CHANNELOPATHIES

• CONCEPTS
  • Sudden cardiac death
    1. Sudden cardiac ceath
    2. Genetic basis of sudden cardiac death
  • Ion Channels
    1. Ion channels
    2. Ion channel function
    3. Ion channel structure
    4. Types of ion channels
    5. Conformational states of the ion channels
    6. Channelosome
    7. References
  • The cardiac action potential
    1. Correlation between the cardiac action potential in the surface electrocardiogram
    2. 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
      1. α1C subunit
      2. ß subunits
      3. α₂δ subunit
    • Calcium channel gating
    • Channelopaties
    • References
  • Cardiac potassium channels

    • Classification

    1. Channels containing six transmembrane segments and one pore-forming region (6TM-1P)
    • Kv channel α-subunits
    • The α subunit of high conductance Ca²⁺ -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
    2. Channels with two transmembrane domains and one poe (2 TM-1P)
    • Structure
    • Other Kir channels
    • Four transmembrane segments and two pore (K2P) channel
    3. References
  • Transient receptor potential (TRP) channels
  • TRPM4 channels
  • References
• PRIMARY ARRHYTHMOGENIC CARDIOMYOPATHIES . CHANNELOPATHIES
  • Inherited arrhythmogenic diseases
  • Channelopathies: clinical relevance
  1. Long QT Syndrome
     • Prevalence
     • Electrofisiological basis
     • Genetic basis
     • Clinical presentation
     • Diagnosis
     • Electrocardiographic findings
     • Other diagnostic tests
     • Prognosis
     • Treatment
     • Ohter pharmacological treatments
     • References
  2. Short QT Syndrome (SQTS)
  • Clinical presentation
  • Diagnosis
  • Genetic Basis
  • Treatment
  • References
  3. Brugada Syndrome (BrS)
  • Prevalence
  • Genetic basis
  • Electrophysiological basis
  • Clinical manifestations
  • Diagnosis
  • Risk stratification
  • Treatment
  • References
  4. Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)
     • Diagnosis
     • Role of ryanodine and calsequestrin
     • Genetic bases
     • Treatment
     • References
  5. Early Repolarization Syndrome (ERS)
     • Correlation between the J point ERS
     • Genetic basis
     • Clasification of ERS
     • Pathophysyological basis
     • Diagnosis
     • Treatment
     • References
  6. Atrial Fibrillation
     • Genetic basis
     • Treatment
     • References
  7. Progressive Cardiac Conduction Disease (PCCD)
     • Genetic basis
     • Treatment
     • References
  8. Sick Sinus Syndrome
     • Genetic basis
     • Treatment
     • References