Korean J Anesthesiol.  2009 Jan;56(1):96-101. 10.4097/kjae.2009.56.1.96.

An anesthetic experience during open heart surgery in a patient with Budd-Chiari syndrome combined with superior vena cava syndrome: A case report

• Moon HS
• Lee SK
• Choi EJ
• Shin JW

• KMID: 2031870
• DOI: http://doi.org/10.4097/kjae.2009.56.1.96

Abstract

Budd-Chiari syndrome (BCS) is a rare disorder that arises from obstruction of the hepatic venous outflow tract. BCS causes various clinical status from liver cirrhosis and other systemic diseases that are usually fatal. BCS is caused by hypercoagulability, e.g, arising from malignancy, oral contraceptives, and deficiency of protein S or C. It is not rare that BCS often shows venous thrombosis, including in superior vena cava. We performed a cardiac anesthesia for a 44 year old male with BCS and total superior vena cava syndrome (SVCS) due to the hereditary protein S and C deficiency. Surgical relief of the hepatic outflow stenosis was performed during deep hypothermic circulatory arrest. The patient was managed successfully without conventional intraoperative hemodynamic monitoring such as central venous catheterization, pulmonary artery catheterization, or transesophageal echocardiography due to underlying SVCS and the risk of varix bleeding. After weaning of cardiopulmonary bypass, mild acidosis and hypoxia improved slowly in an intensive care unit. Hypercoagulability was controlled by warfarin during the first postoperative day.

Keyword

Budd-Chiari syndrome; Deep hypothermic circulatory arrest; Superior vena cava syndrome

MeSH Terms

Acidosis
Anesthesia
Anoxia
Budd-Chiari Syndrome
Cardiopulmonary Bypass
Catheterization, Central Venous
Catheterization, Swan-Ganz
Central Venous Catheters
Circulatory Arrest, Deep Hypothermia Induced
Constriction, Pathologic
Contraceptives, Oral
Echocardiography, Transesophageal
Heart
Hemodynamics
Hemorrhage
Humans
Intensive Care Units
Liver Cirrhosis
Male
Protein S
Superior Vena Cava Syndrome
Thoracic Surgery
Thrombophilia
Varicose Veins
Vena Cava, Superior
Venous Thrombosis
Warfarin
Weaning
Contraceptives, Oral
Protein S
Warfarin