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
    • Channels containing six transmembrane segments and one pore-forming region (6TM-1P)
    • Kv channel α-subunits
    • The a subunit of high conductance Ca 2+ -activated K + channels ( BKCa)
    • Kv channel kinetics
    • Auxiliary subunits
    • Channels with two transmembrane domains and one poe (2 TM-1P)
    • Four transmembrane segments and two pore (K2P) channel
    • The rapidly activating componente (IKr) of the delayed rectifier K + current
    • Slow component of the delayed rectifier or IKs
    • 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)
     • Role of ryanodine and calsequestrin
     • Genetic basis
     • Treatment
     • References