{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T18:52:42Z","timestamp":1769799162002,"version":"3.49.0"},"reference-count":21,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T00:00:00Z","timestamp":1769731200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Fiscal Year 2025 President\u2019s Discretionary Funds"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs), a life-threatening congenital heart defect (CHD), is frequently associated with abnormal pulmonary blood flow and vascular remodeling, causing hypoxia and heart failure. Segmental pulmonary hypertension (PH), a distinct PH type, may exist in some patients. Pulmonary vasodilators have been considered for treatment; however, evidence of their efficacy and safety remains lacking. Methods: A systematic review was conducted using PubMed, MEDLINE, The Cochrane Library, and Ichushi Web, encompassing studies from inception to May 2023. Inclusion criteria focused on patients with PA\/VSD\/MAPCAs treated with PH medications. Results: Of 86 studies screened, 6 met the inclusion criteria, including 1 cohort study and 5 case reports, comprising 22 patients. The most frequently administered medications were sildenafil (14 cases) and bosentan (12 cases), with 16 patients receiving monotherapy. Clinical improvements were observed in pulmonary vascular resistance (8\/8 patients), oxygen saturation (8\/19 patients), and symptoms (19\/21 patients). Adverse effects were noted in five patients, including treatment discontinuation in two. Conclusions: PH medications may benefit some patients with PA\/VSD\/MAPCAs; however, the extremely limited sample size (n = 22) and substantial heterogeneity in anatomy, age, surgical status, and treatment regimens severely limit interpretability and clinical applicability. Considering the potential benefits and risks associated with these medications, their use should be considered cautiously and restricted to specialized centers with expertise in CHD and PH management.<\/jats:p>","DOI":"10.3390\/jcm15031087","type":"journal-article","created":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T08:19:38Z","timestamp":1769761178000},"page":"1087","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Systematic Review of the Application of Pulmonary Hypertension Treatments in Ventricular Septal Defect, Pulmonary Atresia, and Major Aortopulmonary Collateral Arteries"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4224-9053","authenticated-orcid":false,"given":"Keiichi","family":"Hirono","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Toyama University Hospital, Toyama 930-0194, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-9249-6345","authenticated-orcid":false,"given":"Keiko","family":"Uchida","sequence":"additional","affiliation":[{"name":"Department of Physiology, Tokyo Medical University, Tokyo 160-8402, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taku","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, Institute of Science Tokyo, Tokyo 113-8510, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7284-8914","authenticated-orcid":false,"given":"Hidekazu","family":"Ishida","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Osaka University Graduate School of Medicine, Suita 565-0871, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5875-4628","authenticated-orcid":false,"given":"Shinichi","family":"Takatsuki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Toho University, Omori Medical Center, Tokyo 143-8541, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Fukushima","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Tokyo Dental College Ichikawa General Hospital, Ichikawa 272-8513, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kei","family":"Inai","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology and Adult Congenital Cardiology, Tokyo Women\u2019s Medical University, Tokyo 272-8513, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susumu","family":"Hosokawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Japanese Red Cross Musashino Hospital, Musashino 180-8610, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reina","family":"Ishizaki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8069-5751","authenticated-orcid":false,"given":"Hirofumi","family":"Sawada","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medicine, Mie University, Tsu 514-8507, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naofumi F.","family":"Sumitomo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine, Keio University, Tokyo 160-8582, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1277-6119","authenticated-orcid":false,"given":"Ayako","family":"Chida-Nagai","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hokkaido University Hospital, Sapporo 060-8648, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8139-685X","authenticated-orcid":false,"given":"Yuichi","family":"Ishikawa","sequence":"additional","affiliation":[{"name":"Minatomirai Medical Corporation, Chigasaki Kanazawa Internal Medical Clinic, Chigasaki 253-0052, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirohiko","family":"Motoki","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Shinshu University, Matsumoto 390-8621, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsushi","family":"Yao","sequence":"additional","affiliation":[{"name":"Health Service Promotion, The University of Tokyo, Tokyo 113-8654, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigetoyo","family":"Kogaki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Osaka General Medical Center, Osaka 558-8558, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Yamagishi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Tokyo Metropolitan Children\u2019s Medical Center, Tokyo 183-8561, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shozaburo","family":"Doi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, Institute of Science Tokyo, Tokyo 113-8510, 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