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Congenital total pulmonary venous return anomaly

Disease definition

A form of congenital pulmonary venous return where all of the pulmonary veins drain into the right atrium or one of its tributaries, instead of the left atrium, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure.

ORPHA:99125

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: Neonatal
  • ICD-10: Q26.2
  • ICD-11: LA86.20
  • OMIM: 106700
  • UMLS: C5681626
  • MeSH: -
  • GARD: -
  • MedDRA: -
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