Consensus report
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consensus report Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II) 13 1 3
N, N-bis(2-chloroethyl)-N-nitrosourea (BCNU) or multiple alkylating agents [ 94 ] (Table 2 ). As no estab- lished causal treatment exists, management should include prophylactic management strategies avoiding the precipitating factors. 3. BCS and portal vein thrombosis have similar patho- physiology where, in addition to local factors such as liver abscess and abdominal trauma, the acquired causes such as Behçet’s disease, hypereosinophilic syndrome, and granulomatous venulitis are risk fac- tors. Weak risk factors for BCS include factor V Leiden and prothrombin gene mutations and oral contracep- tive use (at least with earlier generation contracep- tives until 1985) [ 95 ].
cause or precipitating factor should be carried out in all patients with clinical manifestation of decompen- sated portal hypertension. Comprehensive guidelines [ 95 ], a systematic meta analy- sis [ 96 ] and authoritative reviews [ 97 – 99 ] have been pub- lished on vascular disorders of the liver, which allow us to give the following recommendations:
1. In all patients with first presentation or worsening of portal hypertension, duplex sonography of the por- tal vein and hepatic veins should be carried out to exclude portal vein thrombosis or Budd-Chiari syn- drome (III). 2. In patients with high clinical suspicion, contrast en- hanced CT, MRI, or angiography should be carried out despite normal ultrasound to exclude or confirm portal vein thrombosis or Budd-Chiari syndrome (III). 3. Acute portal vein thrombosis (with or without con- comitant intestinal infarction) should be considered in all patients with abdominal pain for more than 24 h (III).
4. Portal vein thrombosis and Budd-Chiari syndrome should prompt investigations for local prothrombotic conditions (infection, abscess, tumor, cysts, etc.) and systemic thrombophilic conditions listed in Table 1 .
hematocrit or negative testing for the JAK2 mutation p.Val617Phe are insufficient to exclude myeloprolif- erative disease (IIc). 5. Sinusoidal obstruction syndrome should be consid- ered in patients who received total body irradiation or drugs listed in Table 2 (typically patients being condi- tioned for marrow transplantation), who present with portal hypertension and hyperbilirubinemia (IIc). Management of noncirrhotic portal hypertension 1. Whenever possible, correct the underlying risk factor for venous thrombosis (III). 2. The possibility for correcting venous outflow in BCS by angioplasty or the possibility to increase portal flow in BCS or PVT by insertion of a TIPS should be evaluated by an experienced interventionist. TIPS im- proves transplant-free survival in BCS but not in SOS [ 95
3. Patients with PVT or BCS should receive anticoagula- tion therapy for at least 3 months unless liver trans- plantation is imminent. For patients with a permanent prothrombotic condition, lifelong anticoagulation is advisable. Low molecular weight heparins are initi- ated and shifted to oral anticoagulation after stabili- zation of the patient. In patients with high bleeding risk, therapeutic drug monitoring of LMW heparin is recommended (anti F Xa level 0.5–0.8 IU/ml). The tar- get INR for oral anticoagulation is 2–3 (IIb). 4. Patients with PVT or BCS should be screened for esophageal or gastric varices. Large varices should be managed endoscopically before long term anticoagu- lation is initiated (III). 5. Long term anticoagulation is recommended in pa- tients with an unknown cause or thrombus extension into the mesenteric vein (III). 6.
Liver transplantation should be considered in all patients with noncirrhotic portal hypertension and significant liver failure. This includes patients with recently diagnosed BCS and patients with SOS and a favorable hematological prognosis (III). Hepato-pulmonary vascular disorders Portopulmonary hypertension Definition Portopulmonary hypertension (PPHTN) is kind of pul- monary hypertension that is associated with portal hypertension. PPHTN is defined as [ 100 ,
]: 1. Underlying disease with portal hypertension 2. Mean pulmonary artery pressure (PAPm) > 25 mmHg 6-mercaptopurine 6-thiogunanine Actinomycin D Azathioprine Busulfan
Cytosine arabinoside Cyclophosphamide Dacarbazine Gemzutumab-ozogamicin Melphalan Oxaliplatn Urethane
14 Austrian consensus on the definition and treatment of portal hypertension and its complications (Billroth II) consensus report 1 3
3. Mean pulmonary artery occlusion pressure < 15 mmHg Pulmonary vascular resistance > 240 dyn×sec×cm −5 is
recommended additionally by the ERS Task Force Pul- monary-Hepatic Vascular Disorders [ 101 ].
2 ] Mild PAPm > 25 mmHg–< 35 mmHg Moderate PAPm ≥ 35 mmHg–45 mmHg Severe PAPm ≥ 45 mmHg PPHTN is a frequent cause of pulmonary hypertension [ 102 ]. Its prevalence in patients with cirrhosis that are listed for liver transplantation ranges between 2–16 % (II-2). PPHTN is the second most common cause of pul- monary-hepato-pulmonary vascular disorder in patients with cirrhosis [ 103
– 109
] (II-2). Diagnosis Symptoms of portal hypertension usually occur prior to symptoms of pulmonary hypertension (dyspnea, syn- cope, chest pain) [ 110
, 111
] (II-2). Patients with portal hypertension and dyspnea at rest and during exercise should be tested for presence of PPHTN. They should undergo transthoracic echocardiographic evaluation including contrast-enhanced echocardiography to rule out hepatopulmonary syndrome (III). Patients with estimated systolic pulmonary artery pressure > 40–50 mmHg or signs of abnormal right ventricular function via transthoracic echocardiogra- phy should undergo pulmonary artery catheterization, which is the gold standard for assessment of pulmonary hemodynamics. Preferably, this testing should be performed in addi- tion to hepatic hemodynamic assessment in the hepatic catheter laboratory. Also, pulmonary function testing including arterial blood gas analysis, thoracic CT scan, pulmonary angiography and/or ventilation–perfusion scan, assessment of autoantibodies (ANA, ANCA,…), and HIV status are recommended [ 100
, 101
]. Therapy
Several case series reported effects of medical therapy of PPHTN. Currently, there is a lack of randomized con- trolled studies. Patients that suffer from NYHA II dyspnea or higher despite optimized therapy of portal hyperten- sion, should receive medical therapy [ 101
] (II-2). This could include: • Prostaglandin-derivates (Epoprostenol, Iloprost,…) improved pulmonary hemodynamics and exercise ca- pacity [ 112
– 116
] (II-2). • Case series using endothelin receptor antagonists (Bosentan, Ambrisentan) reported improved pulmo- nary hemodynamics without adverse events on the hepatic function [ 117
– 120
] (II-2). • Sildenafil improved pulmonary hemodynamics in a small uncontrolled cohort [ 118
] (II-2). • Long term oxygen therapy (LTOT) is recommended in patients with PaO 2 < 60 mmHg [ 101 ] (III).
• Beta-blocking agents have deleterious effects on he- modynamics and exercise capacity in patients with PPHTN and should be strictly avoided [ 121
] (II-2). • Calcium channel blockers are not recommended as they may increase HVPG [ 101
] (III). • Systemic anticoagulation cannot be recommended for all patients because of the lack of data and because it may increase the risk of bleeding. Administration should be performed after individualized evaluation of the risk-benefit ratio [ 101 ] (III).
• TIPS is not recommended in PPHTN as it may increase the risk of right heart decompensation [ 101 ] (III).
• Liver transplantation is an effective treatment option of PPHTN. However, patients must be stratified ac- cording to severity of PPHTN [ 101
] (II-2): 1. Patients with mild PPHTN (PAPm < 35 mmHg, good cardiac function) should proceed to liver transplantation. 2. Patients with moderate PPHTN (PAPm ≥ 35 mmHg– 45 mmHg, good cardiac function) should receive vasodilator therapy prior to liver transplantation. 3. Liver transplantation is contraindicated in patients with severe PPHTN (PAPm ≥ 45 mmHg). These patients should be considered for medical therapy of PPHTN, only.
Definition Hepatopulmonary syndrome (HPS) is the most common hepatopulmonary vascular disorder in patients with liver cirrhosis with a prevalence of 20–30 % [ 122
, 123
] (II-2). It is defined as gas exchange abnormality as a consequence of liver disease due to intrapulmonary vascular dilatation and shunting [ 101 ].
1. Presence of liver disease. 2. Gas exchange abnormality (alveolar arterial oxygen tension difference (AaDO 2 ) ≥ 15 mmHg or AaDO 2
≥ 20 mmHg in patients for patients aged > 64 years, respectively. 3. Positive contrast-enhanced echocardiography (intra- pulmonary shunting is defined as detection of micro- bubbles in the left atrium 4–6 heartbeats after the ini- tial appearance in the right side of the heart).
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