This document provides an overview of medical management in patients with Eisenmenger syndrome. It discusses:
1. The history and pathophysiology of Eisenmenger syndrome, which develops when a pre-existing left-to-right shunt reverses direction due to high pulmonary vascular resistance, causing hypoxemia.
2. The clinical presentation and complications of Eisenmenger syndrome including pulmonary hypertension, heart failure, erythrocytosis, bleeding, cholelithiasis and others.
3. Evaluations including echocardiogram, cardiac catheterization and imaging studies to characterize the underlying anatomy and assess pulmonary pressures and resistance.
4. Medical management focuses on pulmonary vasodilators like
A 30-minute talk, presented as part of the weekly teaching activities in Alder Hey Children's Hospital (Liverpool, UK). It addresses PDA evaluation in children - starting with embryology & anatomy with the basis behind physiological closure versus patency after birth. What is the role of echo study in diagnosing/evaluating PDA? Modes used with some clear movies? Its limitations?
A 30-minute talk, presented as part of the weekly teaching activities in Alder Hey Children's Hospital (Liverpool, UK). It addresses PDA evaluation in children - starting with embryology & anatomy with the basis behind physiological closure versus patency after birth. What is the role of echo study in diagnosing/evaluating PDA? Modes used with some clear movies? Its limitations?
Cardiac catheteriztion, Oximetery study in a patient with VSDPRAVEEN GUPTA
In this ppt i am going to discuss how to do cardiac catheterisation study, oximetry study and how to analyse its data in a patient with VSD who came to our hospital
Cardiac catheteriztion, Oximetery study in a patient with VSDPRAVEEN GUPTA
In this ppt i am going to discuss how to do cardiac catheterisation study, oximetry study and how to analyse its data in a patient with VSD who came to our hospital
Acyanotic Congenital Heart Diseases;
1. Left-to-right shunts
a. Ventricular Septal Defect(VSD)
b. Atrial Septal Defect(ASD)
c. Patent Ductus Arteriosus(PDA)
d. Atrioventricular Septal Defect(AVSD)
e. Aortopulmonary window
* Eisenmenger Syndrome – The shunt becomes right-to-left
2. Left-sided obstructive lesions
a. Coarctation of the Aorta(COA)
b. Congenital Aortic Stenosis
c. Mitral Stenosis
d. Interrupted Aortic Arch
Cyanotic Congenital Heart Diseases;
1. Right-to-left shunts
a. Tetralogy of Fallot
b. Pulmonary stenosis
c. Pulmonary atresia
d. Tricuspid atresia
e. Ebstein’s anomaly
2. Complete mixed lesions
a. Transposition of the great vessels
b. Double outlet right ventricle(DORV)
c. Total anomalous pulmonary venous return
d. Truncus arteriosus
e. Hypoplastic left heart syndrome
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
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.
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 Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
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
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.
1. Medical management in patients
with Eisenmenger syndrome
Dr Nishant Tyagi
Moderator: Dr S Radhakrishnan
Escorts Heart Institute And Research CentreEscorts Heart Institute And Research Centre
2. History
• 1897 Vicktor Eisenmenger
– 32 year old man with dyspnea, cyanosis,
Hemoptysis
– Autopsy showed Large VSD
• 1958 Paul Wood coined the term “Eisenmenger
Syndrome”:
• Eisenmenger Syndrome- “Defined as pulmonary vascular
obstructive disease that develops as a consequence of a
large preexisting left-to-right shunt such that pulmonary
artery pressures approach systemic levels and the
direction of the flow becomes bi-directional or right to left”.
3. Causes: L R shunt
• Atrial Shunts
– ASD: secundum
– primum,
– Common atrium
• Ventricular shunts
– VSD,
– Single Ventricle,
– TGV with VSD,
– DORV,
– AV Canal
• Aortic Shunts
– PDA,
– aortopulmonary window,
– truncus arteriosus,
– pulmonic valve atresia with large aorto-pulmonary collaterals
• Palliative, surgically created systemic-to-pulmonary
anastomosis for treatment of congenital heart disease
5. Pathophysiology
Endothelial Injury
Production of elastase
Medial hypertrophy
Cellular Intimal Proliferation
Progressive arteriolar &
capillary occlusion
Destruction of small arterioles
Plexiform lesions &
Necrosis
Leakage of serum
Release of growth factors
6. Hypothesis of PAH
• Christman et al: increased ratio of thromboxane to
prostacycline
• Stewart and Giaid et al: increased levels of endothelin
7. Histopathology
Despite different etiology, histopathology often has
the following common features:
• Heith–Edward classification: grade 1 – 6
1.Medial hypertrophy in arterioles
2.Cellular intimal fibrosis
3.Acellular intimal fibrosis
4.Plexiform lesions + dilated muscular a.+ thrombus
5.Fibrosis of media
6.Necrotizing arteritis, granulation tissue
8. • Pulmonary hypertension
– Breathlessness
– Fatigue
– Lethargy
– Chest pain
– Presyncope
– Syncope
• Heart failure
– Exertional dyspnea
– Orthopnea
– Paroxysmal nocturnal dyspnea
– Edema
– Ascites
• Erythrocytosis
– Myalgias
– Anorexia
– Fatigue
– Lassitude
– Paresthesias of the digits
– Tinnitus
– Blurred or double vision
– Headache
– Slowed mentation
– Decreased alertness
• Bleeding tendency
– Mild mucocutaneous bleeding
– Epistaxis
– Menorrhagia
– Pulmonary hemorrhage
• Cholelithiasis
– Right upper quadrant pain
– Biliary colic
– Fever
– Pale stools
– Jaundice
• Nephrolithiasis
– Renal colic
– Secondary gout
– Joint pain and swelling
• Vasodilation
– Presyncope
– Syncope
• Paradoxical embolus can cause
symptoms of localized vascular
insufficiency end-organ ischemia.
• Hypertrophic osteoarthropathy
can cause long bone pain and
tenderness.
• Retinal complications include
episodes of transient visual loss and
spontaneous hyphemas.
9. • Cardiovascular
– Central cyanosis (differential
cyanosis in the case of a PDA)
– Clubbing
– JVP: A-wave dominant, and, in
the presence of a significant
tricuspid regurgitation, the V
wave may be prominent
– Precordial palpation reveals a
right ventricular heave and,
frequently, a palpable S2.
– Loud P2
– High-pitched early diastolic
murmur of pulmonic
insufficiency
– Right-sided fourth heart sound
– Pulmonary ejection click
– A fixed, widely split S2 is
characteristic of an ASD
pansystolic murmur of tricuspid
regurgitation.
– The continuous murmur of a
PDA disappears when
Eisenmenger physiology
develops; a short systolic
murmur may remain audible.
• Other signs
– Hematologic signs include
bruising and bleeding;
funduscopic abnormalities
related to erythrocytosis include
engorged vessels, papilledema,
microaneurysms, and blot
hemorrhages.
– Jaundice, right upper quadrant
tenderness, and positive
Murphy sign (acute
cholecystitis).
– Focal ischaemia (paradoxical
embolus).
– Musculoskeletal signs include
clubbing, tenderness over the
metacarpal or metatarsal joints
(hypertrophic osteoarthropathy),
and joint effusions
• Ocular signs include
conjunctival injection, rubeosis
iridis, and retinal hyperviscosity
changes
10. Predisposition
• “Post-tricuspid valve” lesions highest risk
– VSD, PDA, truncus arteriosus
• “Large”
– Aortopumonary >0.6 cm
– VSD >1.5 cm
– For patients < 1.5 cm only 3% will develop PVOD
• Genetic determinants
11. Natural History
• 8% of CHD &11% of those with L-R shunts develops E.S.
• Size & location of the intracardiac defect decides likehood
of developing ES
• VSD-
- VSD diameter < 1.5 cm - 3% cases
- VSD diameter > 1.5 cm - 50% cases
• Large defects-
- 100% - Truncus Arteriosus
- 50% - VSD & PDA
- 10% - ASD
• Time of development ES
- VSD & PDA -80% During Infancy
- ASD -90% During Adulthood
12. Prognosis
• Survival Rate
- 80%-10 yrs
- 77%-15 yrs
- 42%-25 yrs
• Two important factors on which survival depends are mean RA
pressure and PVR
• The prognosis is not influenced by location of defect
• Variables associated with poor prognosis
- Syncope
- Increased Rt.heart filling pressures
- Severe hypoxemia (Sao2 < 85%)
• CHF usually occurs after 40 yrs of age
Vongpatanasin, W. et. al. Ann Intern Med 1998;128:745-755
13. Lab Studies:
• CBC
– hematocrit
– hemoglobin
– MCV,MCH,MCHC.
• Red cell mass
• Iron studies
– ferritin
– total iron binding capacity
– uric acid
– Urea and creatinine sometimes elevated
• Biochemical profile
– Increased conjugated bilirubin
• Bleeding time
• Urine biochemical analysis reveals proteinuria.
• Arterial blood gases
– Reduced resting PaCO2 due to resting tachypnea and reduced PaO2 due
to right-to-left shunting
– Mixed respiratory and metabolic acidosis
14. ECG
Almost always abnormal
- RVH : Tall R wave in V1, deep S wave in V6,
± ST and T wave abnormalities
-P pulmonale
± Atrial and ventricular arrhythmias
15. Chest X-Ray
-Right ventricular and right atrial enlargement
-Features of pulmonary hypertension,
dilated main pulmonary artery,
increased hilar vascular markings, and
pruned peripheral vessels
16. TTE/TEE
• Underlying cardiac defect
• RV pressure +/- volume
overload
• RVH
• RV dilatation and
hypocontractility
• Dilated main of PAs
• Elevated RVSP,Identification
of surgical systemic-to-
pulmonary shunts
• The addition of supine
bicycle ergometry can
demonstrate increased right-
to-left shunting with exercise.
• TEE is useful for imaging
posterior structures,
including the atria and
pulmonary veins.
17. Other
• Ventilation – perfusion scintigraphy
• Cardiac catheterization
– Conduit patency and pressure gradient
– RA pressure
– PA pressure
– Cardiac output
– PVR
• CT/CT Angiography
– PA enlargement, thrombosis and mural calcification
– Lung parenchyma
• Embolic infarction
• Hemorrhage
• MRI
18. Measuring PVR
• Normal=155-255dynes.sec/cm5
• Convert to woods unit by dividing by 79
• Non invasive: Peak TR vel x 10 + 0.16 in woods unit
TVI of RVOT
• Invasive : mean PAP – PCWP in woods unit
CO
• PVRI = PVR / BSA
19. Operability
Cath :
PVR index > 6 U/m2
-Reversibility testing
O2, NO, Prostacyclin
-Temporarily balloon occlusion of defect
increase in the RV filling pressure
decrease in the CO
-PVR > 0.5 of SVR
Open lung Biopsy
-Previously used to decide operability
-Drawback:
changes not uniformly distributed
Significant bleeding risk
21. Cause of Symptoms
Inability to increase pul. blood flow
Limiting oxygen uptake
Impaired exercise tolerance
Decreased SVR
Syncope
Increased R to L shunt
Increased cyanosis
22. Modes of Death
• Sudden death (30%)
• CHF (25%)
• Hemoptysis (15%)
• Pregnancy
• Noncardiac surgery
• Infection
– Brain abscess
– Endocarditis
23. Predictors of Survival
Hazard
Ratio
Standard
Error
P 95% CI
Complex CHD 4.91 3.67 0.03 1.09-22.01
Creatinine 1.01 0.003 0.002 1.00-1.01
RV Dysfunc 3.24 1,51 0.01 1.27-8.27
Age at onset 0.83 0.02 <0.001 0.77-0.88
Daliento, L Somerville, Eur Heart J 1998
-Usually do not survive beyond the second or third decade.
-The most frequent terminal event is a combination of hypoxemia and
arrhythmia in the setting of rapid increases in PVR or decreases in SVR
24. Advice to the patient and the
attendants
• Fluid balance
– Avoid dehydration,
– avoid hot humid climate
– Avoid extra salt intake
• Avoid high altitude
• Avoid competitive sports
• IE prophylaxis
– Dental and skin care
– Infections not to be taken lightly
• Haemoptysis not to be taken lightly
• Contraception in females
• Regular follow up – 3 monthly even if asymptomatic
25. Right heart failure
digoxin diuretics
However, diuretics should be used cautiously in these patients
given their preload-dependent state
Use of ASE –I is controversial as they decrease SVR
26. Oxygen therapy
– The use of oxygen supplementation is
controversial.
– Oxygen therapy has shown no impact on exercise
capacity and survival in adult patients with ES
(Bowyer, 1986; Sandoval 2001).
– Some patients might benefit from nocturnal
supplementation, although it is most useful as a
bridge to heart-lung transplant.
– Air travel appears to be safe as long as the
airplanes are adequately pressurized.
Supplemental oxygen during commercial air travel
is often recommended, but limited data exist
regarding this issue (Harinck, 1996).
27. • Epoprostenol (Flolan) -- Strong vasodilator of all vascular beds. May
decrease thrombogenesis and platelet clumping in the lungs by inhibiting
platelet aggregation.
• Continuous chronic infusion should be administered through a central
venous catheter
• Dose: Initiate infusion at 4 ng/kg/min IV less than maximum tolerated
infusion rate determined during acute dose ranging
If maximum tolerated infusion rate <5 ng/kg/min, start chronic infusion rate
at one half maximum tolerated infusion rate
• CI:Documented hypersensitivity; hyaline membrane disease; dominant left-
to-right shunt; respiratory distress syndrome; congestive heart failure due to
severe left ventricular systolic dysfunction
• In pregnancy: Usually safe but benefits must outweigh the risks
• Long-term prostacyclin therapy has shown to improve hemodynamics
(decrease in mean PA pressure, improvement in cardiac index, and
decrease in PVR) and quality of life in patients with congenital heart disease
and PAH (Rosenzweig, 1999).
• Another study evaluated epoprostenol infusion in adolescents with
congenital heart disease and Eisenmenger physiology showing improved
oxygenation (from 69% to 85%) and improvement in a 6-minute walk test
distance (from 48 yd to 375 yd) (Fernandes, 2003).
Prostacyclin analogues: Epoprostenol
29. • Bosentan (Tracleer) -- Endothelin receptor antagonist Inhibits vessel
constriction and elevation of blood pressure by competitively binding to ET-1
receptors EtA and EtB in endothelium and vascular smooth muscle. This
leads to significant increase in cardiac index (CI) associated with significant
reduction in pulmonary artery pressure, PVR, and mean right atrial
pressure.
• Dose: <40 kg: 62.5 mg PO bid; not to exceed 125 mg/d
>40 kg: 62.5 mg PO bid for 4 wk initially, then increase to 125 mg PO bid
• Contraindicated in pregnancy
• exclude pregnancy before initiating treatment and prevent thereafter by use
of reliable contraception
• not recommended while breastfeeding
• Causes at least 3-fold elevation of liver aminotransferases (ie, ALT, AST) in
about 11% of patients; avoid in patients with moderate or severe liver
impairment
Endothelin receptor antagonist:
Bosentan
30. • A 16-week, multicenter, randomized, double-blind, placebo-controlled study
• 54 patients were randomized 2:1 to bosentan (n=37) or placebo (n=17) for
16 weeks
• Primary end point : Compared with placebo, bosentan reduced pulmonary
vascular resistance index (-472.0 dyne.s.cm(-5); P=0.0383)
• Secondary end point : mean pulmonary arterial pressure decreased (-5.5
mm Hg; P=0.0363), and the exercise capacity increased (53.1 m; P=0.0079)
• CONCLUSIONS: In this first placebo-controlled trial in patients with
Eisenmenger syndrome, bosentan was well tolerated and improved exercise
capacity and hemodynamics without compromising peripheral oxygen
saturation.
Nazzareno et al:Circulation. 2006 Jul 4;114(1):48-54. Epub 2006 Jun 26.
Bosentan Randomized Trial of Endothelin
Antagonist Therapy-5 (BREATHE-5)
31. Cont..
• Clinical course of 43 patients (M/F 13/30, age 34.0 +/- 12.7 years),
registered with ES, was retrospectively analysed. These patients were
divided into two groups: those treated with and those treated without
advanced therapy(prostacyclin analogues, endothelin receptor
antagonists)
• Total cohort was followed for a median period of 4.9 (range 0.2-14.9)
years
• Mean survival time for patients treated with (n = 26) and without (n =
17) advanced therapy therapies were 8.5 +/- 1.5 and 8.5 +/- 0.9 years,
respectively (log rank testing, P = 0.31)
• Mean time to death or inscription on the active waiting list was
significantly longer for patients treated with advanced therapy when
compared with those without (7.8 +/- 1.0 vs. 3.4 +/- 0.9 years, P =
0.006)
• CONCLUSION: no improvement in survival time could be documented
in adult patients with unstable ES treated with advanced therapy.
However, the need for heart-lung transplantation can be substantially
delayed with new drugs.
Adriaenssens et al, Eur Heart J 2006 Jun; 27(12): 1472-7
32. Phosphodiesterase (type 5) enzyme
inhibitors
• Sildenafil (Revatio) -- Promotes selective smooth muscle relaxation
in lung vasculature possibly by inhibiting PDE5. This results in
subsequent reduction of blood pressure in pulmonary arteries and
increase in cardiac output.
• Dose: 20 mg PO tid
• Pregnancy: Usually safe but benefits must outweigh the risks.
• Adverse effects include
– headaches (16%),
– flushing (10%),
– upset stomach (7%),
– nasal congestion (4%),
– adverse effects occur more often in men taking the 100 mg dose
– those who are taking nitrates
– rates of MI are 1.7 and 1.4 per 100 man-years for sildenafil and placebo
groups
33. Cont..
• Randomized, double-blind, placebo-controlled crossover study
• Twenty patients, 10 of each of idiopathic PAH and ES, were
randomized to receive placebo or sildenafil in a double-blind manner for
6 weeks
• Primary end point :of distance covered in 6-minute walk test improved
from 262 +/- 99 to 358.9 +/- 96.5 m (P < .0001)
• Secondary end point :Pulmonary artery pressure, improved from the
baseline of 98.8 +/- 20.5 to 78.3 +/- 15.3 mm Hg (P < .0001), NYHA
class improved from 2.65 +/- 0.59 to 1.55 +/- 0.51 (P < .0001), exercise
duration from 6.4 +/- 3.1 to 10.2 +/- 2.05 minutes (P < .0001), and Mets
achieved from 3.32 +/- 1.57 to 6.04 +/- 1.87 (P < .0001)
• CONCLUSIONS: Sildenafil significantly improved the symptomatic
status, exercise capacity, NYHA class, and hemodynamic parameters
of patients with severe PAH and can be safely used as a primary or
adjunctive treatment of the same.
Singh et al chandigarh, Am Heart J. 2006 Apr;151(4):851.e1-5
34. CCBs and ACE - I
• Use of CCB nifedipine showed increased exercise
toleranceand decrease in PVR
– Its use is still investigational
• Hopkins et al showed the role of ACE I in 10 patients of
ES increasing the functional capacity
– its use is still controversial
35. Erythrocytosis
– Almost always present in patients with ES.
– Phlebotomy is not usually recommended, with exceptions
• presence of hyperviscosity symptoms or
• hematocrit level is > 65%.
– Before initiating phlebotomy rule out
• dehydration since it can falsely increase the hematocrit level
• iron deficiency anemia: have apparent normal hematocrit level
and low MCV. The iron deficient erythrocytes are less
deformable than normal erythrocytes, and this lack of
deformability can worsen hyperviscosity
– Procedure: Venesect 250-500 mL of blood and replace with an
equivalent volume of isotonic sodium chloride (or 5% dextrose if in
heart failure).
– Volume = Hct (patient) – expected Hct(60) x Wt x 70
Hct (patient)
36. Vongpatanasin, W. et. al. Ann Intern Med 1998;128:745-755
Management of the patient with the Eisenmenger
syndrome and Erythrocytosis
37. Endocarditis
– Patients with ES are at very high risk for endocarditis.
– Infective endocarditis prophylaxis - chemotherapeutic
– nonchemotherapeutic
• Encourage good oral hygiene (soft-bristle tooth brushing
twice a day, mouthwash or hydrogen peroxide rinses,
semi-annual dental visits).
• Skin care advice
• Nail biting should be avoided.
38. Thrombotic and bleeding
complications
• Prone to thrombotic events due to hyperviscosity.
• Are also susceptible to bleeding due to platelets
dysfunction.
• One study by Silversides et al reported that the
incidence of proximal pulmonary artery thrombus in this
patient population was 21%.
• Anticoagulation is still not routinely used.
39. Systemic Issues
• Hyperuricemia
– Due to decreased excretion
– Uric acid rises in proportion of cyanosis
– No therapy if asymptomatic
– Gout tx: allopurinol, colchicine
• Avoid NSAIDs b/c platelet function/hemostasis
• Pigment gallstones
– RBC turnover increases unconjugated bilirubin
– Risk of acute cholecystits and emergent surgery
• Scoliosis 25-30% patients w/CHD
• Renal dysfunction
– IV hydration prior to radiographic contrast
40. Contraception, pregnancy, and
genetic counseling
– Avoid pregnancy. Tubal ligation is strongly
recommended
– If patients refuse tubal ligation, OCs are preferred
over IUDs, which can cause significant menorrhagia
and potentially increase the risk of endocarditis.
– The fetal mortality rate is approximately 25%, and the
maternal mortality rate exceeds 50%.
– Therapeutic abortion is recommended for women in
the early stages of pregnancy.
– The risk of congenital heart defects in offspring is
approximately 10%
41. Maternal and Fetal considerations with
pregnancy
– Excessive straining should be avoided during the second stage of
labor. Therefore, assisted delivery usually is recommended.
– The most critical time is postpartum, and the majority of deaths
occur in the first week.
– Factors that increase the risk of a peripartum death include
congestive heart failure, sudden increases in pulmonary vascular
resistance or decreases in SVR, bleeding/anemia, hematocrit
greater than 60%, oxygen saturation less than 80%, and syncope.
– CS carries a higher mortality rate and should be reserved for
obstetric indications,
– The use of anticoagulants is controversial.
– ensure proper leg care, use of elastic bandages, sufficient
hydration, and early mobilization to prevent deep venous
thrombosis.
– The main risks to the fetus include arterial oxygen desaturation,
hypoxemia, and polycythemia
– The fetal mortality rate 25%.
– Only 15% of babies are born at term.
– Fetal echocardiography is recommended for pregnant patients or
siblings.
42. Vongpatanasin, W. et. al. Ann Intern Med 1998;128:745-755
Pooled Data from Studies of Pregnant Patients with the
Eisenmenger Syndrome
43. Indications for Hospitalization
• Severe hemoptytis
• Absolute decrease O2 sat > 10% from baseline (or
<65%)
• Severe RV failure
– Intravenous inotropic agents and diuretics
• Arrhythmia
• Pregnancy
44. When to go for Transplantation
• Reserve for severely symptomatic patients when 1 year survival is
expected to be < 50%
• 2002 Registry (International society of heart and lung
transplantation)
– 605 Eisenmenger’s patients
• 430 pts heart/lung tx
• 106 bilateral lung tx
• 69 single lung tx
– Patient survival greater in HLT compared to isolated lung
• 81 vs.68% at 30 days
• 70 vs. 55% at 1 year
– Benefit greatest in patients with VSD
Wassell TK et al J Heart Lung Transplant 2002;21:731
45. yes
General advice
Avoid dehydration
Less salt intake
Avoid competitive sports
Dental hygiene
IE prophylaxis
Contraception in females
Digoxin
Diuretics
ACE I ??
Hematocrit >65%
Symptoms of hyper viscosity
A patient comes with cyanosis and clubbing
Non invasive evaluation – echo + TEE
CHD with PAH with R->L or bidirectional shunt
Cath : reversibility Corrective procedure
Eisenmenger S.
no
Manage CCF
venesection
Advanced therapies
Epoprostenol
Bosentan
Sildenafil
When I year survival < 50%
Detiorating RV function
Dropping O2 saturation
HLT
Echo findings in pulmonary hypertension ?tei index, diastolic prarmeters…?
Significant reading, filling for cath in PVOD
If close defect in patient with Eisenmengers will change course to one similar to PPH.
Older age of onset of symptoms the better the survival\younger age of onset of symptoms worse survival
IV hydration prior to IV contrast even with a normal BUN/Cr
Peripartum and first several weeks postpartum –circ 1994 Presbitero
Reserve for severely symptomatic patients as the survival is usually quite good with medical management.The outcome for transplant is reasonable. The largest experience comes from a review of 605 patients with Eisenmengers syndrome in the United NEtweork for organ sharing/international society of H and l trnsplant joint registry