Epidemiology

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Etiology

• Jervell and Lange-Neilsen Syndrome
  • Epidemiology: less common type
  • Inheritance: autosomal recessive
  • Clinical: sensorineural deafness + prolonged QT
  • Prognosis: more malignant clinical course
• Romano-Ward Syndrome
  • Epidemiology: more common type
  • Inheritance: autosomal dominant
  • Clinical: purely cardiac phenotype (prolonged QT only)
• Idiopathic Congenital Long QT Syndrome

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Physiology

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Diagnosis

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Clinical Manifestations

Cardiovascular Manifestations

• Atrial Fibrillation (see Atrial Fibrillation, [[Atrial Fibrillation]]): less common
• Atrioventricular Blocks: high-grade blocks (requiring permanent pacemaker placement) are rare
  • Second Degree Atrioventricular Block-Mobitz Type I (Wenkebach) (see Second Degree Atrioventricular Block-Mobitz Type I, [[Second Degree Atrioventricular Block-Mobitz Type I]])
  • Second Degree Atrioventricular Block-Mobitz Type II (see Second Degree Atrioventricular Block-Mobitz Type II, [[Second Degree Atrioventricular Block-Mobitz Type II]])
  • Third Degree Atrioventricular Block/Complete Heart Block (see Third Degree Atrioventricular Block, [[Third Degree Atrioventricular Block]]): few reported cases
• Sinus Bradycardia (see Sinus Bradycardia, [[Sinus Bradycardia]])
• Torsade/Polymorphic Ventricular Tachycardia (VT) (see Torsade, [[Torsade]]): common presentation in congenital long QT syndrome

Other Manifestations

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Treatment

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References

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