30% pasien dengan obstruksi vena hepatica. Kondisi ini memerlukan tindakan angioplasty atau pemasangan stent untuk menjaga patensi. Pada pasien-pasien yang tidak responsif terhadap terapi antikoagulan dan juga bukan merupakan kandidat untuk angioplasty atau pemasangan stent, dapat dipikirkan untuk dilakukan tindakan transjugular intrahepatic portosystemic shunt (TIPS) atau orthotopic liver transplantation (OLT).6 Etiologi dari BCS • Proteins C and S are two vitamin K-dependent plasma proteins that work in concert as a natural anticoagulant system. Activated protein C is the proteolytic component of the complex and protein S serves as an activated protein C binding protein that is essential for assembly of the anticoagulant complex on cell surfaces. The anticoagulant activity is expressed through the selective inactivation of Factors Va and VIIIa. Many patients deficient in proteins C and S have been described and have an associated thrombotic tendency, but not all heterozygous protein C and S deficient individuals experience thrombotic complications. Multiple mechanisms and/or drugs can lead to acquired deficiencies of these proteins: oral anticoagulation, liver disease, DIC and in the case of protein S, lupus erythematosus, nephrotic syndrome, pregnancy and certain hormones. The anticoagulant activity of protein C decreases rapidly after administration of warfarin (i.e., with a time course similar to Factor VII). This rapid decrease may lead to a transient imbalance and contribute to coumarin induced skin necrosis. Protein S antigen levels do not decrease as rapidly, but protein S functional levels are often low in patients with an acute thrombus. The discrepancy between antigen and function results from elevations in C4b-binding protein, which complexes reversibly with protein S. Unlike free protein S, the complex does not function in the anticoagulant pathway. The available information all suggest that deficiency of protein C and protein S should be considered a risk factor contributing to recurrent thrombotic disease and that the function of these proteins is altered by many common clinical conditions which have associated an increased risk of thrombosis. • Collateral pathways in Budd Chiari syndrome: multiple collateral path ways have been described in BCS. These could be intrahepatic or extra hepatic. 1) Intrahepatic collaterals: Two forms of intrahepatic collaterals are seen Those that drain into the systemic veins through subcapsular venous plexus and Those that shunt the segment of occluded vein into the nonoccluded venous segment and are seen as “comma” shaped veno-venous collaterals. These are best seen on sonography [4] (Figure 1). 2) Left renal hemiazygous pathway Left renal vein has complex anatomy due to the fact that many veins like inferior phrenic, gonadal and adrenal veins drain into it. Left renal vein communicates with the retroperitoneal veins namely lumbar, ascending lumbar and hemiazygous vein. These communications ultimately reach into the right atrium. This drainage pathway can form the major drainage pathway in the cases of BCS [5] (Figure 2). 3) Vertebrolumbar azygous pathway Flow in the IVC could reverse and reach common iliac vein and then into the ascending lumbar veins thereby draining into the azygous vein. Ascending lumbar veins are seen parallel to the spine and poster lateral to the aorta and IVC. Reversal of flow could be seen in the gonadal or ureteric veins towards the common iliac vein and then into the azygous system [2]. It is the most common collateral pathway in BCS (Figure 3). 4) Anterior abdominal wall collaterals • The collaterals comprising this system include one between the superior and inferior epigastric veins and other between the superficial epigastric and lateral thoracic veins. • Inferior epigastric vein arises from the external iliac vein joins superior epigastric vein above the level of umbilicus to drain into the subclavian vein and ultimately right heart. • Flow may also follow the common femoral vein through superficial circumflex iliac vein to superficial epigastric vein and anastomosing with the lateral thoracic vein. • Collaterals comprising the superior and inferior epigastric communications are seen medially in contrast to the posterolaterally situated superficial epigastric and lateral thoracic veins. The laterally situated superficial epigastric veins are seen clinically as caput medusa [6] (Figure 4). 5) Inferior phrenic pericardio- phrenic collaterals • Pericardiophrenic vein is a tributary of the left brachiocephalic vein and has communications with inferior phrenic vein. Inferior phrenic vein on right side drains into the IVC while on left side can drain into the IVC and into the left renal vein. Left hepatic vein and left phrenic vein have ostia close to each other. Sometimes left phrenic vein can drain into the left hepatic vein. This pathway drains the intrahepatic collaterals into the phrenic vein and then into the pericardiophrenic vein (Figure 5). Myeloproliferative Neoplasms • (1) Polisitemia vera, • (2) trombositopenia esensial, • (3) Chronic myelomonocytic leukaemia (CMML), • (4) Chronic neutrophilic leukaemia, • (5) Chronic eosinophilic leukaemia, • (6) Idiopathic myelofibrosis Tabel 3. Well’s score Interpretasi: Skor ≥2 adalah DVT likely
Kriteria klinis Skor Pada pasien
Active cancer (any treatment within past 6 months) +1 0
Calf swelling where affected calf circumference measures >3 cm more than the other calf +1 +1 (measured 10 cm below tibial tuberosity) Prominent superficial veins (non-varicose) +1 0 Pitting oedema (confined to symptomatic leg) +1 +1 Swelling of entire leg +1 +1 Localised pain along distribution of deep venous system +1 0 Paralysis, paresis, or recent cast immobilisation of lower extremities +1 0 Recent bed rest for >3 days or major surgery requiring regional or general anaesthetic within past +1 +1 12 weeks Previous history of DVT or PE +1 0 Alternative diagnosis at least as probable -2 0 What about CVP? • (MAP – CVP) = CO x SVR
• Since CVP is usually much less than MAP, we may be able to ignore or estimate the CVP value and still get clinically useful estimates for SVR.