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Inherited Structural Heart Diseases Associated with Arrhythmias: Defects in Laterality

  • Baruch S. Ticho
  • Richard Van Praagh
Part of the Developments in Cardiovascular Medicine book series (DICM, volume 231)

Abstract

The clinical presentation of patients with defects in the determination of laterality is extremely varied. Abnormalities can occur in 1) establishment of visceral and atrial situs resulting in situs inversus or heterotaxy, 2) looping of the cardiac tube resulting in ventricular inversion, and 3) positioning of the heart in the chest resulting in dextrocardia. These disorders are usually easily recognized, and can be associated with complex cardiac anomalies. Affected individuals may be completely asymptomatic or may present at any age with a broad range of severity of symptoms. Conduction defects occur most frequently in patients with heterotaxy or with “corrected” transposition of the great arteries. The clinical and conduction system manifestations of these diseases will be presented, followed by a discussion of the molecular and genetic bases for these disorders.

Keywords

Ventricular Septal Defect Great Artery Situs Inversus Complete Heart Block Congenital Heart Block 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 2000

Authors and Affiliations

  • Baruch S. Ticho
    • 1
  • Richard Van Praagh
    • 2
  1. 1.Massachusetts General HospitalBostonUSA
  2. 2.Children’s HospitalBostonUSA

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