Complex Intrahepatic Vascular Shunts in Hereditary Hemorrhagic Telangiectasia: From Portal Hypertension to High-Output Cardiac Failure
DOI:
https://doi.org/10.60591/crspmi.557Keywords:
Hypertension, Portal, Liver Diseases, Telangiectasia, Hereditary HemorrhagicAbstract
Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant vascular disorder that may involve the liver through complex intrahepatic vascular malformations. We report the case of a 64-year-old man with genetically confirmed HHT type 2 who developed symptomatic hepatic vascular shunts, complicated by clinically significant portal hypertension, recurrent gastrointestinal bleeding, and high-output cardiac failure. The patient was treated with supportive measures and antiangiogenic therapy with bevacizumab, resulting in transient clinical and hemodynamic improvement. However, disease progression was marked by refractory ascites, variceal bleeding, severe malnutrition, sarcopenia, and progressive frailty, ultimately limiting therapeutic options, including liver transplantation. This case illustrates the heterogeneous clinical expression of hepatic involvement in HHT, the potential benefits and limitations of bevacizumab therapy, and highlights the impact of frailty and nutritional deterioration on clinical decision-making and prognosis.
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Copyright (c) 2026 Filipe Nery, Diana Valadares, Manuel Teixeira Gomes
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