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Primary Arrhythmogenic Cardiomyopathies. Channelopathies
CHANNELOPATHIES
CONCEPTS
Sudden cardiac death
Sudden cardiac ceath
Genetic basis of sudden cardiac death
Ion Channels
Ion channels
Ion channel function
Ion channel structure
Types of ion channels
Conformational states of the ion channels
Channelosome
References
The cardiac action potential
Correlation between the cardiac action potential in the surface electrocardiogram
Pacemaker activity
CARDIAC ION CHANNELS
The voltage-gated sodium channel
Structure
Auxiliary subunits
Channel gating
Cardiac Na + channelopathies
Channelosome
References
Cardiac L-type calcium channels
Channel structure
α1C subunit
ß subunits
α
2
δ subunit
Calcium channel gating
Channelopaties
References
Cardiac potassium channels
Classification
Channels containing six transmembrane segments and one pore-forming region (6TM-1P)
Kv channel α-subunits
The
α
subunit of high conductance Ca
2+
-activated K
+
channels (BKCa)
Kv channel kinetics
Auxiliary subunits
The rapidly activating componente (I
Kr
) of the delayed rectifier K
+
current
HERG channelopathies
Slow component of the delayed rectifier (I
Ks
)
Channelopathies
Channels with two transmembrane domains and one poe (2 TM-1P)
Structure
Other Kir channels
Four transmembrane segments and two pore (K2P) channel
References
Transient receptor potential (TRP) channels
TRPM4 channels
References
PRIMARY ARRHYTHMOGENIC CARDIOMYOPATHIES
. CHANNELOPATHIES
Inherited arrhythmogenic diseases
Channelopathies: clinical relevance
Long QT Syndrome
Prevalence
Electrofisiological basis
Genetic basis
Clinical presentation
Diagnosis
Electrocardiographic findings
Other diagnostic tests
Prognosis
Treatment
Ohter pharmacological treatments
References
Short QT Syndrome (SQTS)
Clinical presentation
Diagnosis
Genetic Basis
Treatment
References
Brugada Syndrome (BrS)
Prevalence
Genetic basis
Electrophysiological basis
Clinical manifestations
Diagnosis
Risk stratification
Treatment
References
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)
Diagnosis
Role of ryanodine and calsequestrin
Genetic bases
Treatment
References
Early Repolarization Syndrome (ERS)
Correlation between the J point ERS
Genetic basis
Clasification of ERS
Pathophysyological basis
Diagnosis
Treatment
References
Atrial Fibrillation
Genetic basis
Treatment
References
Progressive Cardiac Conduction Disease (PCCD)
Genetic basis
Treatment
References
Sick Sinus Syndrome
Genetic basis
Treatment
References