{"Name":"Idiopathic pulmonary artery dilatation","DiseaseID__c":"GARD:0006757","id":6757,"encodedName":"idiopathic-pulmonary-artery-dilatation","IsDeleted":false,"Disease_Name_Full__c":"Idiopathic pulmonary artery dilatation","Xref_IDs__c":"C1384816; MEDGEN:1842555; MONDO:0015664; ORPHA:1676","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0015664","Disease_Description__c":"Idiopathic pulmonary artery dilatation is a rare developmental defect during embryogenesis characterized by the dilatation of the main pulmonary artery, with or without dilatation of the right and left pulmonary artery branches, and not attributed to any other cardiac, pulmonary and/or arterial wall disease. It may present with exertional dyspnea, fatigue, cough, hemoptysis, palpitation and chest pain, but may also be asymptomatic. In serious cases, trachea constriction due to postural changes may lead to attacks of cyanosis with severe dyspnea. Sudden cardiac death has been reported in some cases.","GARD_Name__c":"Idiopathic pulmonary artery dilatation","GARD_Synonym__c":null,"Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","Curated_Disease_Description__c":"Idiopathic pulmonary artery dilatation is a rare condition that happens during embryo development. It is characterized by the widening of the main pulmonary artery. It is not caused by any other heart, lung, or artery disease. Some people may experience symptoms such as shortness of breath, fatigue, cough, coughing up blood, palpitations, and chest pain. Others may not have any symptoms at all. In severe cases, the trachea may become narrow due to changes in posture. This can lead to attacks of bluish skin and severe shortness of breath. In some cases, sudden cardiac death has been reported.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":"ORPHA:1676","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0015664","ORPHANET_ID__c":"ORPHA:1676","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Dilatación idiopática de la arteria pulmonar","Spanish_Description_Source__c":"ORPHA:1676","Spanish_Description__c":"La dilatación idiopática de la arteria pulmonar es un defecto del desarrollo durante la embriogénesis poco frecuente que se caracteriza por la dilatación de la arteria pulmonar principal, con o sin dilatación de las ramas derecha e izquierda, y no atribuida a ninguna otra enfermedad cardíaca, pulmonar y/o de la pared arterial. Puede presentarse con disnea de esfuerzo, fatiga, tos, hemoptisis, palpitaciones y dolor en el pecho, pero también puede ser asintomática. En casos graves, la constricción de la tráquea debido a cambios posturales puede provocar ataques de cianosis con grave disnea. En algunos casos, se ha informado de muerte súbita cardíaca.","Spanish_Disease_Name__c":"dilatación idiopática de la arteria pulmonar","Spanish_GARD_Synonym__c":null,"Category_Linearization__c":"ORPHA:93890","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Idiopathic pulmonary artery dilatation is a rare condition that happens during embryo development. It is characterized by the widening of the main pulmonary artery. It is not caused by any other heart, lung, or artery disease. Some people may experience symptoms such as shortness of breath, fatigue, cough, coughing up blood, palpitations, and chest pain. Others may not have any symptoms at all. In severe cases, the trachea may become narrow due to changes in posture. This can lead to attacks of bluish skin and severe shortness of breath. In some cases, sudden cardiac death has been reported.","Curated_Disease_Description_Source__c":"PlainLanguagePilotV1-Sep23","Name":"Idiopathic pulmonary artery dilatation","estimateUsa":""}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cardiology","Tag_Category__c":"Specialist"},{"Tag_Name__c":"Congenital Abnormality","Tag_Category__c":"Disease Category","category_description":"Birth defects are structural changes present at birth that can affect almost any part of the body, including how the body looks, works, or both.","curated_tag_name":"Birth defects"},{"Tag_Name__c":"Congenital Heart Disease","Tag_Category__c":"Specialist","curated_tag_name":"Congenital heart disease"},{"Tag_Name__c":"Vascular Medicine","Tag_Category__c":"Specialist","curated_tag_name":"Vascular diseases"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.orpha.net/en/disease/detail/1676","Source__c":"C1384816; MONDO:0015664; ORPHA:1676","Xref__c":"ORPHA:1676"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=1842555","Source__c":"C1384816","Xref__c":"MEDGEN:1842555"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1384816","Source__c":"C1384816","Xref__c":"C1384816"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0015664","Source__c":"GARD:0006757","Xref__c":"MONDO:0015664"}],"tags":{"Specialist":["Cardiology","Congenital Heart Disease","Vascular Medicine"],"Disease Category":["Congenital Abnormality"]},"synonyms":[""]}