Thrombophilia in Budd-Chiari syndrome patient

A 27-years old female patient presented with a severe general condition of generalized edema and hypotension. Two months earlier computed tomography imaged thrombosis the inferior vena cava three hepatic veins, or Budd-Chiari syndrome (BCS). Additional findings were right common iliac vein renal veins bilaterally. Genetic testing proved congenital thrombophilia. Anticoagulation therapy did not affect thrombotic occlusions. In another cardiovascular center, an unsuccessful attempt for interventional treatment jugular approach was made. The admitted to our hospital further evaluation decision on strategy. Laboratory non-invasive imaging at admission rejected cirrhosis. An abdominal ultrasound scan demonstrated complete occlusion post portal hypertension. When all detected transjugular intrahepatic portosystemic shunt (TIPS) option. endovascular strategy undertaken revascularization achieved femoral as bridge TIPS procedure, since didn’t meet criteria liver transplantation. diagnosing disease, main contributors gastroenterologist, diagnostic specialist, hematologist, while multidisciplinary team included also cardiologist, angiologist. this case decisions played major role in building appropriate therapeutic systemic illnesses conditions which medical guidance does yet have clear guidelines.