Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava

Abstract Background Budd–Chiari syndrome is a rare and severe vascular liver disease. We presented patient with fulminant liver failure secondary to leiomyosarcoma of the IVC and thrombosis. Case presentation A 44-year-old female presented with fulminant liver failure secondary to inferior vena cava...

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Main Authors: Maciej Wiewiora, Hanna Wiewiora, Ewa Chmielik, Michal Jarzab, Michael Grynkiewicz, Marcin Kubeczko
Format: Article
Language:English
Published: BMC 2024-11-01
Series:Cardio-Oncology
Subjects:
Online Access:https://doi.org/10.1186/s40959-024-00287-1
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author Maciej Wiewiora
Hanna Wiewiora
Ewa Chmielik
Michal Jarzab
Michael Grynkiewicz
Marcin Kubeczko
author_facet Maciej Wiewiora
Hanna Wiewiora
Ewa Chmielik
Michal Jarzab
Michael Grynkiewicz
Marcin Kubeczko
author_sort Maciej Wiewiora
collection DOAJ
description Abstract Background Budd–Chiari syndrome is a rare and severe vascular liver disease. We presented patient with fulminant liver failure secondary to leiomyosarcoma of the IVC and thrombosis. Case presentation A 44-year-old female presented with fulminant liver failure secondary to inferior vena cava (IVC) thrombosis. Contrast-enhanced computed tomography subsequently revealed a thrombus within the IVC, extending cranially to the right atrium and caudally to the renal veins. The patient’s condition, characterized by early comatose symptoms, necessitated surgical intervention. Under extracorporeal circulation, a right atriotomy with thrombus lesion removal and descending thrombectomy of the IVC was performed. Hepatic congestion resolved after the thrombus was removed. A pathological examination of the excised thrombus revealed the presence of high-grade leiomyosarcoma. Conclusions In cases where a thrombus extends from the IVC to the right atrium, urgent surgical intervention with extracorporeal circulation should be considered.
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issn 2057-3804
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publisher BMC
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series Cardio-Oncology
spelling doaj-art-19b91d0bc1d5468c9767b519a31428372024-12-01T12:47:10ZengBMCCardio-Oncology2057-38042024-11-011011410.1186/s40959-024-00287-1Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cavaMaciej Wiewiora0Hanna Wiewiora1Ewa Chmielik2Michal Jarzab3Michael Grynkiewicz4Marcin Kubeczko5Department of Cardiac Vascular and Endovascular Surgery and Transplantology, Faculty of Medical Sciences in Zabrze, Medical University of Silesia in KatowiceStudent Scientific Society, Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Faculty of Medical Sciences in ZabrzeTumor Pathology Department, Maria Sklodowska-Curie National Research Institute of OncologyBreast Cancer Center, Maria Sklodowska-Curie National Research Institute of OncologyStudent Scientific Society, Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Faculty of Medical Sciences in ZabrzeBreast Cancer Center, Maria Sklodowska-Curie National Research Institute of OncologyAbstract Background Budd–Chiari syndrome is a rare and severe vascular liver disease. We presented patient with fulminant liver failure secondary to leiomyosarcoma of the IVC and thrombosis. Case presentation A 44-year-old female presented with fulminant liver failure secondary to inferior vena cava (IVC) thrombosis. Contrast-enhanced computed tomography subsequently revealed a thrombus within the IVC, extending cranially to the right atrium and caudally to the renal veins. The patient’s condition, characterized by early comatose symptoms, necessitated surgical intervention. Under extracorporeal circulation, a right atriotomy with thrombus lesion removal and descending thrombectomy of the IVC was performed. Hepatic congestion resolved after the thrombus was removed. A pathological examination of the excised thrombus revealed the presence of high-grade leiomyosarcoma. Conclusions In cases where a thrombus extends from the IVC to the right atrium, urgent surgical intervention with extracorporeal circulation should be considered.https://doi.org/10.1186/s40959-024-00287-1Budd–Chiari syndromeThrombosisLeiomyosarcoma of the inferior vena cava
spellingShingle Maciej Wiewiora
Hanna Wiewiora
Ewa Chmielik
Michal Jarzab
Michael Grynkiewicz
Marcin Kubeczko
Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
Cardio-Oncology
Budd–Chiari syndrome
Thrombosis
Leiomyosarcoma of the inferior vena cava
title Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
title_full Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
title_fullStr Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
title_full_unstemmed Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
title_short Sternotomy and extracorporal circulation for fulminant Budd-Chiari syndrome due to leiomyosarcoma of the inferior vena cava
title_sort sternotomy and extracorporal circulation for fulminant budd chiari syndrome due to leiomyosarcoma of the inferior vena cava
topic Budd–Chiari syndrome
Thrombosis
Leiomyosarcoma of the inferior vena cava
url https://doi.org/10.1186/s40959-024-00287-1
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