Portopulmonary Hypertension Complicating Portal Vein Thrombosis : 1 Patient, 2 Outcomes

Article Article scientifique Rapport de cas Portopulmonary Hypertension Complicating Portal Vein Thrombosis : 1 Patient, 2 Outcomes eng 2025-12-26 2026-06 author Credeville Mathilde Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada. 715602 author Joye Raphaël Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada. 678463 author Flanagan Mary Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada. author Siddiqui Iram Division of Pathology, Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada. author Mets Gilles Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada. author Reyes Janette Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada. author Mertens Luc Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada. author Dragulescu Andreea Division of Cardiology, The Hospital for Sick Children, Toronto, Ontario, Canada. Electronic address: andreea.dragulescu@sickkids.ca. 1258-1260 10.1016/j.cjca.2025.12.034 41456703 0828-282X https://www.sciencedirect.com/science/article/pii/S0828282X25016022 Portopulmonary hypertension (PoPH) is characterized by the combination of pulmonary arterial hypertension (PAH) and portal hypertension or portosystemic shunt. Although 1%-5% of children with portal hypertension develop PoPH, the prevalence in those with congenital portosystemic shunt reaches up to 15%. The prognosis of patients with PoPH is poor, with a 5-year survival rates of 35%-50%. Pediatric data on PoPH management remain limited and extrapolated from adult guidelines. In this study, we present the case of a child who underwent 2 separate surgeries aimed at restoring intrahepatic blood flow, each with distinct preoperative hemodynamic profiles, resulting in 2 different outcomes. The patient and her parents provided written informed consent for this report. Meso-Rex bypass Liver transplantation Portal vein thrombosis Portopulmonary hypertension true 179 L'hypertension pulmonaire (pédiatrie) 228 Canadian journal of cardiology 42 6 2c77f692-bc77-4493-b3b8-51e4d8a0f13d Article (Published version) Restricted PIIS0828282X25016022.pdf 1686902 application/pdf 62ed912bf7ac299dbd18ac5a3f5426270229cad317aa6cc99dce61ef0cdbc58c restrictedAccess

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Portopulmonary Hypertension Complicating Portal Vein Thrombosis : 1 Patient, 2 Outcomes	Canadian journal of cardiology			2026	9	0

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Credeville, Mathilde