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PoPH is driven by pulmonary vasoconstriction and progressive pulmonary arterial remodeling, whereas HPS results from pathologic vasodilation and intrapulmonary shunt formation leading to arterial hypoxemia. Their different mechanisms shape quality of life, survival, and liver transplant eligibility. Early symptoms are often mild and nonspecific, delaying diagnosis. Standard evaluation relies on echocardiography, right-heart catheterization, and arterial blood gas analysis. Therapeutic options remain limited. PoPH management uses vasodilators and supportive measures, while HPS care mainly preserves oxygenation until transplant. Future directions include targeted therapies and standardized diagnostic criteria with multicenter collaboration to improve recognition and long-term outcomes.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/portopulmonary-hypertension-and-hepatopulmonary-syndrome-contrasting-pathophysiology-and-implications-for-liver-transplantation/461447/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/portopulmonary-hypertension-and-hepatopulmonary-syndrome-contrasting-pathophysiology-and-implications-for-liver-transplantation/461447.png","ImageObject",300,407,{"name":92,"@type":93},"Muffet","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-30","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":39},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"How do PoPH and HPS differ in underlying pathophysiology?","Question",{"text":112,"@type":113},"PoPH is characterized by pulmonary vasoconstriction and gradual remodeling of pulmonary arteries. HPS develops through pathologic vasodilation with intrapulmonary shunts causing arterial hypoxemia.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"Why can diagnosis of PoPH or HPS be delayed in clinical practice?",{"text":117,"@type":113},"Early symptoms are often mild and nonspecific and may overlap with symptoms of advanced liver disease, which delays forming a diagnosis.",{"name":119,"@type":110,"acceptedAnswer":120},"What diagnostic tests are included in the standard workup?",{"text":121,"@type":113},"Standard evaluation includes echocardiography, right-heart catheterization, and analysis of arterial blood gases.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},461447,1790794554,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":39,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},962090768927,"https://ap-avatar.wpscdn.com/davatar_085a072bc5b1113ac321206ff7593b45","Review  \nPortopulmonary Hypertension and Hepatopulmonary Syndrome: Contrasting Pathophysiology and Implications for  \nLiver Transplantation  \nVanja Sili´c 1, Daniela Bandi´c Pavlovi´c 1, Fea Džubur 2, Ivan Romi´c 3, Igor Petrovi´c 3, Goran Pavlek 3, Jurica Zedelj 3, Gzim Redžepi 4 and Miroslav Samaržija 2, *  \nAcademic Editor: Ana-Maria Singeap  \nReceived: 11 November 2025  \nRevised: 10 December 2025  \nAccepted: 19 December 2025  \nPublished: 22 December 2025  \nCopyright: © 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license.  \n1 Clinic for Anesthesiology, Reanimatology and Intensive Care, University Hospital Centre Zagreb,  \n10000 Zagreb, Croatia; [vanja.silic@gmail.com](vanja.silic@gmail.com) (V.S.); [dani.bandic@gmail.com](dani.bandic@gmail.com) (D.B.P.)  \n2 Clinic for Pulmonary Diseases Jordanovac, University Hospital Centre Zagreb, 10000 Zagreb, Croatia; [fedja1104@gmail.com](fedja1104@gmail.com)  \n3 Department of Hepatobiliary and Transplantation Surgery, Clinic for Surgery, University Hospital Centre Zagreb, 10000 Zagreb, Croatia; [i.romic@gmail.com](i.romic@gmail.com) (I.R.); [igor.petrovic33@gmail.com](igor.petrovic33@gmail.com) (I.P.); [goranpavlek@gmail.com](goranpavlek@gmail.com) (G.P.); [jzedelj@gmail.com](jzedelj@gmail.com) (J.Z.)  \n4 Special Hospital Primamed, 10000 Zagreb, Croatia; [ravnatelj@primamed.hr](ravnatelj@primamed.hr)  \n* [Correspondence: miroslav.samarzija@gmail.com](Correspondence: miroslav.samarzija@gmail.com)  \nAbstract  \nPortopulmonary hypertension (PoPH) and hepatopulmonary syndrome (HPS) present two vascular complications of portal hypertension, which make opposite extremes occur against the same pathophysiological background. In PoPH, vasoconstriction predominates, along with gradual remodeling of pulmonary arteries, while HPS develops due to pathological vasodilation and creation of intrapulmonary shunts. Even though they come about by different mechanisms, both disorders significantly affect quality of life, survival, and the possibility of liver transplant. In the early phases, in clinical practice, symptoms are mainly mild and nonspecific, and overlapping with symptoms of advanced liver disease often delays forming a diagnosis. In PoPH, elevated pressures in pulmonary arteries and increased vascular resistance are observed, while HPS exhibits arterial hypoxemia with normal or lowered pulmonary pressure. Standard diagnostic workup includes echocardiography, right-heart catheterization, and analysis of the arterial gases. In patients with severe PoPH, pronounced pulmonary hypertension can represent absolute contraindication for liver transplantation due to risk of acute right heart failure during operation. Conversely, HPS usually resolves itself after a successful transplant, which confirms that the transplant is an indication of being potentially curative. Therapeutic possibilities for both states are still limited. In PoPH, specific vasodilators and supportive measures are applied, while HPS treatment is mostly supportive, directed at maintaining oxygenation until the transplant. Future research should be focused on the development of targeted therapies that address vascular remodeling, angiogenesis, and oxidative stress, as well as on the standardization of diagnostic criteria and multicentric cooperation. This approach would facilitate earlier recognition, a precise assessment of transplantability, and a better long-term outcome for patients with portal hypertension and lung vascular complications. Key Points: Portopulmonary hypertension (PoPH) and hepatopulmonary syndrome (HPS) represent two opposite vascular complications of portal hypertension, posing distinct challenges for liver transplantation. This review summarizes their pathophysiology, diagnostic pathways, and therapeutic strategies, emphasizing the importance of hemodynamic profiling and mul","cbCaii4yQr1dptB6","https://ap.wps.com/l/cbCaii4yQr1dptB6","pdf",1049629,14,"English","# Abstract\n# Key Points\n# 1. Introduction\n## 1.1. Consolidated Epidemiology","[{\"question\":\"How do PoPH and HPS differ in underlying pathophysiology?\",\"answer\":\"PoPH is characterized by pulmonary vasoconstriction and gradual remodeling of pulmonary arteries. HPS develops through pathologic vasodilation with intrapulmonary shunts causing arterial hypoxemia.\"},{\"question\":\"Why can diagnosis of PoPH or HPS be delayed in clinical practice?\",\"answer\":\"Early symptoms are often mild and nonspecific and may overlap with symptoms of advanced liver disease, which delays forming a diagnosis.\"},{\"question\":\"What diagnostic tests are included in the standard workup?\",\"answer\":\"Standard evaluation includes echocardiography, right-heart catheterization, and analysis of arterial blood gases.\"}]","Portopulmonary Hypertension and Hepatopulmonary Syndrome: Contrasting Pathophysiology and Implications for Liver Transplantation | PDF",1790761638,35]