CONCEPTS

• Sudden cardiac death
• Genetic basis of sudden cardiac death

• Ion channels
• Ion channel function
• Ion channel structure
• Types of ion channels
• Conformational states of the ion channels
• Channelosome
• References

• Correlation between the cardiac action potential in the surface electrocardiogram
• Pacemaker activity

CARDIAC ION CHANNELS

• Structure
• Auxiliary subunits
• Interacting proteins
• Channel gating

• Channel structure
  • α1C subunit
  • ß subunits
  • α₂δ subunit
• Calcium channel gating
• Channelopaties
• References

• 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
• Channels with two transmembrane domains and one poe (2 TM-1P)
• Structure
• Other Kir channels
• Four transmembrane segments and two pore (K2P) channel
• References

• 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