, second only to bicuspid aortic valves
They are more common in premature infants and those born with low weight
VSDs are slightly more common in females (56%)
ebstein anomaly is rare congenital disorder,with variable presentation in neonate to adults,early diagnosis and timely take decision make remarkable difference in patients life.
June 3, 2024 Anti-Semitism Letter Sent to MIT President Kornbluth and MIT Cor...Levi Shapiro
Letter from the Congress of the United States regarding Anti-Semitism sent June 3rd to MIT President Sally Kornbluth, MIT Corp Chair, Mark Gorenberg
Dear Dr. Kornbluth and Mr. Gorenberg,
The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
• The Committee on Oversight and Accountability is investigating the sources of funding and other support flowing to groups espousing pro-Hamas propaganda and engaged in antisemitic harassment and intimidation of students. The Committee on Oversight and Accountability is the principal oversight committee of the US House of Representatives and has broad authority to investigate “any matter” at “any time” under House Rule X.
• The Committee on Ways and Means has been investigating several universities since November 15, 2023, when the Committee held a hearing entitled From Ivory Towers to Dark Corners: Investigating the Nexus Between Antisemitism, Tax-Exempt Universities, and Terror Financing. The Committee followed the hearing with letters to those institutions on January 10, 202
Embracing GenAI - A Strategic ImperativePeter Windle
Artificial Intelligence (AI) technologies such as Generative AI, Image Generators and Large Language Models have had a dramatic impact on teaching, learning and assessment over the past 18 months. The most immediate threat AI posed was to Academic Integrity with Higher Education Institutes (HEIs) focusing their efforts on combating the use of GenAI in assessment. Guidelines were developed for staff and students, policies put in place too. Innovative educators have forged paths in the use of Generative AI for teaching, learning and assessments leading to pockets of transformation springing up across HEIs, often with little or no top-down guidance, support or direction.
This Gasta posits a strategic approach to integrating AI into HEIs to prepare staff, students and the curriculum for an evolving world and workplace. We will highlight the advantages of working with these technologies beyond the realm of teaching, learning and assessment by considering prompt engineering skills, industry impact, curriculum changes, and the need for staff upskilling. In contrast, not engaging strategically with Generative AI poses risks, including falling behind peers, missed opportunities and failing to ensure our graduates remain employable. The rapid evolution of AI technologies necessitates a proactive and strategic approach if we are to remain relevant.
The Roman Empire A Historical Colossus.pdfkaushalkr1407
The Roman Empire, a vast and enduring power, stands as one of history's most remarkable civilizations, leaving an indelible imprint on the world. It emerged from the Roman Republic, transitioning into an imperial powerhouse under the leadership of Augustus Caesar in 27 BCE. This transformation marked the beginning of an era defined by unprecedented territorial expansion, architectural marvels, and profound cultural influence.
The empire's roots lie in the city of Rome, founded, according to legend, by Romulus in 753 BCE. Over centuries, Rome evolved from a small settlement to a formidable republic, characterized by a complex political system with elected officials and checks on power. However, internal strife, class conflicts, and military ambitions paved the way for the end of the Republic. Julius Caesar’s dictatorship and subsequent assassination in 44 BCE created a power vacuum, leading to a civil war. Octavian, later Augustus, emerged victorious, heralding the Roman Empire’s birth.
Under Augustus, the empire experienced the Pax Romana, a 200-year period of relative peace and stability. Augustus reformed the military, established efficient administrative systems, and initiated grand construction projects. The empire's borders expanded, encompassing territories from Britain to Egypt and from Spain to the Euphrates. Roman legions, renowned for their discipline and engineering prowess, secured and maintained these vast territories, building roads, fortifications, and cities that facilitated control and integration.
The Roman Empire’s society was hierarchical, with a rigid class system. At the top were the patricians, wealthy elites who held significant political power. Below them were the plebeians, free citizens with limited political influence, and the vast numbers of slaves who formed the backbone of the economy. The family unit was central, governed by the paterfamilias, the male head who held absolute authority.
Culturally, the Romans were eclectic, absorbing and adapting elements from the civilizations they encountered, particularly the Greeks. Roman art, literature, and philosophy reflected this synthesis, creating a rich cultural tapestry. Latin, the Roman language, became the lingua franca of the Western world, influencing numerous modern languages.
Roman architecture and engineering achievements were monumental. They perfected the arch, vault, and dome, constructing enduring structures like the Colosseum, Pantheon, and aqueducts. These engineering marvels not only showcased Roman ingenuity but also served practical purposes, from public entertainment to water supply.
The French Revolution, which began in 1789, was a period of radical social and political upheaval in France. It marked the decline of absolute monarchies, the rise of secular and democratic republics, and the eventual rise of Napoleon Bonaparte. This revolutionary period is crucial in understanding the transition from feudalism to modernity in Europe.
For more information, visit-www.vavaclasses.com
Model Attribute Check Company Auto PropertyCeline George
In Odoo, the multi-company feature allows you to manage multiple companies within a single Odoo database instance. Each company can have its own configurations while still sharing common resources such as products, customers, and suppliers.
Palestine last event orientationfvgnh .pptxRaedMohamed3
An EFL lesson about the current events in Palestine. It is intended to be for intermediate students who wish to increase their listening skills through a short lesson in power point.
Biological screening of herbal drugs: Introduction and Need for
Phyto-Pharmacological Screening, New Strategies for evaluating
Natural Products, In vitro evaluation techniques for Antioxidants, Antimicrobial and Anticancer drugs. In vivo evaluation techniques
for Anti-inflammatory, Antiulcer, Anticancer, Wound healing, Antidiabetic, Hepatoprotective, Cardio protective, Diuretics and
Antifertility, Toxicity studies as per OECD guidelines
Operation “Blue Star” is the only event in the history of Independent India where the state went into war with its own people. Even after about 40 years it is not clear if it was culmination of states anger over people of the region, a political game of power or start of dictatorial chapter in the democratic setup.
The people of Punjab felt alienated from main stream due to denial of their just demands during a long democratic struggle since independence. As it happen all over the word, it led to militant struggle with great loss of lives of military, police and civilian personnel. Killing of Indira Gandhi and massacre of innocent Sikhs in Delhi and other India cities was also associated with this movement.
Read| The latest issue of The Challenger is here! We are thrilled to announce that our school paper has qualified for the NATIONAL SCHOOLS PRESS CONFERENCE (NSPC) 2024. Thank you for your unwavering support and trust. Dive into the stories that made us stand out!
2. Ebstein’s Anomaly
Ebstein’s anomaly of the
tricuspid valve is a congenital
malformation, in which there
is downward (apical)
displacement of insertion of
septal and posterior leaflets.
3. History:
This anomaly was first
described by Wilhelm Ebstein
in 1866 in an autopsy of a 19-
year-old laborer who had
cyanosis and dyspnea since
early childhood.’
The very first reported case in
a live patient was in 1949 by
Tourniaire.
Wilhelm Ebstein
German physician
4. Epidemiology
• Ebstein’s anomaly occurs in 0.3-0.7% of all congenital
heart diseases
• 1 in 20,000 live births
• Equal male: female occurrence
• Mortality in children presenting in the neonatal period is 30-
50%
• Mortality at all ages is 12.5%
5. Etiology
• Congenital disease of often uncertain cause.
• Environmental factors
• Maternal ingestion of lithium in first trimester
• Maternal benzodiazepine use
• Maternal exposure to varnishing substances
• Maternal history of previous fetal loss
• Risk is higher in whites than in other races.
6. Genetic factors
• Rare cases of cardiac transcription factor NKX2.5 mutations,
10p13-p14 deletion, and 1p34.3-p36.11 deletion have been
described in the anomaly .
• Recently, Postma et al. reported the results of a
mutational analysis in a cohort of 141 unrelated probands
with Ebstein anomaly.
• Eight were found to have a mutation in the gene MYH7 and
six of the eight patients also had left ventricular
noncompaction.
• This may warrant genetic testing and family evaluation in
this subset
9. TV annulus
• The tricuspid valve is the most apically (or caudally) placed
valve with the largest orifice among the four valves.
• The tricuspid annulus is oval-shaped and when dilated
becomes more circular.
• 20% larger than MV annulus .
• Normal TV annulus= 3.0 - 3.5 cm
10. Leaflets
• The tricuspid valve has three
distinct leaflets described as
septal, anterior, and posterior.
• The septal and the anterior leaflets are
larger.
• The posterior leaflet is smaller and
appears to be of lesser functional
significance since it may be
imbricated without impairment of valve
function.
S
p
A
11. • The septal leaflet is in immediate
proximity of the membranous
ventricular septum
• The anterior leaflet is attached to
the anterolateral margin of the
annulus and is often voluminous
and sail-like in Ebstein’s anomaly.
12. Papillary Muscles & Chordae
• There are three sets of small papillary muscles, each set
being composed of up to three muscles.
• The chordae tendinae arising from each set are inserted
into two adjacent leaflets.
• The anterior set chordae insert into half of the septal and
half of the anterior leaflets.
• The medial and posterior sets are similarly related to adjacent
valve leaflets.
13. Embryologic aspect
• The leaflets and tensile apparatus of the atrio ventricular
valves seem to be formed by a process of delamination of the
inner layers of the inlet zone of the ventricles.
• Delamination of the right ventricular free wall,
continues to the level of AV junction.
• In EA, delamination of these leaflets may have failed to occur
due to an incompletely understood mechanism
• Process of delamination is incomplete and falls short of
reaching the level of the AV junction.
• The apical portions of the valve tissue fail to resorb completely.
• Distortion and displacement of the tricuspid valve leaflets,
and a part of the right ventricle becomes
14.
15. DEFINITION
• Congenital defect
• Origins of septal or posterior leaflets, or
both, are displaced downward into RV
• Leaflets are variably deformed
• Atrialization of right ventricle
• Anterior leaflet is enlarged and sail- like
16. Pathology
There are several anatomic components of Ebstein anomaly,
namely:
1. Displacement of the septal and posterior leaflets towards apex
of the right ventricle (RV).
2. Anterior leaflet is usually attached at the annular level and is
large and sail-like with multiple attachments to ventricular wall.
3. The portion of RV that is proximal to the level of the displaced
septal and posterior leaflets is called the ‘atrialized RV’ and is
usually thin and dysplastic
17. Pathology
4. RV cavity beyond the attachment of the septal and posterior
leaflets is the ‘functional RV’ and is smaller, lacks inlet portion
and has a small trabecular portion.
5. Infundibular portion of RV is sometimes obstructed by
redundant anterior leaflet or its chordal attachments.
18. Pathophysiology
Hemodynamic abnormalities depends on severity of the lesion
• Degree of displacement of the tricuspid valve
• Degree of tricuspid regurgitation.
Mild Ebstein
• Tricuspid valve function close to normal.
19. Moderate to severe forms
• with each atrial contraction, the blood is propelled into the
atrialized RV.
• With ventricular contraction that follows, the blood is forced
back into the right atrium. (More pronounced in tricuspid
regurgitation. )
• With the next atrial contraction, this blood is forced back into
the atrialized RV.
• This back and forth blood flow causes right atrial dilatation
and increases right atrial pressure;
• right to left shunt across ASD/PFOarterial desaturation and
pulmonary oligemia.
20. • In severe case, absence of flow
from ineffective RV cause
physiological pulmonary atresia.
• All the systemic venous return
must pass from right to left
across PFO or ASD.
• thus causing severe cyanosis
and acidosis.
21. Clinical Presentation
May present at any age
Fetal life:
• Diagnosed incidentally by echocardiography.
Neonatal life and infancy:
• Cyanosis and severe heart failure
• SVT
• Improve as pulmonary vascular resistance decreases.
Adult life:
• Fatigue, exertional dyspnea, cyanosis, heart failure, and palpitations
arrhythmias are common.
22. Physical examination
• Cyanosis - Varying degrees of cyanosis at various times in life
and transient worsening with arrhythmias.
• Clubbing
• Precordial asymmetry
• Usually left parasternal prominence and occasionally
right parasternal prominence.
23. JVP –
• The jugular pulse is normal except for a prominent C wave
that coincides with mobility of the anterior tricuspid leaflet.
• An attenuated X descent and a systolic venous V wave of
tricuspid regurgitation rarely appear despite severe regurgitant
flow because of ;
1. the damping effect of the commodious right atrium
2. the thin-walled toneless atrialized right ventricle
3. tricuspid regurgitation is low-pressure and hypokinetic
24. Arterial pulses
• Usually normal
• Diminished volume
Heart sounds
• First heart sound
• widely split with loud tricuspid component ( THE SAIL
SOUND )
• Mitral component - soft (long PR interval)
• Second heart sound
• widely split
25. Additional heart sounds and murmurs
Third and fourth heart sounds
• Commonly present.(QUADRUPLE RHYTHM)
• Summation of third and fourth heart sounds, especially with
prolonged PR interval, can mimic an early diastolic murmur
The systolic murmur of tricuspid regurgitation
• Medium frequency and decrescendo in nature (grade2/6 or 3/6).
• At the lower left parasternal area and sometimes at the apex .
27. ECG
• Tall P wave and prolonged PR interval
• Features of RBBB
• WPW preexcitation
• SVT
• Deep Q wave in V1 to V4 and in inferior leads
• Atrial fibrillation, atrial flutter, VT,VF
28.
29.
30. CXR
1. Cardiomegaly (Rounded or Box-like contour )
2. Decreased pulmonary vasculature
3. The infundibulum either straightens the left cardiac border
or forms a conspicuous convex shoulder.
4. The most consistent and dramatic radiologic feature is the
right atrial silhouette, which is almost always enlarged.
33. Echocardiogram
Two-dimensional
Apical displacement of the septal leaflet of greater
than 8 mm/m2 –
Abnormalities in morphology and septal
attachment of the septal and anterior tricuspid
leaflets
Eccentric leaflet coaptation
Dilated right atrium
Dilated right ventricle with decreased contractile
performance
Various left heart structural abnormalities
35. Echocardiogram from a patient with Ebstein’s anomaly. Color flow shows the jet of
tricuspid regurgitation (TR) originating at the junction of the functional right ventricle
(RV) and the atrialized right ventricle
36. Assessment of severity by Echo
• (GOSE) score, with grades 1 to 4.
• The ratio of the combined area of the RA and atrialized RV is
compared to the functional RV and left heart.
• This classification is particularly helpful with neonatal
Ebstein’s anomaly.
RA area + aRV area
fRV area+ LA area + LV area
39. Carpentier’s classification
In 1988, Carpentier et al.proposed the following classification of
Ebstein’s anomaly –
Type A: True RV volume is adequate
Type B: A large ARV but mobile anterior leaflet
Type C: The anterior leaflet movement restricted causing RVOT
obstruction
Type D: Almost complete atrialization of the ventricle except for
a small infundibular component.
41. Cardiac MRI
1. MRI is preferred for quantitative measurement of right atrial
and RV size and systolic function.
2. Provides complimentary information about TV anatomy .
3. Axial imaging provides the most reliable information about
atrialized RV volume.
4. The ability to create 3D images may also provide
greater delineation of disease severity.
42.
43.
44. Cardiac Catheterization
• Infant associated with critical pulmonary stenosis requiring
balloon valvuloplsty
• To assess intracardiac and pulmonary pressure prior to surgical
intervention.
• Selective right ventricular angiography demonstrates displaced TV
leaflets, degree of tricuspid insufficiency and size and function of
the RV.
45. Natural History
• Patient with less severe anomaly may be either asymptomatic
or mildly symptomatic.
• Cyanosis tend to improve as the PVR falls during the newborn
period. Cyanosis may appear later
• Hemodynamic deterioration with increasing cyanosis, CHF and
LV dysfunction develop later in life.
• Some 18% of symptomatic newborn die in the neonatal period;
30% of patients die before the age of 10 years.
• Attacks of SVT with associated WPW preexcitation occur in 15-
20% of all patients.
48. Medical management-
Asymptomatic children with mild Ebstein’s anomaly
Regular observation
If CHF develops due to TR—Frusemide + Digoxin
Varying degrees of activity restriction may be necessary.
Acute episode of SVT
• Adenosine
• β blocker
• Radiofrequency Catheter Ablation
49. Surgical management
Principles of Surgical Management for Ebstein Anomaly
1. Complete or subtotal closure of intra-atrial communications
2. Tricuspid valve repair or replacement
3. Elimination of arrhythmias
4. Selective plication of the atrialized RV
5. Right reduction atrioplasty
6. Repair of associated defects (VSD closure, relief of RVOT
obstruction)
50. Neonatal Ebstein
• Most of the cases do not need surgical intervention.
• Severe forms with persistent cyanosis or important associated
lesions will require intervention.
51. The surgical options in neonates are
A. Tricuspid valvuloplasty—poor outcome
B. Starnes procedure
C. Neonatal biventricular repair
D. Cardiac transplantation
52. Starnes procedure
1. Fenestrated patch closure of the
tricuspid valve orifice,
2. Enlargement of the ASD,
3. Plication of large RA (atrialized RV), and
4. Placement of a systemic-to pulmonary
artery shunt (BT shunt)
Particularly useful when there is anatomic
RVOT obstruction.
55. Surgery in Children and Adults
Indications
• Decreased exercise tolerance
• Cyanosis
• Progressive right ventricular dilatation (Cardiothoracic ratio
>60%)
• Prior to significant right ventricular dysfunction
• Onset or progression of atrial arrhythmias
• Prior to left ventricular dysfunction
56. Surgical management in children
• Cone Reconstruction
• Tricuspid Valve Repair
• Danielson “Monocusp” Repair
• Carpentier Repair
• Tricuspid Valve Replacement
• Ventricularization Procedure
• Bidirectional Cavopulmonary Connection as an Adjunct to Tricuspid
Valve Repair
• Atrialized Right Ventricular Plication and Right Atrial Reduction
• Surgical Treatment of Arrhythmias
• Cardiac Transplantation
57. Cone reconstruction
Procedure:
• Separation of adherent segment of tricuspid valve from
anatominal annulus and underlying RV.
• Cone created using released tricuspid valve tissue.
• Base of cone attached to AV junction
• Hinge point of tricuspid valve attached to AV junction.
• Atrialized portion of RV reduced by elliptical resection or
plication.
59. Cone reconstruction
Modified cone repair:
• Insertion of complete or partial annuloplasty ring.
• Selective application of Sebening stitch (approximation of
anterior papillary muscle to ventricular septum)
• BDCPA (bidirectional cavopulmonary anastomosis, 1.5 ventricle
physiology)
60. Cone reconstruction
Contraindication:
• Patient age >60 years,
• Moderate pulmonary hypertension,
• Severe LV dysfunction (EF <30%),
• Absent septal leaflet,
• Heavy muscularization of the anterior leaflet,
• Severe tricuspid valve annular dilation with massive RV
enlargement and systolic dysfunction.
61. Tricuspid valve repair
Danielson monocusp repair:
• Creation of a monocusp valve using the anterior leaflet to coapt
with the ventricular septum.
• Plication of atrialized RV
• Posterior tricuspid annuloplasty
• Right reduction atrioplasty.
63. Carpentier procedure:
• Detachment of anterior and septal leaflet
• Plication of atrialized RV
• Posterior tricuspid annuloplasty
• Reposition of leaflet and cover the orifice
• Placement of prosthetic ring
65. The Ventriculization Procedure
• This is characterized by reintegration of the atrialized portion of
the RV into the right ventricular cavity (ventricularization).
• This can be obtained by orthotopic transposition of the
detached septal and posterior leaflets of the TV.
• The reimplanted septal leaflet serves as an opposing
structure for coaptation of the reconstructed AV valve.
66. Bidirectional Cavopulmonary Connection
• The BDCPA (bidirectional cavopulmonary shunt) does two
important things in the setting of Ebstein’s anomaly.
1. Reduces venous return to the enlarged, dysfunctional RV
by approx. one-third.
2. Provides sufficient preload to the LV to sustain adequate
systemic perfusion when right-sided output is low.
67. BDCPA-
Indications for BDCPA include:
1. Severe RV enlargement and/or dysfunction,
2. Compression of the LV due to shift of the interventricular
septum,
3. Moderate tricuspid valve stenosis (mean gradient >8 mm Hg)
after CR, or
4. RA to LA pressure ratio >1.5 (an indicator of poor RV
function).
68. Outcomes
Short Term
• The severity of the valve malformation and dysfunction of both
ventricles will affect outcome.
• Early mortality in neonates has been linked to marked RV
enlargement, severe tethering of all leaflets, LV dysfunction,
and pulmonary atresia.
• Patients with severe cardiomegaly (CT ratio >0.6) may also
have lung hypoplasia.
• In the current era, early results in children are more favorable
and operative mortality is∼3% in experienced centers.
69. Outcomes
Long Term
• Most patients with Ebstein anomaly will need surgical
intervention.
• Patients with Ebstein anomaly experience a high incidence of
atrial tachyarrhythmias including atrial fibrillation and atrial
flutter.
• Long-term outcomes after surgery for valve repair and
replacement are excellent.
• Except for very ill newborns, adult survival with a good quality of
life is expected for patients with Ebstein anomaly.
70. Take home points
Ebstein anomaly is a RV myopathy with failure of TV
delamination and highly variable TV morphology with severe
regurgitation.
It is the only congenital heart lesion that has a range of clinical
presentation from the severely symptomatic neonate to an
asymptomatic adult.
Neonatal operation has high operative mortality, whereas
operation performed in childhood and adulthood has low
early mortality.
Late survival and quality of life are excellent for the vast
majority of patients in all age brackets.
71. Atrial tachyarrhythmias are the most common late
complication and increase with age.
The cone reconstruction can achieve nearly anatomic
restorations of TV anatomy and function, and early-to-
intermediate results are encouraging.
Reduced RV function continues to be a challenge for some
patients, as is the need for reoperation for recurrent TR.
Innovative surgical and regenerative medicine strategies to
address poor RV function and associated right-sided heart
failure are evolving.
