{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:49:01Z","timestamp":1753876141682,"version":"3.41.2"},"reference-count":7,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T00:00:00Z","timestamp":1661126400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,9,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Calcium channel blockers (CCB), the first accepted treatment, is effective only in a small number of idiopathic pulmonary arterial hypertension (I-PAH) patients with vasoreactivity [these patients are identified by a positive acute pulmonary vasoreactive test (AVT) response]. While the majority of I-PAH patients is non-vasoreactive and CCB non-responders, modern advanced pulmonary hypertension (PH)-specific therapies, which act on one of the three different mechanistic pathways\u2014endothelin, nitric oxide (NO), and prostacyclin pathways, are effective. Treatment response to advanced PH-specific vasodilators in PAH patients with vasoreactivity is unknown.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>A 30-year-old woman with I-PAH was referred to our centre with worsening symptoms and deteriorating PH. She was being administered oral triple combination of advanced PH-specific treatment including a phosphodiesterase-5 inhibitor, an endothelin receptor antagonist, and a long-acting prostacyclin analogue. The patient showed positive AVT with NO inhalation while on these advanced PH-specific drugs. We added high-dose CCB, which dramatically normalized her pulmonary blood pressure without further symptoms, and she has remained stable for 5 years.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>Our case describes a PAH patient with vasoreactivity, who was resistant to three different types of advanced PH-specific vasodilators but was exclusively sensitive to CCB treatment. Some CCB responders may have a specific CCB-sensitive PAH phenotype refractory to other pulmonary vasodilators. This case highlights the role of identifying CCB responders in this era of use of modern, advanced PH-specific vasodilators. The investigation of the mechanisms underlying CCB sensitivity in PAH is necessary.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytac351","type":"journal-article","created":{"date-parts":[[2022,8,22]],"date-time":"2022-08-22T15:48:22Z","timestamp":1661183302000},"source":"Crossref","is-referenced-by-count":5,"title":["Pulmonary arterial hypertension sensitive to calcium channel blocker, but not advanced pulmonary hypertension treatment: a case report"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3598-0235","authenticated-orcid":false,"given":"Kyoko","family":"Hirakawa","sequence":"first","affiliation":[{"name":"Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre , 6-1, Kishibe-Shimmachi, Suita, Osaka 564-8565 , Japan"},{"name":"Department of Cardiovascular Medicine, Kumamoto University , Kumamoto 860-8556 , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatsuo","family":"Aoki","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre , 6-1, Kishibe-Shimmachi, Suita, Osaka 564-8565 , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Tsuji","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre , 6-1, Kishibe-Shimmachi, Suita, Osaka 564-8565 , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1180-9262","authenticated-orcid":false,"given":"Takeshi","family":"Ogo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre , 6-1, Kishibe-Shimmachi, Suita, Osaka 564-8565 , Japan"},{"name":"Department of Advanced Medicine in Pulmonary Hypertension, National Cerebral and Cardiovascular Centre , Osaka 564-8565 , Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,8,22]]},"reference":[{"key":"2022090612313273500_ytac351-B1","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1056\/NEJM199207093270203","article-title":"Effect of high doses of calcium-channel blockers on survival in patients with primary pulmonary hypertension","volume":"327","author":"Rich","year":"1992","journal-title":"N Engl J Med"},{"key":"2022090612313273500_ytac351-B2","doi-asserted-by":"crossref","first-page":"3105","DOI":"10.1161\/CIRCULATIONAHA.104.488486","article-title":"Long-term response to calcium channel blockers in patients with idiopathic pulmonary arterial hypertension","volume":"111","author":"Sitbon","year":"2005","journal-title":"Circulation"},{"key":"2022090612313273500_ytac351-B3","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.1183\/09031936.00042107","article-title":"A USA-based registry for pulmonary arterial hypertension: 1982\u20132006","volume":"30","author":"Thenappan","year":"2007","journal-title":"Eur Respir J"},{"key":"2022090612313273500_ytac351-B4","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European society of cardiology (ESC) and the European respiratory society (ERS): endorsed by: association for European paediatric and congenital cardiology (AEPC), international society for heart and lung transplantation (ISHLT)","volume":"37","author":"Galie","year":"2016","journal-title":"Eur Heart J"},{"key":"2022090612313273500_ytac351-B5","doi-asserted-by":"crossref","first-page":"H1546","DOI":"10.1152\/ajpheart.00944.2011","article-title":"New mechanisms of pulmonary arterial hypertension: role of Ca2+ signaling","volume":"302","author":"Kuhr","year":"2012","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"2022090612313273500_ytac351-B6","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1016\/j.rmed.2009.11.015","article-title":"Pulmonary vasodilator testing and use of calcium channel blockers in pulmonary arterial hypertension","volume":"104","author":"Tonelli","year":"2010","journal-title":"Respir Med"},{"key":"2022090612313273500_ytac351-B7","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1086\/680355","article-title":"Sildenafil versus nitric oxide for acute vasodilator testing in pulmonary arterial hypertension","volume":"5","author":"Milger","year":"2015","journal-title":"Pulm Circ"}],"container-title":["European Heart Journal - 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