{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T12:58:54Z","timestamp":1792673934478,"version":"4.5.0"},"reference-count":35,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T00:00:00Z","timestamp":1753920000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Nutr."],"abstract":"<jats:p>Thiamine deficiency (TD), also known as vitamin B1 deficiency, is an often overlooked potential cause of pulmonary arterial hypertension (PAH). It may contribute to the pathological process of PAH through the deficiency of related coenzymes, thereby inhibiting the citric acid cycle. TD can manifest in various clinical forms, including dry beriberi and Shoshin beriberi, the latter characterized by high-output heart failure and lactic acidosis, which can lead to fatal outcomes if not promptly treated. This article reviews the physiological functions of thiamine, the pathophysiological mechanisms of TD, and its relationship with PAH. It explores the hemodynamic changes and diagnostic challenges of Shoshin beriberi and highlights the potential value of thiamine supplementation therapy. Although large-scale randomized controlled trials are currently lacking, minor studies and case reports suggest that thiamine supplementation may benefit PAH patients. Future high-quality research is needed to clarify the role of thiamine in the treatment of PAH.<\/jats:p>","DOI":"10.3389\/fnut.2025.1633864","type":"journal-article","created":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T15:31:16Z","timestamp":1753975876000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["An easily overlooked cause of pulmonary arterial hypertension\u2014thiamine deficiency"],"prefix":"10.3389","volume":"12","author":[{"given":"Qianqian","family":"Zhao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Menglin","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fangfang","family":"Qiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,7,31]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1016\/0002-9343(66)90016-7","article-title":"Hemodynamic studies in beriberi heart disease","volume":"41","author":"Akbarian","year":"1966","journal-title":"Am J Med"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1042\/BSR20230374","article-title":"The importance of thiamine (vitamin B1) in humans","volume":"43","author":"Mrowicka","year":"2023","journal-title":"Biosci Rep"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"973","DOI":"10.1172\/jci.insight.178973","article-title":"Dldhcri3 zebrafish exhibit altered mitochondrial ultrastructure, morphology, and dysfunction partially rescued by probucol or thiamine","volume":"9","author":"Lavorato","year":"2024","journal-title":"JCI Insight"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.neuint.2003.12.007","article-title":"Increased brain endothelial nitric oxide synthase expression in thiamine deficiency: relationship to selective vulnerability","volume":"45","author":"Kruse","year":"2004","journal-title":"Neurochem Int"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1161\/circresaha.113.301129","article-title":"The right ventricle in pulmonary arterial hypertension","volume":"115","author":"Ryan","year":"2014","journal-title":"Circ Res"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1159\/000366012","article-title":"Beriberi, a severe complication after metabolic surgery - review of the literature","volume":"7","author":"Stroh","year":"2014","journal-title":"Obes Facts"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1128\/mBio.00886-20","article-title":"Vitamin biosynthesis by human gut butyrate-producing Bacteria and cross-feeding in synthetic microbial communities","volume":"11","author":"Soto-Martin","year":"2020","journal-title":"MBio"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"892","DOI":"10.1007\/s00018-007-6423-5","article-title":"Structure, mechanism and catalytic duality of thiamine-dependent enzymes","volume":"64","author":"Frank","year":"2007","journal-title":"Cell Mol Life Sci"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1186\/s12937-015-0039-7","article-title":"Shoshin beriberi in critically-ill patients: case series","volume":"14","author":"Dabar","year":"2015","journal-title":"Nutr J"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"23","DOI":"10.12659\/AJCR.935308","article-title":"A case of pulmonary hypertension in a 67-year-old woman with thiamine deficiency following partial gastrectomy and exacerbated by diuretics","volume":"23","author":"Shibayama","year":"2022","journal-title":"Am J Case Rep"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"e0010","DOI":"10.1097\/MD.0000000000010010","article-title":"Wet beriberi with multiple organ failure remarkably reversed by thiamine administration","volume":"97","author":"Lei","year":"2018","journal-title":"Medicine"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1111\/nyas.14536","article-title":"Thiamine deficiency disorders: a clinical perspective","volume":"1498","author":"Smith","year":"2021","journal-title":"Ann N Y Acad Sci"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/s12937-016-0156-y","article-title":"Case report: pericardial effusion with constrictive physiology in a patient with wet beriberi","volume":"15","author":"Yamamura","year":"2015","journal-title":"Nutr J"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"1312","DOI":"10.1093\/eurheartj\/ehad005","article-title":"ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European reference network on rare respiratory diseases (ERN-LUNG)","volume":"44","author":"Humbert","year":"2022","journal-title":"Eur Heart J"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"1534","DOI":"10.1016\/j.cjco.2024.09.013","article-title":"Rapid reversal of pulmonary hypertension by high-dose thiamine supplementation: a case report","volume":"6","author":"Moriizumi","year":"2024","journal-title":"CJC Open"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"199","DOI":"10.3390\/children7110199","article-title":"Thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI)-a case series and clinical review","volume":"7","author":"Panigrahy","year":"2020","journal-title":"Children (Basel, Switzerland)"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"614","DOI":"10.3389\/fphys.2017.00614","article-title":"Regulation of pulmonary vascular smooth muscle contractility in pulmonary arterial hypertension: implications for therapy","volume":"8","author":"Lyle","year":"2017","journal-title":"Front Physiol"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"1115","DOI":"10.1007\/s10237-012-0467-7","article-title":"Effects of collagen deposition on passive and active mechanical properties of large pulmonary arteries in hypoxic pulmonary hypertension","volume":"12","author":"Wang","year":"2013","journal-title":"Biomech Model Mechanobiol"},{"key":"ref19","first-page":"1115","article-title":"Shoshin beriberi. A rapidly curable hemodynamic disaster","volume":"25","author":"Meurin","year":"1996","journal-title":"Presse Med"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1007\/s00431-013-2034-3","article-title":"Rapid progression to pulmonary arterial hypertension crisis associated with mixed connective tissue disease in an 11-year-old girl","volume":"172","author":"Okura","year":"2013","journal-title":"Eur J Pediatr"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1111\/chf.12037","article-title":"Thiamine supplementation for the treatment of heart failure: a review of the literature","volume":"19","author":"DiNicolantonio","year":"2013","journal-title":"Congest Heart Fail"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"1408","DOI":"10.1111\/j.1468-1331.2010.03153.x","article-title":"EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy","volume":"17","author":"Galvin","year":"2010","journal-title":"Eur J Neurol"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"569","DOI":"10.4070\/kcj.2013.43.8.569","article-title":"A case of cardiac beriberi: a forgotten but memorable disease","volume":"43","author":"Lee","year":"2013","journal-title":"Korean Circ J"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"665","DOI":"10.1016\/j.ccm.2013.09.001","article-title":"Diagnosis of pulmonary arterial hypertension","volume":"34","author":"Forfia","year":"2013","journal-title":"Clin Chest Med"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"1795","DOI":"10.1007\/s00134-014-3525-z","article-title":"Consensus on circulatory shock and hemodynamic monitoring. Task force of the European Society of Intensive Care Medicine","volume":"40","author":"Cecconi","year":"2014","journal-title":"Intensive Care Med"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"147","DOI":"10.5847\/wjem.j.1920-8642.2024.018","article-title":"Extracorporeal membrane oxygenation as a bridging therapy for patients with thiamine deficiency-induced pulmonary arterial hypertension","volume":"15","author":"Qiu","year":"2024","journal-title":"World J Emerg Med"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"e73","DOI":"10.1016\/j.jemermed.2008.03.040","article-title":"Shoshin beriberi mimicking a high-risk non-ST-segment elevation acute coronary syndrome with cardiogenic shock: when the arteries are not guilty","volume":"41","author":"Loma-Osorio","year":"2011","journal-title":"J Emerg Med"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"2686","DOI":"10.1002\/ehf2.14374","article-title":"Pulmonary artery catheter usage in diagnosis of shoshin beriberi presented with unexplained lactic acidosis","volume":"10","author":"Hung","year":"2023","journal-title":"ESC Heart Failure"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"37","author":"Gali\u00e8","year":"2016","journal-title":"Eur Heart J"},{"key":"ref30","doi-asserted-by":"publisher","first-page":"827","DOI":"10.1097\/MAT.0000000000001510","article-title":"ELSO interim guidelines for Venoarterial extracorporeal membrane oxygenation in adult cardiac patients","volume":"67","author":"Lorusso","year":"2021","journal-title":"ASAIO J"},{"key":"ref31","doi-asserted-by":"publisher","first-page":"1478889","DOI":"10.3389\/fped.2025.1478889","article-title":"Case report: severe pulmonary hypertension in a child with micronutrient deficiency","volume":"13","author":"Pache-Wannaz","year":"2025","journal-title":"Front Pediatr"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"e67445","DOI":"10.7759\/cureus.67445","article-title":"A case report of wet beriberi due to excessive white rice consumption in an elderly male patient: a potentially forgotten and underrecognized disease","volume":"16","author":"Kurisu","year":"2024","journal-title":"Cureus"},{"key":"ref33","doi-asserted-by":"publisher","first-page":"396","DOI":"10.1016\/j.amjms.2022.12.006","article-title":"Raise vigilance against refractory distributive shock due to severe wet beriberi","volume":"365","author":"Ma","year":"2023","journal-title":"Am J Med Sci"},{"key":"ref34","doi-asserted-by":"publisher","first-page":"42","DOI":"10.5830\/CVJA-2021-051","article-title":"Resurgence of shoshin beriberi during the COVID-19 pandemic","volume":"34","author":"Govind","year":"2023","journal-title":"Cardiovasc J Afr"},{"key":"ref35","doi-asserted-by":"publisher","first-page":"e241654","DOI":"10.1136\/bcr-2021-241654","article-title":"Wide pulse pressure and quincke\u2019s pulse in high-output heart failure","volume":"14","author":"Berry","year":"2021","journal-title":"BMJ Case Rep"}],"container-title":["Frontiers in Nutrition"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fnut.2025.1633864\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T15:31:17Z","timestamp":1753975877000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fnut.2025.1633864\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,31]]},"references-count":35,"alternative-id":["10.3389\/fnut.2025.1633864"],"URL":"https:\/\/doi.org\/10.3389\/fnut.2025.1633864","relation":{},"ISSN":["2296-861X"],"issn-type":[{"value":"2296-861X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,31]]},"article-number":"1633864"}}