{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T20:39:19Z","timestamp":1786653559629,"version":"build-2736575974"},"reference-count":7,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:sec>\n                    <jats:title>ABSTRACT<\/jats:title>\n                    <jats:p>Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/apc.apc_256_20","type":"journal-article","created":{"date-parts":[[2023,2,23]],"date-time":"2023-02-23T00:50:18Z","timestamp":1677113418000},"page":"73-76","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Severe pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia and multiple pulmonary and hepatic arteriovenous malformations"],"prefix":"10.4103","volume":"15","author":[{"given":"Sunitha","family":"Aramalla","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srinivas","family":"Bhyravavajhala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bharathi","family":"Vanaparty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramakrishna","family":"Narayanan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sreekanth","family":"Yerram","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-15-20240901","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1378\/chest.15-0535","article-title":"Pulmonary hypertension in hereditary hemorrhagic telangiectasia","volume":"149","author":"Lyle","year":"2016","journal-title":"Chest"},{"key":"R2-15-20240901","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1136\/jmg.2009.069013","article-title":"International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia","volume":"48","author":"Faughnan","year":"2011","journal-title":"J Med Genet"},{"key":"R3-15-20240901","doi-asserted-by":"crossref","first-page":"145","DOI":"10.21693\/1933-088X-14.3.145","article-title":"Distinct forms of pulmonary hypertension complicate hereditary hemorrhagic telangiectasia","volume":"14","author":"Battaile","year":"2015","journal-title":"Adv Pulm Hypertens"},{"key":"R4-15-20240901","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1159\/000458447","article-title":"Pulmonary hypertension in a large cohort with hereditary hemorrhagic telangiectasia","volume":"94","author":"Vorselaars","year":"2017","journal-title":"Respiration"},{"key":"R5-15-20240901","doi-asserted-by":"crossref","first-page":"e0184227","DOI":"10.1371\/journal.pone.0184227","article-title":"Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability","volume":"12","author":"Revuz","year":"2017","journal-title":"PLoS One"},{"key":"R6-15-20240901","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1186\/s13023-019-1107-4","article-title":"Rendu-Osler-Weber disease: A gastroenterologist's perspective","volume":"14","author":"Tortora","year":"2019","journal-title":"Orphanet J Rare Dis"},{"key":"R7-15-20240901","doi-asserted-by":"crossref","first-page":"2045894018759918","DOI":"10.1177\/2045894018759918","article-title":"The clinical characteristics and long-term prognosis of pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia","volume":"8","author":"Li","year":"2018","journal-title":"Pulm Circ"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_256_20","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:39:09Z","timestamp":1786649949000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_256_20"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1]]},"references-count":7,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_256_20","relation":{},"ISSN":["0974-2069"],"issn-type":[{"value":"0974-2069","type":"print"}],"subject":[],"published":{"date-parts":[[2022,1]]}}}