This document provides an overview of Ebstein's anomaly, a congenital heart defect characterized by abnormal attachment of the tricuspid valve leaflets to the myocardium. It discusses the history and typical features of the condition, including displaced valve leaflets, enlargement of the right ventricle and atrium, and arrhythmias. The document outlines evaluation with echocardiogram, ECG and chest x-ray, and indicates interventions like tricuspid valve repair are recommended for those with cyanosis, heart failure or decreased functional capacity. While neonatal Ebstein's carries a poor prognosis, satisfactory valve repair can result in good medium and long-term outcomes.
In this ppt i am going to discuss various spotters, including ECG, X-ray, fluroscopy images and there answers. These spotter now days asked in various DM cardiology exam conducted all over India, so it will help you in your DM Cardiology exam preperationn.
preop TEE assessment of atrial septal defect is very important for making decision for device closure, properly assessed adequate rims of ASD will reduce risk of device embolization to almost nil.
In this ppt i am going to discuss various spotters, including ECG, X-ray, fluroscopy images and there answers. These spotter now days asked in various DM cardiology exam conducted all over India, so it will help you in your DM Cardiology exam preperationn.
preop TEE assessment of atrial septal defect is very important for making decision for device closure, properly assessed adequate rims of ASD will reduce risk of device embolization to almost nil.
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.
Our concepts of heart disease are based on the enormous reservoir of physiologic and anatomic knowledge derived from the past 70 years' of experience in the cardiac catheterization laboratory.
As Andre Cournand remarked in his Nobel lecture of December 11, 1956, the cardiac catheter was the key in the lock.
By turning this key, Cournand and his colleagues led us into a new era in the understanding of normal and disordered cardiac function in huma
Left ventricular non compaction is rare congenital cardiomyopathy with gaining interest due to advancement in imaging modalities for diagnosis and assessment of undulating phenotype
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.
Our concepts of heart disease are based on the enormous reservoir of physiologic and anatomic knowledge derived from the past 70 years' of experience in the cardiac catheterization laboratory.
As Andre Cournand remarked in his Nobel lecture of December 11, 1956, the cardiac catheter was the key in the lock.
By turning this key, Cournand and his colleagues led us into a new era in the understanding of normal and disordered cardiac function in huma
Left ventricular non compaction is rare congenital cardiomyopathy with gaining interest due to advancement in imaging modalities for diagnosis and assessment of undulating phenotype
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.
Most common type of birth defect
Defect in structure or function of the heart and great vessels
1 in 1000 live births
The incidence is higher in stillborns (3-4%), spontaneous abortuses (10-25%), and premature infants
About 1 in 4 babies born with a heart defect has a critical heart disease
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle.
A detailed discussion on embryogenesis of heart and ennumeration of all congenital diseases and description of cyanotic congenital heart disease , each disease in detail.
Similar to Ebsteins anomaly dr hafeesh fazulu (20)
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
HOT NEW PRODUCT! BIG SALES FAST SHIPPING NOW FROM CHINA!! EU KU DB BK substit...GL Anaacs
Contact us if you are interested:
Email / Skype : kefaya1771@gmail.com
Threema: PXHY5PDH
New BATCH Ku !!! MUCH IN DEMAND FAST SALE EVERY BATCH HAPPY GOOD EFFECT BIG BATCH !
Contact me on Threema or skype to start big business!!
Hot-sale products:
NEW HOT EUTYLONE WHITE CRYSTAL!!
5cl-adba precursor (semi finished )
5cl-adba raw materials
ADBB precursor (semi finished )
ADBB raw materials
APVP powder
5fadb/4f-adb
Jwh018 / Jwh210
Eutylone crystal
Protonitazene (hydrochloride) CAS: 119276-01-6
Flubrotizolam CAS: 57801-95-3
Metonitazene CAS: 14680-51-4
Payment terms: Western Union,MoneyGram,Bitcoin or USDT.
Deliver Time: Usually 7-15days
Shipping method: FedEx, TNT, DHL,UPS etc.Our deliveries are 100% safe, fast, reliable and discreet.
Samples will be sent for your evaluation!If you are interested in, please contact me, let's talk details.
We specializes in exporting high quality Research chemical, medical intermediate, Pharmaceutical chemicals and so on. Products are exported to USA, Canada, France, Korea, Japan,Russia, Southeast Asia and other countries.
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Factory Supply Best Quality Pmk Oil CAS 28578–16–7 PMK Powder in Stockrebeccabio
Factory Supply Best Quality Pmk Oil CAS 28578–16–7 PMK Powder in Stock
Telegram: bmksupplier
signal: +85264872720
threema: TUD4A6YC
You can contact me on Telegram or Threema
Communicate promptly and reply
Free of customs clearance, Double Clearance 100% pass delivery to USA, Canada, Spain, Germany, Netherland, Poland, Italy, Sweden, UK, Czech Republic, Australia, Mexico, Russia, Ukraine, Kazakhstan.Door to door service
Hot Selling Organic intermediates
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Anti ulcer drugs and their Advance pharmacology ||
Anti-ulcer drugs are medications used to prevent and treat ulcers in the stomach and upper part of the small intestine (duodenal ulcers). These ulcers are often caused by an imbalance between stomach acid and the mucosal lining, which protects the stomach lining.
||Scope: Overview of various classes of anti-ulcer drugs, their mechanisms of action, indications, side effects, and clinical considerations.
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
2. HISTORY
• 1866 – Autopsy Report by Wilhelm Ebstein
reported a strikingly ABNORMAL Tricuspid
valve
• Anterior leaflet – normal
• But SEPTAL & INFERIOR leaflets were
abnormal
• 1950 – clinically recognised.
4. • Normal tricuspid leaflets consist of basal attachments to the annulus
(right atrioventricular sulcus), peripheral zones into which chordae
tendineae insert, and clear zones that lie between the basal
attachments and the peripheral zones.
• The semicircular or quadrangular anterior leaflet is the largest of the
three.
• The posterior leaflet is scalloped.
5. • The septal leaflet attaches chiefly to the
ventricular septum , but part of its basal
attachment is to the posteriorwall of the right
ventricle.
• The septal leaflet normally exhibits a slight but
distinct apical displacement of its basal
attachment compared with the mitral valve
6. MORPHOLOGY in EBSTEINS
1. Adherence of the septal and inferior leaflets to the underlying
myocardium.
2. Downward displacement of the functional annulus.
3. Dilation of the “atrialized” portion of the RV, with variable degrees of
thinning of the free wall.
4. Redundancy, fenestrations and tethering of the anterior leaflet.
5. Dilation of the right AV junction (true tricuspid annulus).
11. EPIDEMOLOGY
• 0.3-0.8% of all congenital heart diseases.
• 1 in 20,000-50,000 live births.
• Equal male: female occurrence.
• The relative risk of Ebstein’s anomaly is increased by 500-fold in
offspring exposed to in utero lithium carbonate (Danish registery).
• Mortality in the neonatal period is 20%-40%. and less than 50%
survive to 5 years.
• Mortality at all ages is 12.5%
12. • ASD/PFO in 80-94%
• Additional associated anomalies : VSD +/- pulmonary atresia RVOT
obstruction PDA Coarctation
• Left sided lesions in 39%: MVP, systolic dysfunction,
Subaorticstenosis, BAV, LV muscle bands, LV noncompaction in 18%.
• Accessory conduction pathway in 15-20%, usually near the orifice of
TV.
• CCTGA (15-50% of CCTGA patients meet criteria for EA).
13. ETIOLOGY & GENETIC FACTORS
• More common in twins.
• Family history of congenital heart disease
• Maternal exposure to benzodiazepines.
• Maternal lithium therapy.
• Most cases are sporadic.
• Familial Ebstein’s anomaly is rare.
14. Genetic factors
• Rare cases of cardiac transcription factor NKX2.5 mutations,
• 10p13-p14 deletion, 1p34.3-p36.11 deletion have been described in
the anomaly .
15. NATURAL HISTORY
• Depends on severity
• When valve deformity is extreme – death
in utero
• Severe – Symtoms in Neonate
• Moderate – Symtoms in Adolescence or
young adult
• Mild – survival to 9th decade reported.
16. CLINICAL RECOGNITION
• 1994 Study Celermajer et al – 60% recognised before age 1
• 10% are undiagnosed into adulthood
17. CLINICAL FEATURES
• Most common presentation by age:
• Fetus: abnormal routine scan (86%)
• Neonates: cyanosis (74%)
• Infants: heart failure (43%)
• Children: Incidental murmur (63%)
• Teens and adults: arrhythmia (42%), decreased exercise tolerance,
fatigue, right-sided failure, paradoxical embolism, brain abscess.
18. Physical Examination
• Cyanosis - Varying degrees of cyanosis at various times in life and
transient worsening with arrhythmias.
• Precordial asymmetry
• Usually left parasternal prominence and occasionally right parasternal
prominence.
• Absent left parasternal (ie, right ventricular) lift an important negative
sign.
19. • 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
• usually is normal
• widely split when the pulmonary component is delayed due to RBBB.
20. • 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 .
• Murmur intensity and duration doesnot increase during inspiration.
• The timing and quality of systolic and diastolic murmurs occasionally
create the impression of a pericardial friction rub.
21. Arrythmias
• On average, 35–50% of patients had arrhythmias at some time.
• In some studies there was an association between arrhythmias and
advancing age or increasing amount of tricuspid regurgitation.
• Over half of the arrhythmias are supraventricular tachycardias of
various types.
• About one-quarter are paroxysmal or sustained atrialfibrillation or
flutter.
• A few are high-grade conduction defects. A minority are ventricular
tachycardia, ventricular fibrillation.
23. ECG
• Tall wide P waves
• RBBB – 72% to 92%
• Severity is related to abnormal function of the SEPTAL leaflet
• PR Prolongation – due to RA enlargement and long intra-atrial
conduction times
• Accesoory pathways – Ventricular preexcitation seen in 10-30%
24.
25. CHEST XRAY
• Severe Cardiomegaly
• Cardiothoracic ratio > 0.6
• Was considered an indication for surgery
30. Neonatal Ebstein
• Neonatal Ebstein
• Poor prognosis
• Reported survival only 68%
• Indiactions for surgery
• Heart failure
• Profound cyanosis
31. Initial management
• Prostaglandin infusion
• Initiation of mechanical Ventillation
• Management of pulmonary Hypertension
a) Nitric oxide
b) Sildenafil
• Diuretics for CHF
33. INTERVENTIONAL OPTIONS
• TV repair preferred to replacement
• Repair fesibility – depends on ANTERIOR leaflet adequacy to form a
monocusp /cone like structure
34. • 1970’s – Mayo Clinic – Danielson Monocusp Repair
• 1988 – Fench – Carpentier Repair
• 2004 – Ullman et al – Ventriculisation
• 2007 – De Silva et al – Cone Reconstruction
35. TV Repair – CONE Reconstruction
Dr Da Silva (Brazil)
The schematic demonstrates apical displacement (*) of the septal leaflet of the tricuspid valve and the displaced, long (sail-like) anterior leaflet. Note the large atrialized portion of the RV and dilated TVA.
EC – endomyocardial layer
• Brickner ME,et al N Engl J Med. 2000;342:334 –342.
• Attenhoferet al. Mayo Clin Proc. 2005;80:361–368 .
• Anderson et alCirculation. 1978;58(suppl):I-87–I-91.
Calermejer
The cone repair described by Dr. da Silva from Brazil
when the anatomy allows, as this technique is the most
anatomic of all the repair techniques described.
Specially, some septal leaflet should be present, which
facilitates this repair technique.
The cone technique represents the most anatomic repair
by completion of the delamination process of the TV,
providing 360 degrees of leafet tissue around the AV
junction with its hinge point at the AV groove (true
annulus).