High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations,
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RESEARCH
High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations, evaluation of treatment Lilian B. Olsen1, Anette D. Kjeldsen1,2,5, Mikael K. Poulsen1,3,5, Jens Kjeldsen1,4,5 and Annette D. Fialla1,4,5*
Abstract Background: This report addresses how patients with hereditary hemorrhagic telangiectasia (HHT) and high output cardiac failure (HOCF) due to hepatic vascular malformations, should be evaluated and could be treated. HHT is a genetic disorder, leading to vascular abnormalities with potentially serious clinical implications. In the liver, arteriovenous malformations occur in more than 70% of patients, but only about 8% present clinical symptoms such as HOCF with pulmonary hypertension and less commonly portal hypertension, biliary ischemia and hepatic encephalopathy. Results: Three female patients with HHT type 2 and HOCF caused by severe arteriovenous malformations in the liver are presented in this case series. The patients were seen at the HHT-Centre at Odense University Hospital. Treatment with either orthotopic liver transplantation (one patient) or bevacizumab (two patients) was initiated. All patients experienced marked symptom relief and objective improvement. New York Heart Association—class were improved, ascites, peripheral edema and hence diuretic treatment was markedly reduced or discontinued in all three patients. Bevacizumab also resulted in notable effects on epistaxis and anemia. Conclusion: Our findings substantiate the importance of identification of symptomatic arteriovenous malformations in the liver in patients with HHT. Bevacizumab may possibly, as suggested in this case series and supported by previous case studies, postpone the time to orthotopic liver transplantation or even make it unnecessary. Bevacizumab represents a promising new treatment option, which should be investigated further in clinical trials. Keywords: Hereditary hemorrhagic telangiectasia, HHT, Vascular endothelial growth factor inhibitor, Bevacizumab, Hepatic, Right heart failure Introduction Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder, leading to vascular abnormalities with potentially serious clinical implications [1, 2]. It is an autosomal dominant disease with an estimated prevalence of *Correspondence: [email protected] 1 HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark Full list of author information is available at the end of the article
1:6.500 in Denmark [3]. It is commonly associated with multiple telangiectatic lesions at characteristic sites as the skin of the face and fingertips. HHT also manifests with telangiectatic lesions of the mucosa causing troublesome bleedings and potentially anemia. Finally, the patients may have visceral arteriovenous malformations (AVMs) in particular in the lungs, liver and central nervous system which can result in severe organ dysfunction and failure [4–7].
© The Author(s) 2020. This article is licensed under a Creative Commons Attribution
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