{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T10:28:04Z","timestamp":1775039284825,"version":"3.50.1"},"reference-count":6,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"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>Congenitally corrected transposition of the great arteries (ccTGA) is an uncommon congenital heart defect involving both atrioventricular (AV) and ventriculoarterial (VA) discordance. This condition is further complicated by additional anomalies such as a hypoplastic pulmonary valve, ventricular septal defect (VSD), and dextroversion. These abnormalities may contribute to an increased risk of heart failure and cardiogenic shock, making early detection and appropriate intervention essential for better patient outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 16-year-old female presented with progressive shortness of breath, cyanosis, recurrent episodes of syncope, and exertional palpitations. Upon admission, the patient exhibited severe hypoxia, tachycardia, and hypotension, consistent with cardiogenic shock. She was NYHA grade IV at the time of presentation.Imaging studies, including chest X-ray and echocardiography, GLS- strain imaging and cardiac MRI confirmed the diagnosis of ccTGA with AV and VA discordance, a left-sided aortic arch, subaortic VSD, a hypoplastic pulmonary valve, and left sided morphologic right ventricular systolic dysfunction. Blood investigations indicated polycythemia and signs of congestive hepatopathy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Management and outcome<\/jats:title>\n                    <jats:p>The patient was initially stabilized with inotropic support using norepinephrine (0.05\u20130.5 mcg\/kg\/min), along with diuretics and ACE inhibitors. Over the course of seven days, her condition improved significantly, allowing for the withdrawal of inotropic support. She was subsequently referred for surgical evaluation, with potential options including the double-switch procedure or VSD closure, depending on the extent of structural and functional abnormalities. Our patient underwent double switch procedure despite having RV dysfunction and improved significantly over a period of 3 months.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case underscores the significance of prompt recognition and aggressive management in complex congenital heart conditions like ccTGA. Cases of symptomatic ccTGA in adolescence are exceptionally rare, highlighting the importance of a multidisciplinary approach in optimizing patient care and surgical planning.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s43044-026-00733-8","type":"journal-article","created":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T08:24:15Z","timestamp":1775031855000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Adolescent presentation of L-type ccTGA with pulmonary valve hypoplasia and RV dysfunction: from cardiogenic shock to surgical repair"],"prefix":"10.1186","volume":"78","author":[{"given":"Shubham","family":"Jain","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Deepak","family":"Kumar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naween","family":"Kumar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pankaj Mohan","family":"Shrivastava","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,4,1]]},"reference":[{"key":"733_CR1","volume-title":"The Heart","year":"2007","unstructured":"Hurst\u2019s (2007) In: Fuster V, Walsh R, Harrington R (eds) The Heart, 13th edn. McGraw Hill, New York.","edition":"13"},{"key":"733_CR2","unstructured":"Bonow RO, Mann DL, Zipes DP, Libby P, Netherlands (eds) (2011) Braunwald\u2019s Heart Disease: A Textbook of Cardiovascular Medicine. Elsevier."},{"key":"733_CR3","unstructured":"Perloff JK (ed) (2003) 3rd ed. Philadelphia: Saunders, Clinical Recognition of Congenital Heart Disease."},{"key":"733_CR4","doi-asserted-by":"crossref","unstructured":"Flack EC, Graham TP Congenital Heart Disease: Selected Aspects. London, UK: Intech Open, 2012. Congenitally Corrected Transposition of the Great Arteries.","DOI":"10.5005\/jp\/books\/12075_44"},{"key":"733_CR5","doi-asserted-by":"crossref","unstructured":"A case of congenitally (2017) corrected transposition of great arteries: an infrequent happenstance. Ajmera P, Medep V. Case Rep Med. 7565870.","DOI":"10.1155\/2017\/7565870"},{"key":"733_CR6","first-page":"2014","volume":"21","author":"A Placci","year":"2014","unstructured":"Congenitally corrected transposition, Placci A, Lovato L, Bonvicini M (2014) of the great arteries in an 83-year-old asymptomatic patient: description and literature review. BMJ Case Rep 21:2014.","journal-title":"BMJ Case Rep"}],"container-title":["The Egyptian Heart Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43044-026-00733-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s43044-026-00733-8","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s43044-026-00733-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T08:24:24Z","timestamp":1775031864000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1186\/s43044-026-00733-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,1]]},"references-count":6,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["733"],"URL":"https:\/\/doi.org\/10.1186\/s43044-026-00733-8","relation":{},"ISSN":["2090-911X"],"issn-type":[{"value":"2090-911X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,1]]},"assertion":[{"value":"9 July 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 April 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":"The patient\u2019s mother shared that she had been worried for years due to repeated fainting episodes and was relieved to finally receive a diagnosis and see her child alive after such a dreadful condition, also she improved dramatically within a week of admission.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Patient perspective"}},{"value":"Written informed consent was obtained from the patient\u2019s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"21"}}