The syndrome of sudden Death, right bundle branch block and ST elevation was firstly described by A.Nava and B. Martini in 1988-1989, and only five years later by the Brugada Brothers. The ECG pattern is due to a conduction disturbance of the RVOT, caused by fibrofatty substitution of that structure.
History of ICDs (Internal Cardiac Defibrillators)Jose Osorio
ICDs have been available since the 80s for the prevention of sudden cardiac death. The advancements are quite amazing, with a reduction in size from >250cc to less than 40cc, ease of implantation, safety and longevity.
History of ICDs (Internal Cardiac Defibrillators)Jose Osorio
ICDs have been available since the 80s for the prevention of sudden cardiac death. The advancements are quite amazing, with a reduction in size from >250cc to less than 40cc, ease of implantation, safety and longevity.
Hypocalcemia induced camel-hump t-wave, tee-pee sign, and bradycardia in a ca...YasserMohammedHassan1
Rationale: Electrocardiographic is a fundamental tool for a cardiologist, critical care physician, and emergency medicine specialist. The electrolyte imbalance is a very important entity in clinical medicine management. Camel-hump T-wave and the Tee-Pee sign, recently; Wavy triple and Wavy double signs of hypocalcemia (Yasser’s sign) are electrocardiographic findings linked to electrolyte deficiencies. Patient concerns: A middle-aged male car-painter patient presented to the emergency department with atypical severe twisting chest pain, hypocalcemia, hypokalemia, and hypernatremia.
Diagnosis: Hypocalcemia-induced Camel-hump T-wave, Tee Pee sign, Wavy double sign of hypocalcemia (Yasser’s sign), and bradycardia in a car- painter. Interventions: Electrocardiography, arterial blood gases, oxygenation, and echocardiography. Lessons: The dramatic reversal of Camel-hump T-Wave, Tee-Pee sign, Wavy double sign of hypocalcemia (Yasser’s sign) after calcium gluconate injection interpret that these signs were due to hypocalcemia. The twisting chest pain and its limited disappearance immediately after calcium gluconate injection indicate the pain can be named as “chest tetany”. Non-atropine bradycardia response is evidence that the management of the cause of bradycardia sometimes is essential e.g. hypocalcemia in the current case. Outcomes: There was a dramatic response of both clinical and electrocardiography including Camel-hump T-wave, Tee Pee sign, the wavy double sign of hypocalcemia, and bradycardia.
ECG in Emergency Department - Advances in ACS ECGDr.Mahmoud Abbas
ECG in Emergency Department -Advances in ACS ECG. Lecture presented by Dr Hesham Ibrahim at the Egyptian Critical Care Summit , the leading educational event and medical exhibition in Egypt.
EMGuideWire's Radiology Reading Room: Stress-Induced CardiomyopathySean M. Fox
The Department of Emergency Medicine at Carolinas Medical Center is passionate about education! Dr. Michael Gibbs is a world-renowned clinician and educator and has helped guide numerous young clinicians on the long path of Mastery of Emergency Medical Care. With his oversight, the EMGuideWire team aim to help augment our understanding of emergent imaging. You can follow along with the EMGuideWire.com team as they post these educational, self-guided radiology slides or you can also use this section to learn more in-depth about specific conditions and diseases. This Radiology Reading Room pertains to Stress-Induced Cardiomyopathy and is brought to you by Jenna Pallansch, MD, Claire Lawson, NP, Shelby Hixson, PA, Emily Lipsitz, PA, Ashley Moore-Gibbs, DNP, Laszlo Littmann, MD, and John Symanski, MD.
COVID-19 Pneumonia with Atrial Fibrillation, Coronary Spasm, and Wavy Triple ...YasserMohammedHassan1
Rationale: A novel COVID-19 with severe acute respiratory syndrome had arisen in Wuhan, China in December 2019 Arrhythmias are commonly recognized sequel in COVID-19 patients. Interestingly, the presentation of COVID-19 infection with a newly coronary artery spasm has a risk impact on both morbidity and mortality of COVID-19 patients. Wavy triple an electrocardiographic sign (Yasser Sign) is a new innovated diagnostic sign in hypocalcemia. Patient concerns: An elderly farmer male COVID-19 patient presented to physician outpatient clinic with bilateral pneumonia, atrial fibrillation, evidence of coronary artery spasm, and Wavy triple an electrocardiographic sign (Yasser Sign). Diagnosis: COVID-19 pneumonia with coronary artery spasm and the Wavy triple an electrocardiographic sign (Yasser Sign). Interventions: Chest CT scan, electrocardiography, oxygenation, and echocardiography. Outcomes: Gradual dramatic clinical, electrocardiographic, and radiological improvement had happened. Lessons: The reversal of electrocardiographic ST-segment depressions in a COVID-19 patient after adding oral nitroglycerine is an indicator for the presence of coronary artery spasm. It signifies the role of the anti-infective drugs, anticoagulants, antiplatelet, and steroids in COVID-19 patients with bilateral pneumonia, AF, coronary artery spasm are effective therapies. The disappearance of AF after initial therapy may a guide for a good prognosis in this case study. The evanescence of Wavy triple ECG sign as a hallmark for the existence of the Movable-weaning phenomenon of hypocalcemia is recommended for further wide-study.
A woman in her late 40s with a history of hypertension presented to the emergency department after multiple episodes of palpitations with near syncope. While in the
emergency department, she developed monomorphic ventricular tachycardia (VT) with hemodynamic instability and was successfully cardioverted. She continued to have nonsustained monomorphic VT, so intravenous amiodarone and oral metoprolol were initiated. She was admitted for further evaluation. Results of tests of electrolyte levels and coronary angiography were normal. Cardiac magnetic resonance imaging with
gadolinium contrast revealed normal-sized cardiac chambers and normal biventricular
function without delayed enhancement. The presenting electrocardiogram (ECG)
is shown in Figure 1.
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
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!
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
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
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
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.
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.
1. The “so called” Brugada
syndrome
The true history!
Bortolo Martini M.D.
Bortolo.martini@gmail.com
January 2014
2. Stephen M. Stigler is
Stephen M. Stigler is
Professor of Statistics at
Professor of Statistics at
the University of Chicago.
the University of Chicago.
Stigler’s law
• no scientific
discovery is
named after its
original
discoverer.
4. First oral presentation of a new “clinicalECG syndrome” at the Italian
Association of Cardiology : 1988
5. Nava-sign
• This is the first e.c.g.
trace of a patient with
the syndrome (not an
healty men!) published
by Andrea Nava in
Medical literature.
• Mises a Jour
Cardiologiques
1988;17:157-159
6. This is an ECG and not a syndrome !!!!!!!!!!!!!!!!!!!
7. Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R,
Daliento L, Dalla VS: Ventricular fibrillation without apparent heart
disease: description of six cases. A m Heart J 1989, 118:1203-1209
• 1989. The first
complete description
of the syndrome!!!
• Nava-Martini-Thiene
or Brugada syndrome?
8. In this article all the typical ECG pattern of
In this article all the typical ECG pattern of
the syndrome were described
the syndrome were described
9. The 2nd paper on the syndrome was published in Japan!
The 2nd paper on the syndrome was published in Japan!
Shinzo 1990;22(suppl 2):80
Shinzo 1990;22(suppl 2):80
11. What is the syndrome of sudden
death, RBBB and ST elevation?
• A clinico – ECG association (Not an isolated
ECG!!!)
• A disease with familial involvement, mainly
affecting males
• ECG pattern of RBBB (different degrees), and ST
elevation, often dynamic (not only 3 patterns!!),
mostly due to a conduction delay at the RVOT
• Easily inducible VF
• Organic substrate in ALL the cases submitted to
detailed necropsy study
12. A clinico – ECG association (Not an
isolated ECG!!!)
13. A disease with familial involvement,
mainly affecting males
14. ECG pattern of RBBB (different degrees), and ST
elevation, often dynamic (not only 3 patterns!!),
mostly due to a conduction delay at the RVOT
In this article all the ECG patterns of
the syndrome were described
15.
16. The ecg pattern
The ecg pattern
is due to
is due to
CONDUCTION
CONDUCTION
DELAY AT THE
DELAY AT THE
rvot.
rvot.
Nava 1988
Nava 1988
17. HV interval prolongation indicates ORGANIC
HV interval prolongation indicates ORGANIC
HEART DISEASE
HEART DISEASE
HV interval in the pts with the syndrome
Brugada JACC 1992
18. Late potentials (which mean late
Late potentials (which mean late
depolarization abnormality) are often
depolarization abnormality) are often
present, and can always be induced
present, and can always be induced
with flecainide
with flecainide
22. Prevalence of Nava-sign (coved ECG in V1) in
Healthy population.
Reference Total
sujects
Miyasaka 13929
2001
Tohyou 4092
1995
Viskin
592
2000
Hermida 1000
2000
Overall 19613
Mean age Men
Total
examined Prev.
58
27%
0,12%
Men Prev. Women
Prev.
0,38%
0,03%
46
78%
0,07%
0.09%
0,00%
36
58%
0,00%
0,00%
0,00%
39
63%
0,10%
0,16%
0,00%
0,11%
0,23%
0,03%
23. Mortality at follow up of asymptomatic subjects with
Jwave+coved/saddle-back ecg
Suravicz (Editorial) JACC 2001;38:775
Author
Number
Follow up
Sudden death
3
8%
(normal subjects)
Brugada
JCE 2001;12:7-8
????????
Priori
30
1-3
0%
34
1-3
0%
11
3-4
0%
98
2,6
1%
32
1-14
22,4%
"unpublished"
observations in 239
pts/subjects with the
Brugada ECG"
Circulation 2000;102:2509
Atarashi
AJC 1996;78:581
Takenaka
JCE 2001;12:2
Miyasaka
JACC 2001;38:771
Matsuo
JACC 2001;38:765
(mean age 57!!!)
24. PTS with the syndrome of RBB+ST
elevation and sudden death
T o t a l P a t ie n t s 2 9 2
(G IA C 1 9 9 9 ":1 5 7 -7 7 )
4 9 p a t ie n t s
h a d s o m e c a r d ia c
a b n o r m a lit ie s p a r t ic u la r ly
o f t h e r ig h t v e n t r ic le
241 had no
r e c o g n iz e d h e a r t
d is e a s e
b u t , 1 6 o f B r u g a d a s e r ie s
h a d " n o n s p e c ip h ic a b n o r m a lit ie s "
25. What is the
What is the
pathopysiology of these
pathopysiology of these
ECGs?? Two theories
ECGs?? Two theories
26. The organic theory
• The syndrome is due to a concealed right
ventricular pathology (not the typical right
ventricular cardiomyopathy/dysplasia)
which induces a conduction disturbance at
the septal level (responible for the RBBB
pattern), and infundibular (responsible for
the ST elevation).
• All the cases submitted to autopsy have
organic heart disease !!!
27. The Cardiac Pathology of Sudden,
Unexplained Nocturnal Death in
Southeast Asian Refugees
Kirshner RH JAMA 1986;256:2700
• 18 hearts examined
• 14 had cardiomegaly
• 17 had conduction system
abnormalities:
• 14 persistent fetal
dispersion of AV node or
His
• 13 accessory conduction
fibers
• 1 congenital a-v block
28. Familial Cardiomyopathy Underlies Syndrome of RBBB, ST Segment Elevation and
Sudden Death
Corrado D, Nava A, Buja G, Martini B, Thiene G.
JACC 1996;27:443-8
•
A: atrophy, fibrosis, adiposis
of the RVFW
•
B: severe fibrosis of the
bifurcating His bundle with
sclerotic interruption of right
bundle branch
The ecg may be due to aalesion of the conduction
The ecg may be due to lesion of the conduction
tissue both at septal and infundibular level
tissue both at septal and infundibular level
29. Arrhytmogenic Right Ventricular Cardiomyopathy Underlies Syndrome
of Right Bundle Branch Block, ST-Segment Elevation, and Sudden Death
Tada H. Am J Cardiol 1998;81:519
• a) necropsy study in
pt. 2 shows RVC
• b) histologic specimen
from pt 1, at operation
shows RVC
30. The syndrome of right bundle branch block, persistent ST segment elevation
and sudden cardiac death. Which is the histological substrate?
Morgera T. Eur Heart J 1997;18:1190
31.
32. Right Bundle Branch Block, Right Precordial ST-Segment Elevation, and
Sudden Death in Young People
Domenico Corrado, MD; Cristina Basso, MD, PhD; Gianfranco Buja, MD; Andrea Nava,
MD; Lino Rossi, MD; Gaetano Thiene, MD
Circulation.2001;103:710.
Circulation.2001;103:710.
33. Localized RV morphological anomalyes detected by electron beam CT represent arrhythmogenic substrates in pts with Brugada
Syndrome.
Takagy-Aihara Eur Heart J 2001;22:1032-41
34. Localized RV morphological anomalyes detected by electron beam CT represent arrhythmogenic substrates in pts with Brugada
Syndrome.
Takagy-Aihara Eur Heart J 2001;22:1032-41
• Evidence-based: 81% of 26 pts with the syndrome and
coved or Saddle ST had RV abnormalities, mostly in
RVOT. High correlation between QRS morpology of
ectopic beats and RVWMA.
• Brugada interpretation: “the morphological
abnormalityes are secondary to electrical abnormality:
conduction defect and abnormal repolarization”
Brugada Eur Heart J 2001;22:982-4
• Antzelevitch interpretation: localized stunned
myocardium, which can later become organic lesions
secondary to localized SCN5 abormalities. 2001 Public email
35. The functional theory
• The disorder is due to a functional disorder of
repolarization, genetically determined by SCN5A
abnormalities with produced a notch and absence
of the dome in epicardial layers, wich are
responsible for ventricular reentry arrhythmias.
These ecg abnormalities can be evidentiated by
class 1c drugs
• This theory is based on experimental work on Left
Ventricle
• There is not a single anatomic evidence.
36. Brugada-Antzelevitch-Gussak
theories
•
•
•
•
•
•
1992: “prolonged HV suggest His-Purkinje disease”. “Marked dispersion of
refractory periods or extreme anisotropic conduction
1994: disorder related to “M cells”
1996: IT0 channels involvement
1998 mutations of SCN5A genes inducing eterogenicity in epicardial and
endocardial AP in 50% of pts with the ECG
The available data suggest that the Brugada syndrome is a familial primary
electrical disease caused by a defect in an ion channel gene, resulting in
premature repolarization of some right ventricular epicardial sites.
Gussak,Antzelevitch JACC 1998;33:5-15
“the morphological abnormalityes are secondary to electrical conduction
defect and abnormal repolarization” Brugada Eur Heart J 2001;22:982-4
38. The experiment
The experiment
of Antzelevitch
of Antzelevitch
were devoted to
were devoted to
explain the JJ
explain the
wave in the Left
wave in the Left
Ventricle !!!
Ventricle !!!
39. Images sometime change to demonstrate an
undemonstrable truth
• A) Original imagine by
Antzelevitch. Note V6.
Circulation1996;93:372
• B) Recent imagine by
Alings. Note that V1 has
substitute V6. Moreover,
when J wave is present ST
elevation disappear and
vice-versa. Circulation
1999;99:666
40. The significance of Jwave+st:
science and science fiction
Experiment
Experiment
show nonshow noncoincident
coincident
spontaneous
spontaneous
epicardial
epicardial
notch and loss
notch and loss
of the dome
of the dome
after drug.
after drug.
Note the
Note the
morphology of
morphology of
ECG leads
ECG leads
which
which
rensembles aa
rensembles
V6
V6
Epicardial in vivo
Epicardial in vivo
recording does
recording does
not show
not show
absence of
absence of
epicardial dome
epicardial dome
(2002)
(2002)
Drawing by
Drawing by
Align, transform
Align, transform
the experimental
the experimental
V6 in V1, and
V6 in V1, and
confirms that if
confirms that if
you have JJwave,
you have wave,
you do not have
you do not have
ST elevation
ST elevation
41. Prevalence of SCN5A
abnormalities
• Brugada:50%,
• Priori:20%,
• Breithard: 10%
Does presence of SCNA
Does presence of SCNA
abnormality exclude organic
abnormality exclude organic
heart disease?
heart disease?
43. Variant of SCN5A Sodium Channel Implicated in Risk of
Cardiac Arrhythmia
Igor Splawski,1* Katherine W. Timothy,2 Michihiro Tateyama,3 Colleen E. Clancy,3 Alka Malhotra,2 Alan H. Beggs,4 Francesco P.
Cappuccio,5 Giuseppe A. Sagnella,6 Robert S. Kass,3 Mark T. Keating1*
Every year, ~450,000 individuals in the United States die suddenly of cardiac
arrhythmia. We identified a variant of the cardiac sodium channel gene SCN5A that is
associated with arrhythmia in African Americans (P = 0.000028) and linked with
arrhythmia risk in an African-American family (P = 0.005). In transfected cells, the
variant allele (Y1102) accelerated channel activation, increasing the likelihood of
abnormal cardiac repolarization and arrhythmia. About 13.2% of African Americans
carry the Y1102 allele. Because Y1102 has a subtle effect on risk, most carriers will
never have an arrhythmia. However, Y1102 may be a useful molecular marker for the
prediction of arrhythmia susceptibility in the context of additional acquired risk factors
such as the use of certain medications.
Science 2002 297: 1252
45. Flecainide challenge
• This test has been proposed to make a
speciphic diagnosis of the syndrome.
• Despite enthusiasm this has not beeen
confirmed
• Flecainide is retained to induce
repolarization abnormalities, but all the
evidence is that the drug induces a
depolarization disturbance !!!
46. Ajmaline Test
Ajmaline Test
proposed by
proposed by
Brugada,
Brugada,
should induce
should induce
aatypical
typical
functional
functional
repolarization
repolarization
abnormality
abnormality
47. But Flecainide has effects on depolarization
But Flecainide has effects on depolarization
and not on repolarization ! !
and not on repolarization
MECHANISMS OF THE PROARRHYTHMIC
EFFECTS OF FLECAINIDE AND RELATED
ATIARRHYTHMIC DRUGS
• “Class 1C drugs induce a depression in
conduction property of the electrical impulse”
• “This effect is due to a decrease in the number
of Na+ channels available during phase 0”
• “The upstroke of phase 0 is decreased and
conduction velocity is depressed”
Brugada J.
Brugada J.
The New Frontiers of Arrhythmias.
The New Frontiers of Arrhythmias.
1992, pag. 353
1992, pag. 353
48. Science and science fiction
Experiments with
Experiments with
flecainide, do not induce
flecainide, do not induce
JJwave or ST elevation
wave or ST elevation
But drawing can
But drawing can
do that !!!
do that !!!
49. Flacainide test induces repolarization or
depolarization abnormality?
No late
No late
potentials
potentials
Induction of late
Induction of late
potentials, can
potentials, can
be only due to aa
be only due to
conduction
conduction
disturbance and
disturbance and
not to
not to
repolarizattion
repolarizattion
abnormality !!
abnormality !!
51. Flecainide is speciphic for a functional disorder
Brugada, Circulation 2000
• But
• This patient has RVC
• flecainide test is
positive
• Linkage analysis
shows chromosome 14
involvement
53. Clinical and genetic Heterogeneity of RBBB and
ST_Segment elevation Syndrome.
A prospective evaluation in 52 Families
Priori Circulation 2000;102:2509
• 15% prevalence of SCN
% genes abnormalities
• No SD in asymptomatic
• Limited value of PES
(PPV50%, NPV 46%)
• Flecainide challenge
unable to unmask silent
gene carriers (PPV 35%)
54. Organic and Functional syndromes:
differences and similarities
Nava-Martini-Thiene
Clinical
Sudden death due to VF,
Picture
in middle aged males
Ecg
1)J wave + coved, saddle
or dome ST
2)RBBB+LAD+PR>
3)Isolate ST abnormality
4)Class 1c +
HV interval
Not rarely Prolonged
Late potentials Positive
RVOT delay
Present
Familiarity
Present
Eco, Angio,
Often positive for organic
NMR, electron heart disease
beam CT
Biopsy
Fibrosis, adiposis
Necropsy
Right Ventricular
Cardiomyopathy+ His
lesion
Chromosome 14 ??
Chromosome ???
Genetic
abnormality
Brugada
Same
Same
Same
Same
Not investigated
Present
Always negative.
Recent admission
of localized
abnormalities
(stunned M)
“Non speciphic”
abnormalities
Never performed
SCN5A??
Chromosome 3??
56. The brugada syndrome. Do we need more than the
12-lead ECG?
J. Farré Eur Heart J 2000-21-264
• The syndrome is a
Clinical-ECG
association.
• The ECG pattern itself is
not speciphic
• Flecainide is not
speciphic
• Syncope may be vagal
• We need research tools
other than the ECG
57. The syndrome of sudden death,
RBBB and ST elevation
• Nobody has yet the true !!
58. Andrea Nava and Bortolo Martini
Andrea Nava and Bortolo Martini
59. New book 2013
•
Chapter 1
•
•
ARARE LETHAL SYNDROME IN SEARCH
OF ITS IDENTITY: SUDDEN DEATH, RIGHT
• BUNDLE BRANCH BLOCK AND ST
• SEGMENT ELEVATION
•
•
Bortolo Martini,1,* Jiashin Wu2 and Andrea Nava3
1Director of the Cardiovascular Unit, Boldrini Hospital, Thiene, Italy
•
2University of South Florida, US
•
3Associate Professor of Cardiology, University of Padua, Italy
Brugada Mutations
InsAA – failure of channel to express
Frameshift Mutation-premature stop at 1397 – 4340delA S5-S6 of Domain III – failure to express
R1432G (also bet S5 and S6 of Domain III) – failure to express
1795InsD – Brugada and LQT – positive shift of activation curve (negative shift of inactivation) reduced Ina…
T1620M – positive shift of activation curve and markedly faster inactivation - reduced Ina during phase 1…
L567Q – accelerated inactivation (small positive shit of activation and negative shift of inactivation - reduced Ina during phase 1.
A1924T negative shift of activation curve (-9 mV) - cannot explain phenotype
R1512W negative shift of activation (-5.1 mV) and small negative shift of inactivation (-3.8 mV) curves – cannot explain phenotype