{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:44:11Z","timestamp":1789663451915,"version":"4.0.1"},"reference-count":7,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2023,6,16]],"date-time":"2023-06-16T00:00:00Z","timestamp":1686873600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and objective: Unilateral agenesis of pulmonary arteries (UAPA) is a rare disease, with approximately 400 cases reported to date. UAPA is often associated with congenital heart disease, and the uncomplicated form is isolated UAPA, which accounts for approximately 30% of all cases of UAPA. The incidence of pulmonary hypertension due to UAPA has been reported to range from 19 to 44%. There is no consensus treatment for pulmonary hypertension associated with UAPA. We present the first reported case in which a three-drug combination, comprising of iloprost inhalation, riociguat, and ambrisentan, was administered to a patient with UAPA, and was followed-up for 3 years post-diagnosis. Case presentation: A 68-year-old Japanese woman presented to our hospital with dyspnea and chest discomfort. She underwent chest radiography, blood tests, and echocardiography; however, the cause of the patient\u2019s symptoms could not be identified. During regular follow-up, an echocardiography 21 months after the initial visit revealed elevated right ventricular pressure (peak tricuspid regurgitation velocity: 5.2 m\/s and right ventricular systolic pressure: 120 mmHg) and a diagnosis of pulmonary hypertension was made. Contrast-enhanced computed tomography (CT) of the chest and a pulmonary blood flow scintigram were performed to investigate the cause of pulmonary hypertension, and isolated UAPA was diagnosed. The patient was treated with a three-drug combination of iloprost inhalation, riociguat, and ambrisentan and followed up for 3 years with good therapeutic outcomes. Conclusions: We present a case of pulmonary hypertension caused by isolated UAPA. Although rare, this disease can lead to pulmonary hypertension and should be treated cautiously. While there is no consensus regarding the treatment of this disease, a three-drug combination of iloprost inhalation, riociguat, and oral ambrisentan proved effective.<\/jats:p>","DOI":"10.3390\/medicina59061161","type":"journal-article","created":{"date-parts":[[2023,6,16]],"date-time":"2023-06-16T08:56:01Z","timestamp":1686905761000},"page":"1161","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment"],"prefix":"10.3390","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-0830-575X","authenticated-orcid":false,"given":"Marohito","family":"Nakata","sequence":"first","affiliation":[{"name":"Department of Cardiology, Urasoe General Hospital, Urasoe 901-2132, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoko","family":"Yokota","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Urasoe General Hospital, Urasoe 901-2132, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroki","family":"Uehara","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Urasoe General Hospital, Urasoe 901-2132, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazuiko","family":"Tabata","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Naha City Hospital, Naha 902-8511, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3120-6605","authenticated-orcid":false,"given":"Tsuneaki","family":"Kenzaka","sequence":"additional","affiliation":[{"name":"Division of Community Medicine and Career Development, Graduate School of Medicine, Kobe University,  Kobe 652-0032, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,6,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1007\/s12055-020-01032-w","article-title":"Congenital isolated unilateral agenesis of pulmonary arteries in adults: Case series and review","volume":"37","author":"Jariwala","year":"2021","journal-title":"Indian J. Thorac. Cardiovasc. Surg."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"448","DOI":"10.1016\/j.acvd.2013.05.004","article-title":"Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment","volume":"106","author":"Kruzliak","year":"2013","journal-title":"Arch. Cardiovasc. Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1253\/circj.CJ-66-0158","article-title":"Guidelines for the treatment of pulmonary hypertension (JCS 2017\/JPCPHS 2017)","volume":"83","author":"Fukuda","year":"2019","journal-title":"Circ. J."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"64","DOI":"10.4103\/ejop.ejop_62_19","article-title":"Unilateral absence of pulmonary artery: A rare cause of dyspnea in a 77-year-old woman","volume":"22","author":"Chopra","year":"2020","journal-title":"Eurasian J. Pulmonol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1347","DOI":"10.1157\/13096595","article-title":"Edema de pulm\u00f3n unilateral e hipertensi\u00f3n pulmonar tratada con sildenafilo en la agenesia de la arteria pulmonar [Sildenafil treatment of unilateral pulmonary edema and pulmonary hypertension in pulmonary artery agenesis]","volume":"59","author":"Pujol","year":"2006","journal-title":"Rev. Esp. Cardiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1378\/chest.09-0080","article-title":"A 50-year-old woman with dyspnea, lower extremity edema, and volume loss of the right hemithorax","volume":"136","author":"Shostak","year":"2009","journal-title":"Chest"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"3427","DOI":"10.1002\/ehf2.13459","article-title":"Novel diagnostic and therapeutic approaches to pulmonary hypertension due to the unilateral absence of a pulmonary artery","volume":"8","author":"Nagamatsu","year":"2021","journal-title":"ESC Heart Fail."}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/59\/6\/1161\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T19:56:29Z","timestamp":1760126189000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/59\/6\/1161"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,16]]},"references-count":7,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2023,6]]}},"alternative-id":["medicina59061161"],"URL":"https:\/\/doi.org\/10.3390\/medicina59061161","relation":{},"ISSN":["1648-9144"],"issn-type":[{"value":"1648-9144","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,16]]}}}