{
  "id": 16614,
  "label": "pulmonary arterial hypertension",
  "model": {
    "label": "Mondo Disease Ontology (MONDO)",
    "source": 0
  },
  "type_id": 0,
  "reference_id": "MONDO:0015924",
  "properties": {
    "xrefs": [
      "EFO:0001361",
      "GARD:0007501",
      "MEDGEN:425404",
      "MESH:D000081029",
      "MedDRA:10064911",
      "NANDO:1200425",
      "NANDO:2100103",
      "NANDO:2200298",
      "NORD:1634",
      "Orphanet:182090",
      "Orphanet:422",
      "SCTID:11399002",
      "UMLS:C2973725",
      "icd11.foundation:1931148955"
    ],
    "synonyms": [
      "PAH",
      "pulmonary arterial hypertension",
      "PAH with overt features of venous/capillaries involvement",
      "PVOD/PCH",
      "PPH",
      "idiopathic pulmonary hypertension"
    ],
    "categories": [
      {
        "ref": "MONDO:0004995",
        "name": "cardiovascular disorder"
      }
    ],
    "definition": "Pulmonary arterial hypertension (PAH) is a group of diseases characterized by mean pulmonary artery pressure >20 mmHg and elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, have overt features of venous/capillary involvement (pulmonary veno-occlusive disease, PVOD/pulmonary capillary hemangiomatosis, PCH), induced by drug or toxin (drug-or toxin-induced PAH), or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease)."
  },
  "isLeaf": false,
  "isRoot": false,
  "child_count": 5,
  "parents": [
    {
      "id": 6873,
      "label": "pulmonary hypertension",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        6776
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:6432",
          "GARD:0027347",
          "MEDGEN:9376",
          "MESH:D006976",
          "MedDRA:10037400",
          "SCTID:70995007",
          "UMLS:C0020542",
          "icd11.foundation:1496633964"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          }
        ],
        "definition": "Increased pressure within the pulmonary circulation due to lung or heart disorder."
      },
      "child_count": 6,
      "reference_id": "MONDO:0005149"
    }
  ],
  "children": [
    {
      "id": 4158,
      "label": "idiopathic pulmonary arterial hypertension",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        3703,
        16614,
        24405
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "DOID:14557",
          "GARD:0027594",
          "ICD10CM:I27.0",
          "ICD9:416.0",
          "MEDGEN:468368",
          "MedDRA:10065151",
          "Orphanet:275766",
          "SCTID:697898008",
          "UMLS:C3203102",
          "icd11.foundation:265520344"
        ],
        "synonyms": [
          "IPAH",
          "idiopathic pulmonary arterial hypertension",
          "primary pulmonary arterial hypertension",
          "primary pulmonary hypertension"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          }
        ],
        "definition": "A sporadic form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure. IPAH is progressive and potentially fatal and not associated with an underlying condition or family history of PAH. The etiology is unknown."
      },
      "child_count": 0,
      "reference_id": "MONDO:0001999"
    },
    {
      "id": 17502,
      "label": "heritable pulmonary arterial hypertension",
      "isLeaf": false,
      "isRoot": false,
      "parents": [
        5714,
        16614
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "GARD:0011914",
          "MEDGEN:90953",
          "NANDO:2201047",
          "NCIT:C121945",
          "OMIMPS:178600",
          "Orphanet:275777",
          "SCTID:697897003",
          "UMLS:C0340543",
          "icd11.foundation:943760810"
        ],
        "synonyms": [
          "FPAH",
          "HPAH",
          "HpaH",
          "familial pulmonary arterial hypertension",
          "hereditary pulmonary arterial hypertension"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          }
        ],
        "definition": "Heritable pulmonary arterial hypertension (HPAH) is a form of pulmonary arterial hypertension (PAH), occurring due to mutations in PAH predisposing genes or in a familial context. HPAH is characterized by elevated pulmonary arterial resistance leading to right heart failure. HPAH is progressive and potentially fatal."
      },
      "child_count": 14,
      "reference_id": "MONDO:0017148"
    },
    {
      "id": 17503,
      "label": "drug- or toxin-induced pulmonary arterial hypertension",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        16614
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0009192",
          "GARD:0021026",
          "MEDGEN:573792",
          "Orphanet:275786",
          "UMLS:C0340544"
        ],
        "synonyms": [
          "drug- or toxin-induced PAH"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          }
        ],
        "definition": "Drug- or toxin-induced pulmonary arterial hypertension (PAH) is a form of pulmonary arterial hypertension (PAH) secondary to the exposition to drugs. Drug- or toxin-induced PAH is characterized by elevated pulmonary arterial resistance leading to right heart failure. Drug or toxin induced PAH is progressive and potentially fatal."
      },
      "child_count": 0,
      "reference_id": "MONDO:0017149"
    },
    {
      "id": 18568,
      "label": "pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        6815,
        16614
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0009199",
          "GARD:0021801",
          "MEDGEN:785618",
          "NANDO:1200426",
          "NANDO:1200428",
          "Orphanet:431353",
          "UMLS:C3698354"
        ],
        "synonyms": [
          "PVOD and/or PCH"
        ],
        "categories": [
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          },
          {
            "ref": "MONDO:0005087",
            "name": "respiratory system disorder"
          }
        ],
        "definition": "A rare subgroup of pulmonary arterial hypertension (PAH) characterized by obliterative fibrosis of the small pulmonary veins and venules and/or capillary infiltration of the pulmonary interstitium leading to increased pulmonary vascular resistance and right ventricular dysfunction."
      },
      "child_count": 0,
      "reference_id": "MONDO:0018554"
    },
    {
      "id": 19662,
      "label": "Eisenmenger syndrome",
      "isLeaf": true,
      "isRoot": false,
      "parents": [
        4370,
        16614
      ],
      "type_id": 0,
      "properties": {
        "xrefs": [
          "EFO:0009200",
          "GARD:0006323",
          "ICD10CM:I27.83",
          "MEDGEN:4479",
          "MESH:D004541",
          "MedDRA:10058554",
          "NCIT:C84390",
          "NORD:1081",
          "Orphanet:97214",
          "SCTID:445928005",
          "UMLS:C0013743",
          "icd11.foundation:581886860"
        ],
        "synonyms": [
          "Eisenmenger's syndrome"
        ],
        "categories": [
          {
            "ref": "MONDO:0002254",
            "name": "syndromic disease"
          },
          {
            "ref": "MONDO:0004995",
            "name": "cardiovascular disorder"
          }
        ],
        "definition": "Eisenmenger syndrome (ES) is an form of pulmonary arterial hypertension (PAH) associated with unoperated congenital heart disease and is characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH."
      },
      "child_count": 0,
      "reference_id": "MONDO:0019944"
    }
  ],
  "roots": [
    {
      "id": 6873,
      "label": "pulmonary hypertension"
    }
  ]
}