High output cardiac failure in 3 patients with hereditary hemorrhagic telangiectasia and hepatic vascular malformations, evaluation of treatment.

Autor: Olsen LB; HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark., Kjeldsen AD; HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark.; Department of Otorhinolaryngology Head and Neck Surgery, Odense, Denmark.; Institute of Clinical Research, Odense, Denmark., Poulsen MK; HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark.; Department of Cardiology, Odense University Hospital, Odense, Denmark.; Institute of Clinical Research, Odense, Denmark., Kjeldsen J; HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark.; Department of Medical Gastroenterology and Hepatology, Odense University Hospital, Odense, Denmark.; Institute of Clinical Research, Odense, Denmark., Fialla AD; HHT-Center Odense University Hospital, Part of VASCERN, Odense, Denmark. annette.fialla@rsyd.dk.; Department of Medical Gastroenterology and Hepatology, Odense University Hospital, Odense, Denmark. annette.fialla@rsyd.dk.; Institute of Clinical Research, Odense, Denmark. annette.fialla@rsyd.dk.
Jazyk: angličtina
Zdroj: Orphanet journal of rare diseases [Orphanet J Rare Dis] 2020 Nov 26; Vol. 15 (1), pp. 334. Date of Electronic Publication: 2020 Nov 26.
DOI: 10.1186/s13023-020-01583-6
Abstrakt: 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.
Databáze: MEDLINE
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