{"id":"https://openalex.org/W3217540239","doi":"https://doi.org/10.1161/circ.144.suppl_1.10367","title":"Abstract 10367: An Unusual Vasodilator Response: Recognizing a Rare Subtype of Pulmonary Arterial Hypertension","display_name":"Abstract 10367: An Unusual Vasodilator Response: Recognizing a Rare Subtype of Pulmonary Arterial Hypertension","publication_year":2021,"publication_date":"2021-11-16","ids":{"openalex":"https://openalex.org/W3217540239","doi":"https://doi.org/10.1161/circ.144.suppl_1.10367","mag":"3217540239"},"language":"en","primary_location":{"id":"doi:10.1161/circ.144.suppl_1.10367","doi":"https://doi.org/10.1161/circ.144.suppl_1.10367","is_oa":false,"landing_page_url":"https://doi.org/10.1161/circ.144.suppl_1.10367","pdf_url":null,"source":{"id":"https://openalex.org/S116251202","display_name":"Circulation","issn_l":"0009-7322","issn":["0009-7322","1524-4539"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Circulation","raw_type":"journal-article"},"volume":null,"type":"conference-abstract","is_traditional":false,"indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5065611604","display_name":"Patrick M Hyland","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1316535847","display_name":"Beth Israel Deaconess Medical Center","ror":"https://ror.org/04drvxt59","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1316535847"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Patrick M Hyland","raw_affiliation_strings":["Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","Patrick M Hyland Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]},{"raw_affiliation_string":"Patrick M Hyland Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5052549882","display_name":"Jamie Elizabeth Diamond","orcid":"https://orcid.org/0000-0002-4104-9965","observed_orcids":["https://orcid.org/0000-0002-4104-9965"]},"institutions":[{"id":"https://openalex.org/I1316535847","display_name":"Beth Israel Deaconess Medical Center","ror":"https://ror.org/04drvxt59","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1316535847"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jamie Diamond","raw_affiliation_strings":["Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","Jamie Diamond Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]},{"raw_affiliation_string":"Jamie Diamond Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5004303669","display_name":"Miranda Theodore","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210087915","display_name":"Massachusetts General Hospital","ror":"https://ror.org/002pd6e78","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210087915","https://openalex.org/I48633490"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Miranda Theodore","raw_affiliation_strings":["Div of Pulmonary and Critical Care Medicine, Massachusetts General Hosp, Boston, MA","Miranda Theodore Div of Pulmonary and Critical Care Medicine, Massachusetts General Hosp, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Div of Pulmonary and Critical Care Medicine, Massachusetts General Hosp, Boston, MA","institution_ids":["https://openalex.org/I4210087915"]},{"raw_affiliation_string":"Miranda Theodore Div of Pulmonary and Critical Care Medicine, Massachusetts General Hosp, Boston, MA","institution_ids":["https://openalex.org/I4210087915"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5022614542","display_name":"Arthur Reshad Garan","orcid":"https://orcid.org/0000-0001-7510-075X","observed_orcids":["https://orcid.org/0000-0001-7510-075X"]},"institutions":[{"id":"https://openalex.org/I1316535847","display_name":"Beth Israel Deaconess Medical Center","ror":"https://ror.org/04drvxt59","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1316535847"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Arthur Reshad Garan","raw_affiliation_strings":["Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","Arthur Reshad Garan Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]},{"raw_affiliation_string":"Arthur Reshad Garan Div of Cardiovascular Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5020806678","display_name":"Noah C. Schoenberg","orcid":"https://orcid.org/0000-0003-3165-8529","observed_orcids":["https://orcid.org/0000-0003-3165-8529"]},"institutions":[{"id":"https://openalex.org/I1316535847","display_name":"Beth Israel Deaconess Medical Center","ror":"https://ror.org/04drvxt59","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1316535847"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Noah Schoenberg","raw_affiliation_strings":["Div of Pulmonary, Critical Care & Sleep Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","Noah Schoenberg Div of Pulmonary, Critical Care & Sleep Medicine, Beth Israel Deaconess Med Ctr, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Div of Pulmonary, Critical Care & Sleep Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]},{"raw_affiliation_string":"Noah Schoenberg Div of Pulmonary, Critical Care & Sleep Medicine, Beth Israel Deaconess Med Ctr, Boston, MA","institution_ids":["https://openalex.org/I1316535847"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":"144","issue":"Suppl_1","first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10728","display_name":"Pulmonary Hypertension Research and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12199","display_name":"Vascular Anomalies and Treatments","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11189","display_name":"Transplantation: Methods and Outcomes","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/pulmonary-veno-occlusive-disease","display_name":"pulmonary veno-occlusive disease","score":0.996999979019165},{"id":"https://openalex.org/keywords/pulmonary-arterial-hypertension","display_name":"pulmonary arterial hypertension","score":0.9869999885559082},{"id":"https://openalex.org/keywords/vasodilator-response","display_name":"vasodilator response","score":0.8759999871253967},{"id":"https://openalex.org/keywords/epoprostenol","display_name":"epoprostenol","score":0.9520000219345093},{"id":"https://openalex.org/keywords/pulmonary-edema","display_name":"pulmonary edema","score":0.9089999794960022},{"id":"https://openalex.org/keywords/right-heart-catheterization","display_name":"right heart catheterization","score":0.7699999809265137},{"id":"https://openalex.org/keywords/lung-transplantation","display_name":"lung transplantation","score":0.8920000195503235},{"id":"https://openalex.org/keywords/pulmonary-venules","display_name":"pulmonary venules","score":0.5789999961853027},{"id":"https://openalex.org/keywords/right-ventricular-dysfunction","display_name":"right ventricular dysfunction","score":0.5189999938011169},{"id":"https://openalex.org/keywords/ground-glass-opacities","display_name":"ground-glass opacities","score":0.7149999737739563}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9608945846557617},{"id":"https://openalex.org/C2911202535","wikidata":"https://www.wikidata.org/wiki/Q18345704","display_name":"Pulmonary Veno-Occlusive Disease","level":3,"score":0.7250482439994812},{"id":"https://openalex.org/C164705383","wikidata":"https://www.wikidata.org/wiki/Q10379","display_name":"Cardiology","level":1,"score":0.7017268538475037},{"id":"https://openalex.org/C2780930700","wikidata":"https://www.wikidata.org/wiki/Q1128595","display_name":"Pulmonary hypertension","level":2,"score":0.6530141234397888},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.5984806418418884},{"id":"https://openalex.org/C2781448352","wikidata":"https://www.wikidata.org/wiki/Q1637912","display_name":"Lung transplantation","level":3,"score":0.5380430221557617},{"id":"https://openalex.org/C146556889","wikidata":"https://www.wikidata.org/wiki/Q1414127","display_name":"Pulmonary wedge pressure","level":3,"score":0.43546798825263977},{"id":"https://openalex.org/C2780940725","wikidata":"https://www.wikidata.org/wiki/Q206280","display_name":"Pulmonary artery","level":2,"score":0.42797237634658813},{"id":"https://openalex.org/C2777714996","wikidata":"https://www.wikidata.org/wiki/Q7886","display_name":"Lung","level":2,"score":0.4218176603317261}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1161/circ.144.suppl_1.10367","doi":"https://doi.org/10.1161/circ.144.suppl_1.10367","is_oa":false,"landing_page_url":"https://doi.org/10.1161/circ.144.suppl_1.10367","pdf_url":null,"source":{"id":"https://openalex.org/S116251202","display_name":"Circulation","issn_l":"0009-7322","issn":["0009-7322","1524-4539"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310315671","host_organization_name":"Lippincott Williams & Wilkins","host_organization_lineage":["https://openalex.org/P4310315671","https://openalex.org/P4310318547"],"host_organization_lineage_names":["Lippincott Williams & Wilkins","Wolters Kluwer"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Circulation","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.98580002784729}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W7161546934","https://openalex.org/W7212977416","https://openalex.org/W4410275959","https://openalex.org/W7161592070","https://openalex.org/W7161551130","https://openalex.org/W2894851449","https://openalex.org/W1993426899","https://openalex.org/W1986283646","https://openalex.org/W4396968143","https://openalex.org/W7161582681"],"abstract_inverted_index":{"Introduction:":[0],"Pulmonary":[1],"veno-occlusive":[2],"disease":[3],"(PVOD)":[4],"is":[5,29,240,262,271,284],"a":[6,77,167,172,263],"rare":[7],"subtype":[8],"of":[9,18,31,48,143,158,175,198,232],"pulmonary":[10,20,33,40,99,206,214,234,244,252],"arterial":[11],"hypertension":[12],"(PAH)":[13],"caused":[14],"by":[15],"intimal":[16],"fibrosis":[17],"the":[19,26,39,92,120,192,225,233,246,274,285],"venules":[21],"and":[22,58,64,81,88,107,132,161,182,204],"septal":[23,89],"veins.":[24],"While":[25],"hemodynamic":[27],"profile":[28],"suggestive":[30],"pre-capillary":[32],"hypertension,":[34,245],"vascular":[35,162],"obstruction":[36],"distal":[37],"to":[38,166,171,218,224,229,256],"capillary":[41,215],"beds":[42],"distinguishes":[43],"PVOD":[44,211],"from":[45],"other":[46],"forms":[47,197],"PAH.":[49],"Case:":[50],"A":[51,95],"29-year-old":[52],"woman":[53],"presented":[54],"with":[55,84,119,137,151,259],"progressive":[56],"dyspnea":[57],"fatigue.":[59],"She":[60,114,177],"was":[61,70,178],"tachycardic,":[62],"hypotensive,":[63],"volume":[65],"overloaded":[66],"on":[67],"exam.":[68],"ECG":[69],"notable":[71],"for":[72,112,181,277,280],"right-axis":[73],"deviation.":[74],"TTE":[75],"showed":[76,102],"dilated,":[78],"hypokinetic":[79],"RV":[80],"small":[82],"LV":[83],"mild":[85],"global":[86],"hypokinesis":[87],"flattening":[90],"throughout":[91],"cardiac":[93],"cycle.":[94],"CTA":[96],"chest":[97,154],"excluded":[98],"embolism":[100],"but":[101],"diffuse":[103],"centrilobular":[104],"ground-glass":[105,159],"opacities":[106,160],"lymphadenopathy":[108],"which":[109,281],"raised":[110],"suspicion":[111],"PVOD.":[113,176,268],"underwent":[115,183],"right":[116],"heart":[117],"catheterization":[118],"following":[121],"measurements":[122],"(mmHg):":[123],"RA":[124],"14,":[125],"PA":[126],"99/56":[127],"(mean":[128],"71),":[129],"PCWP":[130,261],"12,":[131],"CI":[133],"1.1":[134],"L/min/m":[135],"2":[136],"PVR":[138],"28.1":[139],"WU.":[140],"Following":[141],"initiation":[142],"an":[144],"epoprostenol":[145],"infusion,":[146],"she":[147],"became":[148],"increasingly":[149],"hypoxemic":[150],"repeat":[152],"CT":[153],"demonstrating":[155],"dramatic":[156],"worsening":[157,251],"congestion.":[163],"This":[164],"response":[165,255],"vasodilator":[168,257],"challenge":[169,258],"led":[170],"clinical":[173],"diagnosis":[174,270],"urgently":[179],"listed":[180],"bilateral":[184],"lung":[185,188,238,282],"transplantation.":[186],"Explanted":[187],"tissue":[189],"subsequently":[190],"confirmed":[191],"diagnosis.":[193],"Conclusion:":[194],"In":[195],"most":[196],"PAH,":[199],"vasodilators":[200],"mitigate":[201],"arteriolar":[202,220],"narrowing":[203,231],"improve":[205],"blood":[207,221],"flow,":[208],"while":[209],"in":[210,242,254],"they":[212],"exacerbate":[213],"congestion":[216],"due":[217,228],"increased":[219],"flow":[222],"relative":[223],"venous":[226],"outflow":[227],"fixed":[230],"venule":[235],"network.":[236],"Because":[237],"biopsy":[239],"contraindicated":[241],"severe":[243],"clinician":[247],"must":[248],"recognize":[249],"that":[250],"edema":[253],"normal":[260],"critical":[264],"clue":[265],"pointing":[266],"towards":[267],"Timely":[269],"essential":[272],"given":[273],"poor":[275],"prognosis":[276],"this":[278],"condition":[279],"transplantation":[283],"definitive":[286],"treatment.":[287]},"counts_by_year":[],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2021-12-06T00:00:00"}