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Familial idiopathic dilatation of the right atrium

Disease definition

A rare congenital heart malformation of unknown etiology that is characterized by an extremely dilated right atrium, and that is usually asymptomatic and fortuitously discovered by echocardiography or chest radiography, and can be sometimes associated with other anomalies such as atrial arrhythmias (e.g. atrial flutter, atrial fibrillation, supraventricular tachycardia), severe tricuspid regurgitation, or atrial thrombus that could lead to potentially life-threatening thromboembolic complications.

ORPHA:1677

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: Unknown 
  • Age of onset: All ages
  • ICD-10: Q20.8
  • ICD-11: LA8F
  • OMIM: -
  • UMLS: C4274283
  • MeSH: -
  • GARD: -
  • MedDRA: -
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