Transdiaphragmatic cavoatrial anastomosis during orthotopic liver transplantation in a patient with chronic Budd-Chiari syndrome and thrombosis of inferior vena cava proximal to the right atrium - case report and review of literature

Introduction: Budd-Chiari syndrome associated with inferior vena cava (IVC) thrombosis extended to the right atrium is a rare condition, which requires liver transplantation with different techniques of caval reconstruction. The procedure is technically demanding, especially if it is performed solely transdiaphragmatically. Case presentation: An orthotopic liver transplantation (OLT) was performed in April 2014 in a 24-year old male patient with Budd-Chiari syndrome due to idiopathic thrombophilia and complete thrombosis of the retrohepatic IVC, up to its intrapericardic portion. Due to the impossibility of performing a cavo-caval anastomosis, a transdiaphragmatic anastomosis between the recipient right atrium and the donor superior cuff of the IVC was performed, without sternotomy. Results: Postoperative course was uneventful except for persitent ascites, which was successfully managed with diuretics. The patient was discharged after 34 days; 9 months postoperatively he has optimal liver function and has returned to normal life Conclusion: A transdiaphragmatic cavo-atrial anastomosis represents a feasible option in the setting of OLT, when the recipient IVC cannot be used due to extensive thrombosis, especially if it is performed by a multidisciplinary operative team.

[1]  G. Han,et al.  Selection of treatment modalities for Budd-Chiari Syndrome in China: a preliminary survey of published literature. , 2014, World journal of gastroenterology.

[2]  Yan Wu,et al.  Staged management of Budd-Chiari syndrome caused by co-obstruction of the inferior vena cava and main hepatic veins. , 2013, Hepatobiliary & pancreatic diseases international : HBPD INT.

[3]  H. Romdhane,et al.  The Budd-Chiari syndrome. , 2013, La Tunisie medicale.

[4]  Hao Xu,et al.  Clinical features and etiology of Budd–Chiari syndrome in Chinese patients: A single‐center study , 2013, Journal of gastroenterology and hepatology.

[5]  A. Mancuso,et al.  A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction. , 2013, World journal of hepatology.

[6]  D. Valla,et al.  Good long‐term outcome of Budd‐Chiari syndrome with a step‐wise management , 2013, Hepatology.

[7]  RUIHUA WANG,et al.  Treatment of Budd-Chiari syndrome with inferior vena cava thrombosis , 2013, Experimental and therapeutic medicine.

[8]  H. Grønbæk,et al.  Treatment of Budd-Chiari syndrome with a focus on transjugular intrahepatic portosystemic shunt. , 2013, World journal of hepatology.

[9]  K. Zieniewicz,et al.  Results of liver transplantation in the treatment of Budd-Chiari syndrome. , 2012, Annals of transplantation.

[10]  J. Isenberg,et al.  Budd–Chiari Syndrome Revisited: 38 Years’ Experience with Surgical Portal Decompression , 2012, Journal of Gastrointestinal Surgery.

[11]  W. Lau,et al.  Superior mesenteric vein-caval-right atrium Y shunt for treatment of Budd-Chiari syndrome with obstruction to the inferior vena cava and the hepatic veins--a study of 62 patients. , 2011, The Journal of surgical research.

[12]  Hongqiang Chen,et al.  Long-term follow-up study and comparison of meso-atrial shunts and meso-cavo-atrial shunts for treatment of combined Budd-Chiari syndrome. , 2011, The Journal of surgical research.

[13]  T. Kanematsu,et al.  Living donor liver transplantation with extensive caval thrombectomy for acute-on-chronic Budd-Chiari syndrome , 2011, Surgery Today.

[14]  Jiayin Yang,et al.  Transdiaphragmatic exposure for direct atrioatrial anastomosis in liver transplantation. , 2010, Chinese medical journal.

[15]  J. Joh,et al.  Living donor liver transplantation in Budd-Chiari syndrome: a single-center experience. , 2010, Transplantation proceedings.

[16]  D. Valla,et al.  Impaired fibrinolysis as a risk factor for Budd-Chiari syndrome. , 2010, Blood.

[17]  F. Rosendaal,et al.  Etiology, management, and outcome of the Budd-Chiari syndrome. , 2009, Annals of internal medicine.

[18]  Shin Hwang,et al.  Toward 300 liver transplants a year , 2009, Surgery Today.

[19]  Y. Takeda,et al.  Utilization of autologous vein graft for replacement of the inferior vena cava in living‐donor liver transplantation for obliterative hepatocavopathy , 2007, Transplant international : official journal of the European Society for Organ Transplantation.

[20]  P. Thuluvath,et al.  Twenty years of liver transplantation for Budd‐Chiari syndrome: A national registry analysis , 2007, Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society.

[21]  N. Heaton,et al.  Liver transplantation with caval thrombectomy and cavoatrial shunt in acute Budd‐Chiari syndrome , 2007, Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society.

[22]  V. de Lédinghen,et al.  Aiming at minimal invasiveness as a therapeutic strategy for Budd‐Chiari syndrome , 2006, Hepatology.

[23]  S. Uemoto,et al.  Surgical Techniques and Long‐Term Outcomes of Living Donor Liver Transplantation for Budd‐Chiari Syndrome , 2006, American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons.

[24]  Lunan Yan,et al.  Living donor liver transplantation for Budd‐Chiari syndrome using cryopreserved vena cava graft in retrohepatic vena cava reconstruction , 2006, Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society.

[25]  S. Olliff,et al.  Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome , 2005, Gut.

[26]  A. Burroughs,et al.  Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres. , 2006, Journal of hepatology.

[27]  K. Zieniewicz,et al.  Early results of orthotopic liver transplantations using the technique of inferior vena cava anastomosis. , 2006, Transplantation proceedings.

[28]  P. Thuluvath,et al.  The Utility of TIPS in the Management of Budd-Chiari Syndrome , 2005, Annals of surgery.

[29]  Zhong Gao Wang,et al.  Evolution of management for Budd–Chiari syndrome: a team's view from 2564 patients , 2005, ANZ journal of surgery.

[30]  P. Kamath,et al.  The Budd-Chiari syndrome. , 2004, The New England journal of medicine.

[31]  F. Rosendaal,et al.  Determinants of survival and the effect of portosystemic shunting in patients with Budd‐Chiari syndrome , 2004, Hepatology.

[32]  R. Dhiman,et al.  Treatment of Budd-Chiari syndrome with inferior vena caval occlusion by mesoatrial shunt. , 2003, The European journal of surgery = Acta chirurgica.

[33]  M. Orloff,et al.  A 27-Year Experience With Surgical Treatment of Budd-Chiari Syndrome , 2000, Annals of surgery.

[34]  Y. Tekant,et al.  Mesoatrial shunt in Budd-Chiari syndrome. , 2000, American journal of surgery.