{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T07:12:44Z","timestamp":1777878764494,"version":"3.51.4"},"reference-count":16,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2025,4,13]],"date-time":"2025-04-13T00:00:00Z","timestamp":1744502400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["World J Pediatr Congenit Heart Surg"],"published-print":{"date-parts":[[2025,9]]},"abstract":"<jats:p>Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors that secrete catecholamines. They can induce severe hypertension and tachycardia; thus, complicating the health of patients with stabilized congenital heart disease. Chronic hypoxemia is a potential driver for tumor development as discovered by the role of hypoxia-inducible factors in tumorigenesis. This case report describes a 24-year-old woman with hypoplastic left heart syndrome and fenestrated Fontan palliation who developed an adrenergic crisis following adjustments to her medications. She was initially on metoprolol for ventricular ectopy and suspended it to initiate phenoxybenzamine, an alpha-blocker, in preparation for surgical removal of a hormonally active retroperitoneal paraganglioma. Six days after discontinuation of the metoprolol, she presented to the emergency department with severe abdominal pain, palpitations, and nausea. The evaluation revealed supraventricular tachycardia and elevated blood pressure, which did not respond to initial treatments. She was admitted to the cardiac intensive care unit where she experienced further deterioration, necessitating intubation and treatment with amiodarone for rhythm control of her identified ectopic atrial tachycardia. Following stabilization and successful extubation, the patient was discharged on a regimen including amiodarone, metoprolol, and doxazosin. She successfully had her surgery in the following months and was weaned off amiodarone and metoprolol while remaining free of arrhythmias. This case highlights the complexities of managing PPGLs in Fontan patients and emphasizes the importance of multidisciplinary care to prevent and manage adrenergic crises.<\/jats:p>","DOI":"10.1177\/21501351251322164","type":"journal-article","created":{"date-parts":[[2025,4,14]],"date-time":"2025-04-14T02:13:21Z","timestamp":1744596801000},"page":"688-691","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Management of a Paraganglioma in a Fontan Patient: A Complex Case of Adrenergic Imbalance and Perioperative Care"],"prefix":"10.1177","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9997-0098","authenticated-orcid":false,"suffix":"Jr","given":"Carlos A.","family":"Carmona","sequence":"first","affiliation":[{"name":"Department of Pediatric Critical Care Medicine, Children's Hospital of Richmond, Richmond, VA, USA"},{"name":"Department of Pediatric Cardiology, Phoenix Children's Hospital, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael R.","family":"Bykhovsky","sequence":"additional","affiliation":[{"name":"Department of Adult Congenital Cardiac Disease, Children's National Hospital, Washington, DC, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth D.","family":"Sherwin","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiac Electrophysiology, Children's National Hospital, Washington, DC, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregory K.","family":"Yurasek","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiac Critical Care, Children's National Hospital, Washington, DC, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2025,4,13]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11886-023-01974-8"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/chd.12625"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1210\/jendso\/bvac004"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.29.5.750"},{"issue":"5","key":"e_1_3_2_6_2","first-page":"834","article-title":"Survival to adulthood in a patient with complete transposition of the great vessels","volume":"57","author":"Roberts WC","year":"1962","unstructured":"Roberts WC. Survival to adulthood in a patient with complete transposition of the great vessels. Intern Med. 1962; 57( 5): 834\u2013 842.","journal-title":"Intern Med"},{"key":"e_1_3_2_7_2","unstructured":"Arenillas C Ruiz-Cantador J Celada L et al. Convergent genetic adaptation in human tumors developed under systemic hypoxia and in populations living at high altitudes. bioRxiv 2024.06.10.594693. doi:https:\/\/doi.org\/10\/1101\/2024.06.10.594693."},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgac362"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2014-3863"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1716652"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1097\/HJH.0000000000002438"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2265.2006.02571.x"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1053\/jcan.2002.124150"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2020.586795"},{"key":"e_1_3_2_15_2","doi-asserted-by":"crossref","unstructured":"Lui GK Saidi A Bhatt AB et al. American Heart Association Adult Congenital Heart Disease Committee of the Council on Clinical Cardiology and Council on Cardiovascular Disease in the Young; Council on Cardiovascular Radiology and Intervention; and Council on Quality of Care and Outcomes Research. Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation. 2017;136(20):e348\u2013e392. doi:10.1161\/CIR.0000000000000535.","DOI":"10.1161\/CIR.0000000000000535"},{"issue":"101","key":"e_1_3_2_16_2","first-page":"1672","article-title":"Prevalence of atrial tachyarrhythmia in adults after Fontan operation","volume":"20","author":"Quinton E","year":"2015","unstructured":"Quinton E, Nightingale P, Hudsmith L, et al. Prevalence of atrial tachyarrhythmia in adults after Fontan operation. Congenit Heart Dis. 2015; 20( 101): 1672\u2013 1677.","journal-title":"Congenit Heart Dis"},{"key":"e_1_3_2_17_2","unstructured":"Stephenson EA Lu M Berul CI et al. Arrhythmias in a contemporary fontan cohort: prevalence and clinical associations in a multicenter cross-sectional study. J Am Coll Cardiol. 2010; 56( 11): 890\u2013 896."}],"container-title":["World Journal for Pediatric and Congenital Heart Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/21501351251322164","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/21501351251322164","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/21501351251322164","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T23:27:25Z","timestamp":1777591645000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/21501351251322164"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,13]]},"references-count":16,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,9]]}},"alternative-id":["10.1177\/21501351251322164"],"URL":"https:\/\/doi.org\/10.1177\/21501351251322164","relation":{},"ISSN":["2150-1351","2150-136X"],"issn-type":[{"value":"2150-1351","type":"print"},{"value":"2150-136X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4,13]]}}}