This document discusses Eisenmenger syndrome, a condition where pulmonary hypertension develops due to increased blood flow through defects between the systemic and pulmonary circulations. It provides details on causes, clinical features, pathology findings, and treatments. Key points include:
- Eisenmenger syndrome is caused by defects like VSDs, ASDs, and PDA that allow high blood flow to the lungs and cause pulmonary hypertension over time.
- Common causes of death include hemoptysis from pulmonary artery ruptures, heart failure, and complications from attempted defect repair surgery.
- Pathological findings show thickened pulmonary arteries that resemble the fetal pattern and contribute to high pulmonary vascular resistance.
- Medical treatments are generally ineffective once int
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.
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.
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
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.
The lecture is for medical student. It is from Dr RUSINGIZA Emmanuel, MD, senior lecture at UR( UNIVERSITY OF RWANDA) .
It will help to understand heart diseases in newborn, infants and children.
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
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.
The lecture is for medical student. It is from Dr RUSINGIZA Emmanuel, MD, senior lecture at UR( UNIVERSITY OF RWANDA) .
It will help to understand heart diseases in newborn, infants and children.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
most common congenital cyanotic heart disease.one of the conotruncal family of heart lesions.. It accounts for 7 to 10% of all congenital heart abnormalities.
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.
Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
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
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
Title: Sense of Taste
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 structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
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.
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
Knee anatomy and clinical tests 2024.pdfvimalpl1234
This includes all relevant anatomy and clinical tests compiled from standard textbooks, Campbell,netter etc..It is comprehensive and best suited for orthopaedicians and orthopaedic residents.
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.
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
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...
EISENMENGER SYNDROME- PAUL WOOD
1. THE EISENMENGER SYNDROME
OR
PULMONARY HYPERTENSION WITH
REVERSED CENTRAL SHUNT
-PAUL WOOD
Paper presented by:
DR. MURTAZA KAMAL
MBBS, MD, DNB
DNB-SS FELLOW PEDIATRIC CARDIOLOGY
DOP: 08/02/2018
murtaza.vmmc@gmail.com
1
5. CAUSES
Patent ductus arteriosus
Aorto-pulmonary septal defect
Persistent truncus
Transposition with ventricular septal defect
Corrected transposition with ventricular septal defect
Ventricular septal defect
Single ventricle
Common atrioventricular canal
Persistent ostium primum atrial septal defect
Single atrium and
Hemianomalous or total anomalous pulmonary venous drainage into the right
side of heart
5
6. DATA
127 cases of Eisenmenger syndrome
Clinical and physiological features analyses
Special emphasis on distinguishing
characteristics of each type
53 necropsis from well investigated during life
studied
6
8. MODE OF DEATH
Cause of death known : 42
Haemoptysis: MCC, 29%
Profuse, Death in minutes
Cause:
Pulmonary infarction from arterial thrombosis
Rupture of a thin walled dilated arteriole immediately
distal to end of a thick muscular artery
Rupture of aneurysm of PA (Most unusual)
8
9. MODE OF DEATH
Surgical repair of defect: 26%
CHF: 17%
VF: 14%
IE/ Cerebral abscess/ Cerebral thrombosis/ Pregnancy
induced: 5%
9
10. Accuracy of Diagnosis
15 necropsied case: 9 correct, 2 wrong, 4 (Correct in respect
to site of shunt, wrong in respect to nature of defect)
40% error: Embarrassing
Same in 38 borrowed cases+ published literatures
Eg:
Simple OS ASD TAPVD into SVC
Single ventricle with intact IAS Common AV canal with
functionally single atrium + virtually single ventricle
10
11. SIZE OF THE DEFECT
No case of PDA with Eisenmenger reaction has ever
been reported with a lumen < 5 mm
No case of Eisenmenger's complex has ever been
reported with a VSD < 1cm
Size of an ASD has relatively little bearing on presence or
absence of a high PVR, and varies freely between 3- 8
cm in both uncomplicated and pulmonary hypertensive
groups
11
12. SIZE OF COMMUNICATION
Normal resistance
usual range
Eisenmnger Range
DUCT .1-1cm .7-1.5 cm
VSD .1-2cm 1.5-3cm
ASD 3-8 cm 3-8 cm
12
13. SIZES
Have in common a large communication b/w 2 circulations
If PVR were normal These would produce Qp/Qs at least
3.5 (4-6)
13
Aorto- pulmonary 0.7 cm
Inter ventricular 1.5 cm
Inter arterial 3 cm
14. ELASTIC ARTERIES
In fetus/ neonates: Elastic fibers in PA; Densely packed +long
as in aorta
As PAP falls Fibers break up, shorten, become loosely
arranged
Found that sections of PA in EC and PDA with reversed shunt
Always resembled foetal (aortic ) pattern
Whereas in ASD with PH and reversed shunt+ all forms of
acquired PH Appearance like a normal PA
14
15. CONCLUDED
PH in ES must be present from birth with VSD and PDA,
but must be acquired in ASD
Function of elastic fibers: To prevent distention from high
pressures
15
16. MUSCULAR ARTERIES
In EC, PAs with diameter< 1 mm have a thick muscular
media very similar to their structure in foetus
It was persistence of foetal type of muscular artery that was
responsible for high resistance, and that this saved infants
with large VSDs from dying as a result of an inadequate
blood flow into aorta (Civin, Edwards)
In their own words, " the pulmonary vascular bed of patients
with EC who survive infancy maintains its foetal function."
16
17. MUSCULAR ARTERIES
They assumed: Thick muscular arteries of foetus were
responsible for high PVR necessary to deflect blood from
RV away from lungs into descending aorta via PDA
During first 2 years of life intima usually remains normal
Thereafter with varying degree and tempo from case to
case, progressive intimal fibrosis develops (Edwards, 1957)
17
18. ARTERIOLES
Arterioles of lung: Vessels < 100u
Consist of endothelium, a single elastic layer, and
adventitia; they are not supplied with muscle
Usually thin-walled and dilated in ES, and that thick-walled
vessels within usually accepted compass of an arteriole
are really muscular arteries in a state of contraction
18
19. THE OVERRIDING AORTA
1. VSD is but one of a total of some 12 kinds of
communication between two circulations, all of which may
be complicated by a high PVR and reversed shunt of similar
magnitude
An overriding aorta or its physiological equivalent is absent
in 7 of these conditions
2. When PVR is high, SPO2 averages close to 80% in
transposition with VSD, persistent truncus, single ventricle,
Taussig-Bing syndrome and EC with or without apparent
override
19
20. 3. EC with all classical features may be associated with a
defect in muscular part of IVS as in cases described by
Weiss (1927) and Heath et al. (1956)
There can be no overriding of the aorta in such instances
20
21. EXPERIMENTAL PHYSIO-PATHOLOGY
Reaction of pulmonary circulation in Eisenmenger's
syndrome to pulmonary vasoconstrictor and vasodilator
drugs
O2:
Adults/ majority children>5: 100% O2 fails to lower
PAP or influence shunt magnitude
Other hand: 10%O2 raises PVR ad increases
reversed shunt (at least in PDA)
Infants< 2 and occasionally small children: When
intimal fibrosis in pulmonary circulation negligible:
100% O2 halves resistance and increases PBF
21
22. Failure of 100% oxygen to lower PV
pressure in case of EC
22
23. ACETYLCHOLINE
When injected direct into PA in a dose of 0.5-1.5 mg.: An ideal
selective pulmonary vasodilator, for this quantity is usually
inactivated by cholinesterase by the time it reaches systemic
circulation (Wood et al., 1957)
When severe PH was primary, or due to MS or cor pulmonale,
acetylcholine lowered PAP and resistance, and raised CO in
85% of cases (Wood, 1958)
In 13 cases of Eisenmenger's syndrome, however, no such
effect could be demonstrated in a single instance
23
25. The youngest patient in series, however, was 5 years old, and
it is probable that infants under 2, without intimal fibrosis,
would react differently
This belief is founded on the different response of these infants
to 100% oxygen, and on the fact that acetylcholine lowers PVR
sharply in foetal lung (Dawes, 1958)
Negative reactions to tolazoline hydrochloride (" priscol ") and
aminophylline have also been without exception in cases of
Eisenmenger's syndrome in patients over 5
25
26. PROTECTIVE EFFECT OF
STENOSIS
Vessels which are protected from extreme pulmonary
hypertension, or which have not had time to develop serious
intimal fibrosis secondary to hypertension, respond briskly to
acetylcholine
This disposes of the idea that there is anything fundamentally
wrong with pulmonary vessels at birth inES
Eg: Follows
26
27. 27
Pressure pulses in a case of ES
associated with an AP septal defect and
stenosis of RPA near its origin
Pressure pulse from RPA beyond the
stricture, showing a marked reduction of
pressure following the
injection of acetylcholine. The pulmonary
vessels have been protected by the
stenosis and are functioning normally
28. This functional evidence stands solidly side by side with
anatomical observation that when origin of LPA is atresic
in ES, its distal branches and muscular arteries, which are
not subjected to high pressure, are perfectly normal
Whereas vessels on other side have all features
characteristic of Eisenmenger's complex (McKim and
Wiglesworth, 1954)
28
29. COURSE & PROGNOSIS
ES commonly established at birth or during the first two years
of life when shunt is aorto-pulmonary or interventricular
But is typically acquired when shunt is interatrial
In present series, cyanosis dated from infancy in 80% of VSD,
but did not start until adult life in 92% ofASD
Onset of reversed shunt in PDA: More difficult to establish,
because differential cyanosis usually overlooked
Aorto-pulmonary septal defects, however, behave likeVSDs
29
30. General rule: Neither cyanosis nor breathlessness changes
appreciably during childhood or adolescence, but
deterioration sets in sooner or later in adult life
Onset of recurrent haemoptysis may signal downward trend
which is due in many cases to the development of
thromboobstructive lesions
Average age of natural death :
33: Aortopulmonary defects
33: VSDs
36: ASDs
30
31. Downhill course usually punctuated with haemoptyses,
which when severe sometimes caused sudden collapse and
death within a few minutes
Bacterial endocarditis: 5% for death
Cerebral abscess from paradoxical embolism occurred once
and proved fatal
Cerebral thrombosis in one case, but not fatal
14% died abruptly, presumably from VF
26% died from a surgical attempt to repair defect, mostly
from ligation of a patent ductus
Patients who survive these risks develop CHF sooner or
later, death in 17% of the fatal cases
31
32. TREATMENT
Medical treatment: Aims at preventing secondary
thromboobstructive lesions by means of permanent
anticoagulants, such as phenindione (" dindevan ")
Pulmonary vasodilators (tolazoline, aminophylline,and
reserpine::Fail to lowerPVR, and do not influence course of
disease
Small venesections, repeated from time to time: May be
helpful in cases with gross polycythaemia
Heart failure management
32
33. Surgical repair of defect: Responsible for more deaths in ES
than any other agent except haemoptysis
Ellis et al. (1956): Surgical mortality in cases with reversed
shunt through a patent ductus was 56
33
34. Direct repair of a defect is justified only when it can be
predicted with confidence that its closure must result in a fall of
pulmonary blood pressure because a hyperkinetic factor of
sufficient magnitude has been eliminated
In practice this prediction can be made when PBF is at least
twice the systemic flow at rest, and PVR does not exceed 10
units (800 dynes sec./cm.')
Borderline cases have Qp/Qs 1.75- 2, and resistances of 10 to
12 units
No case with PBF <1.75 times the systemic flow or with a
resistance over 12 units should have defect repaired in the
ordinary way, whether the shunt is reversed, direct, or
bidirectional
34
35. SYNTHESIS
CO of newborn babe is about 0.5 1./min., and is presumably
much same in foetus at term
0.1 1./min. passes through pulmonary circulation
Since mean PAP is same as aortic, about 50 mm. Hg at birth,
foetal pulmonary vascular resistance must be about 500 units
(40,000 dynes sec./cm.5)
During 1st 3 hours after birth a reversed shunt through PDA is
invariable;
From 3-72 hours after birth shunt may be direct, bidirectional
or reversed and by end of 3rd day duct is usually closed
It follows that in newborn infants PVR is more or less same
as the systemic-that is, about 100 units
35
36. Within a remarkably short time after birth, then, probably
within a few minutes of independent life, a PVR around 500
units falls to about 100
4 questions clamour for an answer:
(1) What is the cause of the fantastic PVR in the foetus?
(2) Why does it drop so precipitously at birth ?
(3) Why is the rapid fall halted temporarily more or less at
systemic level ?
(4) What is responsible for the subsequent slower decline to
normal ?
In the correct answer to these four questions lies the secret
of the Eisenmenger syndrome
36
37. What is the cause of the fantastic PVR in the
foetus?
Resistance to flow in a tube is greatly increased by kinking
the vessel
In the fully developed foetus the pulmonary vessels are as
long as they are a few minutes after birth, but since the lung
is completely collapsed they must be coiled up, or kinked in
innumerable places
Burton has demonstrated their "gnarled" appearance
Vessels in such a state may be highly resistant to flow
37
38. Answer to the second question:
As the lungs expand the vessels uncoil: as the kinks
straighten out the resistance falls; as the blood begins to flow
through the lungs the vessels must dilate, and this also lowers
the resistance
Blood that fills up pulmonary vascular bed acts as a natural
scaffolding and encourages further expansion (Jaykkai, 1957)
Stepwise, and in a matter of a minute or so, and aided by
lusty cries, the lungs are fully expanded (Lind and Wegelius,
1956)
Vessels are wholly unfurled, and PVR is at systemic level, at
least five times lower than in utero
38
39. Why is the rapid fall in resistance at birth
temporarily halted at systemic level ?
This may well be a function of the thick muscular arteries
just mentioned
In the foetus these have developed in relation to the
systemic pressure to which they have been subjected, and,
as shown by Dawes (1958), they are also in a state of active
vasoconstriction, which can be released by acetylcholine
After birth a powerful RV, as big and strong as the left,
pumps its contents into a system of vessels which differs
little from the systemic circulation;it is only natural, therefore,
that pulmonary and systemic resistances should be similar
39
40. During 1st of life, then, physiological and anatomical findings
are identical with ES associated with PDA
2events of great importance
1st: Relaxing effect of an increased alveolar oxygen tension
on the tone of muscular arteries
2nd: Closure of PDA
Before the duct closes, release of vasoconstrictor tone in the
muscular arteries is met by an increased flow from aorta to
PA, and this hyperkinetic factor tends to maintain PA pressure
at systemic level
40
41. When duct closes, and this hyperkinetic factor is eliminated,
release of vasoconstrictor tone lowers the pulmonary artery
pressure, and the approach to normal adult physiology
begins
Just how quickly this goal is reached depends on the speed
of involution of the muscular arteries
Complete involution takes about three months
41
42. What is responsible for the subsequent slower
decline to normal ?
(1) As the pressure falls in the pulmonary circulation
vasoconstrictor tone is diminished, for muscular arteries
react to increased pressure by contracting and to decreased
pressure by relaxing (Bayliss, 1902)
This local response of muscular arteries to any change in
pressure must increase that change in either direction
(2) As the resistance declines PBF increases
This must dilate vessels and so lower the resistance still
further (Williams, 1954)
(3) As the pressure falls the muscular arteries atrophy and
so diminish their resistance to flow
42
43. THE EISENMENGER REACTION
All that is necessary to prevent these natural forces from
lowering PVR to normal is a sufficient increase of flow to
keep the pressure at systemic level
Any large AP or IV communication will do just this, and the
higher the resistance the less increase of flow necessary to
maintain the pressure
If the pressure is maintained pulmonary vasoconstrictor tone
must remain high, and the muscular arteries will not atrophy
This is the situation inES, and it will be understood that it
must be established at birth if the defect is larg enough
43
44. If the defect is not quite critical, however, the shunt flow at rest
may not be sufficient to maintain pressure at systemic level,
and the forces acting in the opposite direction may win or
partially win the competition
Once resistance falls appreciably much larger flows are
necessary to maintain high pressure
44