{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T10:28:14Z","timestamp":1773052094773,"version":"3.50.1"},"reference-count":9,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Egypt Heart J"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Aortopulmonary window (APW) is a rare congenital heart defect, accounting for only 0.2\u20130.6% of all congenital cardiac anomalies, and is usually identified and surgically corrected within the first year of life to prevent irreversible pulmonary hypertension (PH). Once pulmonary vascular resistance (PVR) becomes fixed, surgical repair is generally considered contraindicated. However, emerging evidence suggests that pulmonary vascular reactivity may persist in select adolescents, opening a potential therapeutic window beyond infancy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>We report a 15-year-old male with a large APW diagnosed in infancy but lost to follow-up, who presented in adolescence with exertional dyspnea. Initial cardiac catheterization revealed a high indexed PVR (10.89 WU\u00b7m\u00b2) and absent oxygen vasoreactivity, indicating inoperability. Despite this, preserved biventricular function and lack of cyanosis prompted a trial of targeted medical therapy with sildenafil, spironolactone, lisinopril, and digoxin. After 12 months, repeat catheterization showed dramatic haemodynamic improvement: baseline PVRi decreased to 8.84 WU\u00b7m\u00b2 and fell further to 0.76 WU\u00b7m\u00b2 after 100% oxygen, with a reduction in PVR\/SVR ratio from 0.58 to 0.04. The calculated Qp\/Qs increased to 16.77, although this extreme value was considered likely overestimated because systemic and pulmonary arterial oxygen saturations were almost identical. Definitive surgical repair was undertaken with excellent early results. On follow-up, the patient remained asymptomatic with preserved biventricular function, low estimated pulmonary artery pressures and no residual APW on echocardiography.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case suggests that operability in APW may not be irrevocably lost beyond infancy in carefully selected adolescents. It underscores the importance of individualized assessment, medical preconditioning and serial haemodynamic reassessment to unmask latent pulmonary vasoreactivity. Larger series are needed to define which late-presenting patients with APW and PH may safely benefit from definitive surgical repair.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s43044-026-00726-7","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T08:50:28Z","timestamp":1773046228000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Reversing the irreversible? A case of successful surgical repair in a late-presenting aortopulmonary window with severe pulmonary hypertension"],"prefix":"10.1186","volume":"78","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2169-0971","authenticated-orcid":false,"given":"Rido","family":"Mulawarman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6909-5544","authenticated-orcid":false,"given":"Ericko","family":"Ongko Joyo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8446-1979","authenticated-orcid":false,"given":"Muhamad Adrin","family":"Aefiansyah Putra","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8854-9326","authenticated-orcid":false,"given":"Aditya","family":"Agita Sembiring","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1415-0387","authenticated-orcid":false,"given":"Sisca","family":"Natalia Siagian","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3802-4801","authenticated-orcid":false,"given":"Oktavia","family":"Lilyasari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,3,9]]},"reference":[{"issue":"3","key":"726_CR1","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1016\/j.atssr.2024.04.023","volume":"2","author":"FD Agwar","year":"2024","unstructured":"Agwar FD, Ganjula SG, Waktola TG (2024) Aortopulmonary window in adult patient operated on with echocardiography assessment. Annals Thorac Surg Short Rep 2(3):407\u2013409","journal-title":"Annals Thorac Surg Short Rep"},{"issue":"2","key":"726_CR2","doi-asserted-by":"publisher","first-page":"608","DOI":"10.1016\/j.athoracsur.2016.02.035","volume":"102","author":"B Alsoufi","year":"2016","unstructured":"Alsoufi B, Schlosser B, McCracken C, Kogon B, Kanter K, Border W et al (2016) Current Outcomes of Surgical Management of Aortopulmonary Window and Associated Cardiac Lesions. Ann Thorac Surg 102(2):608\u2013614","journal-title":"Ann Thorac Surg"},{"issue":"12","key":"726_CR3","doi-asserted-by":"publisher","first-page":"1890","DOI":"10.1016\/S0735-1097(02)01886-7","volume":"39","author":"JI Hoffman","year":"2002","unstructured":"Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. 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J Am Coll Cardiol 53(9):733\u2013740","journal-title":"J Am Coll Cardiol"},{"issue":"4","key":"726_CR6","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1136\/heartjnl-2018-314025","volume":"105","author":"DE Van der Feen","year":"2019","unstructured":"Van der Feen DE, Bartelds B, de Boer RA, Berger RMF (2019) Assessment of reversibility in pulmonary arterial hypertension and congenital heart disease. Heart 105(4):276\u2013282","journal-title":"Heart"},{"issue":"9","key":"726_CR7","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1016\/j.healun.2019.06.022","volume":"38","author":"G Hansmann","year":"2019","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. 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Br Heart J 52(4):468\u2013475","journal-title":"Br Heart J"}],"container-title":["The Egyptian Heart Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43044-026-00726-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s43044-026-00726-7","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43044-026-00726-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T08:50:29Z","timestamp":1773046229000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s43044-026-00726-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,9]]},"references-count":9,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["726"],"URL":"https:\/\/doi.org\/10.1186\/s43044-026-00726-7","relation":{},"ISSN":["2090-911X"],"issn-type":[{"value":"2090-911X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,9]]},"assertion":[{"value":"9 August 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 February 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 March 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This article describes a case report. Ethics approval was obtained from the Harapan Kita Hospital, Jakarta. Written informed consent to participate was obtained from the patient\u2019s parent.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent for publication of clinical details and images was obtained from the patient\u2019s parent.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The patient described in this case signed a standard institutional consent form in accordance with COPE guidelines. The consent contains permission to publish the case for scientific purposes. Every effort has been made to anonymize the data being presented.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"15"}}