{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T14:02:39Z","timestamp":1773928959535,"version":"3.50.1"},"reference-count":14,"publisher":"Pensoft Publishers","issue":"2","license":[{"start":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T00:00:00Z","timestamp":1773878400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FM"],"abstract":"<jats:p>We report the case of a boy diagnosed at age 9 with a large aortopulmonary septal defect and Eisenmenger syndrome, highlighting the challenges of late diagnosis and management of fixed pulmonary hypertension. Over the course of six years (2018\u20132024), the patient underwent several follow-ups, including invasive hemodynamic assessments and echocardiography, revealing progressive pulmonary vascular disease. Despite the administration of medical therapy with sildenafil, bosentan, and antiplatelets, the patient\u2019s condition remained unfavorable due to fixed pulmonary vascular obstructive disease. This case underscores the importance of early diagnosis and the complexities of managing advanced pulmonary hypertension in congenital heart defects.<\/jats:p>","DOI":"10.3897\/folmed.68.e149000","type":"journal-article","created":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T10:48:57Z","timestamp":1773917337000},"source":"Crossref","is-referenced-by-count":0,"title":["Long-term management of a child with aortopulmonary septal defect and Eisenmenger syndrome: a 6-year follow-up case study"],"prefix":"10.3897","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-6762-3494","authenticated-orcid":true,"given":"Daniela","family":"Milanova-Ilieva","sequence":"first","affiliation":[{"name":"Department of Pediatrics, St George University Hospital, Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5395-9125","authenticated-orcid":true,"given":"Kostadin","family":"Ketev","sequence":"additional","affiliation":[{"name":"Medical Simulation Training Center, Medical University of Plovdiv, Plovdiv, Bulgaria"},{"name":"Department of Pediatrics and Medical Genetics, St George University Hospital, Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8371-9907","authenticated-orcid":true,"given":"Ivanka","family":"Karavelikova","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, St George University Hospital, Plovdiv, Bulgaria"},{"name":"Department of Pediatrics, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8692-8090","authenticated-orcid":true,"given":"Lyubov","family":"Chochkova-Bukova","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8703-8022","authenticated-orcid":true,"given":"Elisaveta","family":"Levunlieva","sequence":"additional","affiliation":[{"name":"National Heart Hospital, Sofia, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7902-5494","authenticated-orcid":true,"given":"Anna","family":"Kaneva-Nencheva","sequence":"additional","affiliation":[{"name":"National Cardiology Hospital, Sofia, Bulgaria"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2258","published-online":{"date-parts":[[2026,3,19]]},"reference":[{"key":"149000_B1","doi-asserted-by":"crossref","unstructured":"Su-Mei AK, Ju-Le T. Large unrepaired aortopulmonary window - survival into the seventh decade. Echocardiography 2007; 24(1):71\u20133.","DOI":"10.1111\/j.1540-8175.2006.00353.x"},{"key":"149000_B2","doi-asserted-by":"crossref","unstructured":"Cui M, Xia B, Wang H, et al. A rare case of adult aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta leading to Eisenmenger syndrome. J Int Med Res 2021; 49(1):300060520984656.","DOI":"10.1177\/0300060520984656"},{"key":"149000_B3","doi-asserted-by":"crossref","unstructured":"Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; 43(38):3618\u2013731.","DOI":"10.1093\/eurheartj\/ehac237"},{"key":"149000_B4","unstructured":"Elliotson J. Case of malformation of the pulmonary artery and aorta. Lancet 1830; 247:31."},{"key":"149000_B5","unstructured":"Umapathi KK, Nguyen H. Aortopulmonary window [Internet]. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2024 [cited 2024]. Available from: https:\/\/europepmc.org\/article\/NBK\/nbk560616."},{"key":"149000_B6","doi-asserted-by":"crossref","unstructured":"Trowitzsch E, Schneider M, Urban A, et al. Congenital pulmonary sling, aorto- pulmonary window and pulmonary vein obstruction as a diagnostic and therapeutic challenge in an infant with VACTERL association. Clin Res Cardiol 2006; 95(6):338\u201343.","DOI":"10.1007\/s00392-006-0383-x"},{"key":"149000_B7","doi-asserted-by":"crossref","unstructured":"Verma B, Abhinay A, Singh A, et al. Double outlet right ventricle and aortopulmonary window in a neonate with Bohring-Opitz (Oberklaid-Danks) syndrome: First case report. J Family Med Prim Care 2019; 8(3):1279\u201381.","DOI":"10.4103\/jfmpc.jfmpc_74_19"},{"key":"149000_B8","doi-asserted-by":"crossref","unstructured":"Mori K, Ando M, Takao A, et al. Distal type of aortopulmonary window. Report of 4 cases. Br Heart J 1978; 40(6):681\u20139.","DOI":"10.1136\/hrt.40.6.681"},{"key":"149000_B9","doi-asserted-by":"crossref","unstructured":"Jone PN, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: a scientific statement from the American Heart Association. Circ Heart Fail 2023; 16(7):e00080.","DOI":"10.1161\/HHF.0000000000000080"},{"key":"149000_B10","doi-asserted-by":"crossref","unstructured":"Hansmann G, Koestenberger M, Alastalo TP, et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant 2019; 38(9):879\u2013901.","DOI":"10.1016\/j.healun.2019.06.022"},{"key":"149000_B11","unstructured":"Law MA, Mahajan K. Aortopulmonary septal defect. StatPearls. Treasure Island (FL) companies. StatPearls Publishing LLC.; 2024. Available from: https:\/\/www.ncbi.nlm.nih.gov\/sites\/books\/NBK442003\/?report=classic"},{"key":"149000_B12","doi-asserted-by":"crossref","unstructured":"Huang JB, Liang J, Zhou LY. Eisenmenger syndrome: not always inoperable. Respir Care 2012; 57(9):1488\u201395.","DOI":"10.4187\/respcare.01418"},{"key":"149000_B13","doi-asserted-by":"crossref","unstructured":"Jone P-N, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: a scientific statement from the American Heart Association. Circulation: Heart Failure 2023; 16(7):e00080.","DOI":"10.1161\/HHF.0000000000000080"},{"key":"149000_B14","doi-asserted-by":"crossref","unstructured":"Diller GP, K\u00f6rten MA, Bauer UM, et al. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J 2016; 37(18):1449\u201355.","DOI":"10.1093\/eurheartj\/ehv743"}],"container-title":["Folia Medica"],"original-title":[],"link":[{"URL":"https:\/\/foliamedica.bg\/article\/149000\/download\/pdf\/","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/foliamedica.bg\/article\/149000\/download\/xml\/","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/foliamedica.bg\/article_preview.php?id=149000&skip_redirect=1","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T10:49:04Z","timestamp":1773917344000},"score":1,"resource":{"primary":{"URL":"https:\/\/foliamedica.bg\/article\/149000\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,19]]},"references-count":14,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,3,19]]}},"URL":"https:\/\/doi.org\/10.3897\/folmed.68.e149000","relation":{},"ISSN":["1314-2143","0204-8043"],"issn-type":[{"value":"1314-2143","type":"electronic"},{"value":"0204-8043","type":"print"}],"subject":[],"published":{"date-parts":[[2026,3,19]]},"article-number":"e149000"}}