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HUMAN PHENOTYPE - ANNOTATIONS

The Human Phenotype Ontology (HPO) is downloaded weekly from http://compbio.charite.de/hudson/job/hpo/lastStableBuild/artifact/ontology/release/hp.obo. The file downloaded is considered the "last stable build" available for the ontology. For more about the HPO, view their website at http://www.human-phenotype-ontology.org/.
Term:Double outlet right ventricle
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Accession:HP:0001719 term browser browse the term
Definition:Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle.
Comment:During the development of the heart, the outflow tract initially connects exclusively with the primitive right ventricle and must undergo extensive remodeling to divide into a separate pulmonary artery and aorta; subsequently, there is continued remodeling to establish direct continuity from the left ventricle to the aorta. DORV encompasses a wide spectrum of anatomic arrangements and pathophysiologic disturbances. At one end of the spectrum, it mimics tetralogy of Fallot in the presence of pulmonary stenosis, or a large ventricular septal defect (VSD) in the absence of such stenosis. At the other end of the spectrum, it behaves like transposition of the great arteries with a VSD.
Synonyms:exact_synonym: DORV
 xref: Fyler:600;   Fyler:606;   SNOMEDCT_US:7484005;   UMLS:C0013069


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Term paths to the root
Path 1
Term Annotations click to browse term
  Human phenotype 0
    Phenotypic abnormality 0
      Abnormality of the cardiovascular system 0
        Abnormal cardiovascular system morphology 0
          Abnormal heart morphology 0
            Congenital malformation of the right heart 0
              Double outlet right ventricle 0
                Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis 0
                Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis 0
                Double outlet right ventricle with non-committed ventricular septal defect and pulmonary stenosis 0
                Double outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis 0
                Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis 0
                Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis 0
                Double outlet right ventricle with subpulmonary ventricular septal defect and pulmonary stenosis 0
                Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis 0
Path 2
Term Annotations click to browse term
  Human phenotype 0
    Phenotypic abnormality 0
      Abnormality of the cardiovascular system 0
        Abnormality of the vasculature 0
          Abnormal vascular morphology 0
            Abnormal blood vessel morphology 0
              Abnormal morphology of the great vessels 0
                Congenital malformation of the great arteries 0
                  Conotruncal defect 0
                    Double outlet right ventricle 0
                      Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis 0
                      Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis 0
                      Double outlet right ventricle with non-committed ventricular septal defect and pulmonary stenosis 0
                      Double outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis 0
                      Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis 0
                      Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis 0
                      Double outlet right ventricle with subpulmonary ventricular septal defect and pulmonary stenosis 0
                      Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis 0
paths to the root