This document discusses different types of channelopathies, which are inherited arrhythmogenic diseases caused by mutations in ion channel genes. It summarizes the definitions, epidemiology, clinical manifestations, risk stratification approaches, and management recommendations for several major channelopathies:
- Long QT syndrome is associated with prolonged cardiac repolarization and can cause syncope or cardiac arrest. Risk stratification considers clinical factors and genetic testing, and management includes beta-blockers and ICDs.
- Brugada syndrome causes ST segment elevation and is a common cause of sudden cardiac death. Risk is assessed based on symptoms and ECG patterns, and ICDs are often recommended for high-risk patients.
- Cate
differentiating between supraventicular tachycardia and ventricular tachycardia in wide complex rhythm is always confusing and management is totally different. correct diagnosis will make dramatic difference in patient management.
differentiating between supraventicular tachycardia and ventricular tachycardia in wide complex rhythm is always confusing and management is totally different. correct diagnosis will make dramatic difference in patient management.
The long QT syndrome (LQTS) is a rare inherited heart condition in which delayed repolarization of the heart following a heartbeat increases the risk of episodes of torsades de pointes (TDP, a form of irregular heartbeat that originates from the ventricles). These episodes may lead to palpitations, fainting and sudden death due to ventricular fibrillation. Episodes may be provoked by various stimuli, depending on the subtype of the condition.The condition is so named because of the appearances of the electrocardiogram (ECG/EKG), on which there is prolongation of the QT interval. In some individuals the QT prolongation occurs only after the administration of certain medications.
Tachycardias are broadly categorized based upon the width of the QRS complex on the electrocardiogram (ECG). A narrow QRS complex (<120 milliseconds) reflects rapid activation of the ventricles via the normal His-Purkinje system, which in turn suggests that the arrhythmia originates above or within the His bundle (ie, a supraventricular tachycardia). The site of origin may be in the sinus node, the atria, the atrioventricular (AV) node, the His bundle, or some combination of these sites. A widened QRS (≥120 milliseconds) occurs when ventricular activation is abnormally slow. The most common reason that a QRS is widened is because the arrhythmia originates below the His bundle in the bundle branches, Purkinje fibers, or ventricular myocardium (eg, ventricular tachycardia). Alternatively, a supraventricular arrhythmia can produce a widened QRS if there are either pre-existing or rate-related abnormalities within the His-Purkinje system (eg, supraventricular tachycardia with aberrancy), or if conduction occurs over an accessory pathway. Thus, wide QRS complex tachycardias may be either supraventricular or ventricular in origin.
The long QT syndrome (LQTS) is a rare inherited heart condition in which delayed repolarization of the heart following a heartbeat increases the risk of episodes of torsades de pointes (TDP, a form of irregular heartbeat that originates from the ventricles). These episodes may lead to palpitations, fainting and sudden death due to ventricular fibrillation. Episodes may be provoked by various stimuli, depending on the subtype of the condition.The condition is so named because of the appearances of the electrocardiogram (ECG/EKG), on which there is prolongation of the QT interval. In some individuals the QT prolongation occurs only after the administration of certain medications.
Tachycardias are broadly categorized based upon the width of the QRS complex on the electrocardiogram (ECG). A narrow QRS complex (<120 milliseconds) reflects rapid activation of the ventricles via the normal His-Purkinje system, which in turn suggests that the arrhythmia originates above or within the His bundle (ie, a supraventricular tachycardia). The site of origin may be in the sinus node, the atria, the atrioventricular (AV) node, the His bundle, or some combination of these sites. A widened QRS (≥120 milliseconds) occurs when ventricular activation is abnormally slow. The most common reason that a QRS is widened is because the arrhythmia originates below the His bundle in the bundle branches, Purkinje fibers, or ventricular myocardium (eg, ventricular tachycardia). Alternatively, a supraventricular arrhythmia can produce a widened QRS if there are either pre-existing or rate-related abnormalities within the His-Purkinje system (eg, supraventricular tachycardia with aberrancy), or if conduction occurs over an accessory pathway. Thus, wide QRS complex tachycardias may be either supraventricular or ventricular in origin.
Early repolarization (ER), consisting of a J-point elevation, notching or slurring of the terminal portion of the R wave (J wave), and tall/symmetric T wave, is a common finding on the 12-lead electrocardiogram. For decades, it has been considered as benign, barring sporadic case reports and basic electrophysiology research that suggested a critical role of the J wave in the pathogenesis of idiopathic ventricular fibrillation (VF). In 2007-2008, a high prevalence of ER in patients with idiopathic VF was reported and subsequent studies reinforced the results. This PPT describes the current state of knowledge concerning ER syndrome associated with sudden cardiac death.
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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
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
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
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
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!
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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.
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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.
Acute scrotum is a general term referring to an emergency condition affecting the contents or the wall of the scrotum.
There are a number of conditions that present acutely, predominantly with pain and/or swelling
A careful and detailed history and examination, and in some cases, investigations allow differentiation between these diagnoses. A prompt diagnosis is essential as the patient may require urgent surgical intervention
Testicular torsion refers to twisting of the spermatic cord, causing ischaemia of the testicle.
Testicular torsion results from inadequate fixation of the testis to the tunica vaginalis producing ischemia from reduced arterial inflow and venous outflow obstruction.
The prevalence of testicular torsion in adult patients hospitalized with acute scrotal pain is approximately 25 to 50 percent
Triangles of Neck and Clinical Correlation by Dr. RIG.pptx
Approach to Channelopathies ppt
1. Approach to
Channelopathies
DR. NEERAJ VARYANI
ASSISTANT PROFESSOR
CARDIOLOGY DEPARTMENT
SUPER SPECIALITY PAEDIATRIC
HOSPITAL & POST GRADUATE
TEACHING INSTITUTE, NOIDA
2. Definition: Group of inherited arrhythmogenic diseases
(IADs) caused by mutation on genes encoding for ion
channel proteins and proteins that regulate ion
channels.
Incomplete penetrance and variable expressivity -
distinctive ECG patterns may be concealed.
Although rare they are potentially fatal but
preventable causes of sudden cardiac death (SCD) in
children.
3.
4. Comprehensive management of IADs include
diagnosing and treating the proband and
identifying and protecting affected family
members.
The concept of cardiogenetics and molecular
autopsy.
Case based rather than a disease based strategy
is the key to success.
5. Classification of
Channelopathies
Channelopathies Associated With Prolonged
Repolarization- Long QT syndrome (LQTS)
Channelopathies Associated With Abbreviated
Repolarization and Conduction Defects- Brigade
Syndrome (BrS), Short QT syndrome (SQTS), early
repolarization syndrome, Sinus node dysfunction, and
progressive conduction defects and overlap syndromes.
Channelopathies Associated With Abnormal
Calcium Handling- CPVT
6.
7. LQTS
Definitions and epidemiology: either QTc>=480ms in repeated
12-lead ECGs or LQTS risk score ( Schwartz score) >3 or
presence of confirmed pathogenic mutation irrespective of the QT
duration in the absence of secondary causes for QT prolongation(
COR: I LOE: C).
Presence of QTc>=460ms in repeated 12-lead ECGs in patients
with an unexplained syncopal episode in absence of secondary
causes for QT prolongation.
QT interval measurement- traces with constant RR interval for at
least 10 to 20 beats and excluding traces with HR >100 bpm and
< 50 bpm.
8.
9.
10. Mean age- 12-14 years. Manifest with syncope and cardiac
arrest.
Annual rate of SCD in untreated patients is 0.33 - 0.9% and
syncope is around 5%.
10 to 35% of genetically affected patients show normal QTc.
QTc is most important indicator of risk.
Mutation in 13-19 genes have been associated with LQTS.
Genetic screening identifies a disease causing mutation in 75% of
cases and 3 main genes (KCNQ1,KCNH2 and SCN5A) account
for 90% of positively genotyped cases.
11.
12.
13. Subtypes of LQTS may be grouped into 3 categories:
Autosomal dominant LQTS (Romano-Ward syndrome; prevalence 1 in 2500.
Autosomal dominant LQTS with extra cardiac manifestation,
- LQT7 (Anderson Tawil syndrome) has mild QT prolongation with
prominent U wave, polymorphic or bidirectional VT, facial dysmorphisms and
hyper-/hypokalemic periodic paralysis and LQT8 ( Timothy syndrome)
characterized by marked QT prolongation, syndactyly, paroxysmal
hypoglycemia, atrioventricular block, congenital heart defects, autism and
immunodeficiency.
Autosomal Recessive LQTS ( Jervell and Lange-Nielsen syndrome),
prolonged QT with sensineural deafness.
14. Risk stratification and management in LQTS
Clinical, electrocardiographic and genetic parameters are
considered for risk stratification.
Survivors of cardiac arrest have a high risk of recurrence
even on beta-blockers(14% within 5 years on therapy): this
evidence supports use of ICD in this group. ( COR I LOE B)
Syncope and/or VT on beta-blockers: ICD (COR IIa LOE B)
Asymptomatic carriers of KCNH2 or SCN5A when
QTc>500ms: ICD ( COR IIb LOE C)
15.
16. Silent carriers of pathogenic mutations : risk of cardiac
events around 10% between birth and age 40 years; beta-
blockers should be considered.(COR IIa LOE B)
Women with LQTS have an increased risk during the 9
month post-partum period(especially women with LQT2
Genotype) prophylactic ICD may be considered.
Patients with QTc > 500ms and signs of electrical instability
and patients with high risk genetic features (Jervell and
Lange-Nielsen syndrome or Timothy syndrome)-
prophylactic ICD may be.
17. Lifestyle changes: avoidance of QT prolonging
drugs(http://www.crediblemeds.org);correct
dyselectrolytemia;avoidance of genotype specific triggers.(COR I LOE
B)
Beta-blockers recommended with diagnosis of LQTS.(COR I LOE B)
Left cardiac sympathetic denervation (LCSD) in symptomatic LQTS
- beta-blockers not effective, not tolerated or contraindicated;ICD
therapy contraindicated or refused; patients on beta-blockers with an
ICD experience multiple shocks.(COR IIa LOE C)
Sodium channel blockers (mexiletine,flecainide or ranolazine) may
be considered as add on therapy for LQTS3 with QTc>500ms.(COR
IIb LOE C)
18. Brugada syndrome
“Right bundle branch block, persistent ST segment elevation, and
sudden death syndrome”/“ sudden unexplained nocturnal death
syndrome”;bangungut in Philippines,pokkuri in Japan or lai tai in
thailand.
Definitions and epidemiology: ST-segment elevation with type 1
(coved) morphology>= 2mm in one or more leads among the right
precordial leads V1 and/or V2 positioned in the second, third or fourth
intercostal space, occurring either spontaneously or after provocative
drug test with intravenous sodium channel blockers ( ajmaline,
flecainide,procainamide or pilsicainide) ( COR I LOE C)
South east Asia > western countries; prevalence 1 in 1000 to 1 in
10000.
19.
20. Autosomal dominant with age and sex related penetrance.
Mean age 3rd to 4th decade; M:F= 8:1(hormones); during
sleep or rest.
Incidence of arrhythmic events( VT/VF, appropriate ICD
therapy or sudden death): 13.5%/yr ( h/o cardiac arrest),
3.2%/yr ( h/o syncope) and 1%/yr (asymptomatic).
12 to 24 genes have been associated with BrS.
Results of genetic screening do not influence prognosis or
treatment.
21.
22.
23. Risk stratification and management in BrS
Survivors of aborted cardiac arrest and/or documented
sustained VT : ICD recommended (COR I LOE C)
Spontaneous type 1 ECG pattern and h/o syncope : ICD
should be considered (COR IIa LOE C)
ICD may be considered for VF during PVS with two or three
extra stimuli at two sites. (COR IIb LOE C)
Catheter ablation may be considered with h/o electrical storms
or repeated appropriate ICD shocks. (COR IIb LOE C)
24. Quinidine and isoproterenol should be considered in patients
with BrS with electrical storms.(COR IIa LOE C)
Quinidine should be considered in patients who qualify for
ICD but present a contraindication or refuse it and to treat
Supraventricular arrhythmias.(COR IIa LOE C)
Life style changes: Avoid drugs that may induce ST-segment
elevation in right precordial
leads(http://www.brugadadrugs.org);avoid excessive alcohol
intake and large meals; prompt treatment of fever with
antipyretics. (COR I LOE C)
25. Short QT syndrome
(SQTS)
Definitions and epidemiology:
Diagnosis: QTc<=340ms(COR I LOE C).
SQTS should be considered with QTc<= 360ms and one or more of the following:
confirmed pathogenic mutation; family h/o SQTS; family h/o sudden death at < 40
years; survival from a VT/VF episode in absence of heart disease
5-6 genes have been linked , but yield of genetic screening remains low ( around
20%). Autosomal dominant with high penetrance.
Peak of incidence during first year of life followed by a quiescent phase
encompassing adolescence and another peak at old age.
Cardiac arrest probability by age of 40 years is 40%.
Symptoms: cardiac arrest(40%), syncope(25%), AF (20%)
26. Additional ECG findings that facilitate diagnosis: tall, peaked, symmetrical, and
narrow based T waves, prominent U waves, depression of PQ segment, or a QRS
complex directly followed by a T wave. QTc should be measured at different values
of HR and mostly around 60 bpm.
27. Risk stratification and management in SQTS
Survivors of aborted cardiac arrest, and/or documented
spontaneous sustained VT: ICD recommended (COR I LOE C)
Quinidine or sotalol may be considered in patients who qualify for
ICD but present a contraindication or refuse it (COR IIb LOE C)
Quinidine or sotalol may be considered in asymptomatic SQTS
patients with family h/o SCD (COR IIB LOE C)
Invasive EPS with PVS is not recommended for SCD risk
stratification (COR III LOE C)
28. Catecholaminergic Polymorphic
Ventricular Tachycardia (CPVT)
Definitions and epidemiology
CPVT is diagnosed in presence of structurally normal heart, normal ECG and
exercise or emotion- induced bidirectional or polymorphic VT.(COR I LOE C)
CPVT is diagnosed in patients who are carriers of pathogenic mutation(s) in
genes RyR2 (autosomal dominant) or CASQ2 (autosomal recessive) (COR I LOE
C)
Prevalence 1 in 10000. Presents during first decade.Physical activity or
emotional stress induced syncope and cardiac arrest leading to SCD.
Differences from LQTS are more severe clinical manifestation, reduced
response to beta-blockers, higher penetrance, short runs of AF and supra
ventricular arrhythmias during exercise, typical pattern of reproducible
arrhythmias and their prompt decrease on interruption of exercise.
29.
30. Risk stratification and management in CPVT
Diagnosis in childhood, lack of beta-blocker therapy and persistence of
complex arrhythmias during exercise stress test on a full dose of beta-
blockers are predictors for arrhythmia events.
CPVT patients who experience cardiac arrest, recurrent syncope, or
polymorphic/bidirectional VT despite optimal therapy: ICD in addition to
beta-blockers with or without flecainide is recommended(COR I LOE C)
ICD should be programmed with long delays before shock delivery to
avoid malignant cycle of ICD shocks and death.
PVS has no diagnostic or prognostic value.
31. Beta-blockers are recommended in all patients (COR I
LOE C)
Therapy with beta-blockers for genetically positive family
members even after negative stress test(COR IIa LOE C)
Flecainide should be considered in addition to beta-
blockers in patients with recurrent syncope or
polymorphic/bidirectional VT while on beta-blockers when
there are risk/contraindications for ICD or ICD not
available or rejected by the patient (COR IIa LOE C)
31
32.
33. Flecainide should be considered in addition to beta-blockers
in carriers of ICD to reduce appropriate ICD shocks(COR IIA
LOE C)
LCSD may be considered-recurrent syncope or
polymorphic/bidirectional VT/several appropriate ICD shocks
while on beta-blockers and beta-blockers plus flecainide and
patients who are intolerant/contraindication to beta-blockers.
(COR IIb LOE C)
Life-style changes are recommended like avoidance of
triggers (COR I LOE C)
34. Concluding remarks
SCD major cause of death mainly in young
populations.
Comprehensive genotype-phenotype studies in large
cohort of families are needed for IADs.
Cardiogenetic Team approach including cardiologist,
geneticists, genetic counselors, and even
psychologists.
Judicious use of genetic testing in patients with definite
phenotype.