Dr A. Cardenas, Division of Gastroenterology and Hepatology, 110 Francis Street, Suite 8E, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA. E-mail: email@example.com
Patients with cirrhosis and portal hypertension often have abnormal extracellular fluid volume regulation, resulting in accumulation of fluid as ascites, oedema or pleural effusion. These complications carry a poor prognosis with nearly half of the patients with ascites dying in the ensuing 2–3 years. In contrast to what happens in the abdominal cavity where large amounts of fluid (5–8 L) accumulate with the patient only experiencing only mild symptoms, in the thoracic cavity smaller amounts of fluid (1–2 L) cause severe symptoms such as shortness of breath, cough and hypoxaemia. Hepatic hydrothorax is defined as a pleural effusion, usually >500 mL, in patients with cirrhosis without cardiopulmonary disease. The pathophysiology involves the direct movement of ascitic fluid from the peritoneal cavity into the pleural space through diaphragmatic defects. The estimated prevalence among cirrhotic patients is 5–10%. The effusion, which is a transudate, most commonly occurs in the right hemithorax. The mainstay of therapy is similar to that of portal hypertensive ascites and includes sodium restriction and administration of diuretics. Refractory hydrothorax can be managed with transjugular intrahepatic portosystemic shunt in selected cases. Pleurodesis is not routinely recommended. Suitable patients with hepatic hydrothorax should be considered candidates for liver transplantation.
Hepatic hydrothorax is a relatively uncommon complication of end-stage liver with an estimated prevalence among cirrhotic patients of 5–10%.1–4 Despite numerous case reports describing clinical features and treatments for hepatic hydrothorax, the current knowledge of this complication of cirrhosis is limited. Unfortunately the pathogenesis and therapy of hepatic hydrothorax have not been appropriately studied and no large randomized-controlled trials establishing the best treatment options have been performed, therefore, proper guidelines on therapy based on good evidence have been difficult to establish. The aim of this review is to examine current concepts in the pathogenesis, clinical features and management of hepatic hydrothorax and provide recommendations for clinical practice based on published studies and our experience.
Definition and pathogenesis
Hepatic hydrothorax is defined as a pleural effusion (>500 mL) in patients with cirrhosis without coexisting cardiopulmonary diseases.1–4 It is a manifestation of decompensated chronic liver disease, similar to the advent of ascites, hepatic encephalopathy or variceal haemorrhage. The mechanisms involved in fluid retention in cirrhosis have been reviewed in detail elsewhere.5, 6 In brief, ascites formation involves the combination of portal hypertension and splanchnic arterial vasodilation. These two events modify the intestinal microcirculation and result in excess lymph formation relative to the absorptive capacity in the peritoneal cavity. Increased capillary pressure and permeability secondary to splanchnic arterial vasodilation is the predominant mechanism in this process.
Although the exact mechanisms involved in the development of hepatic hydrothorax have not been well-defined, several observations indicate that the most likely cause is passage of a large amount of ascites from the peritoneal to the pleural cavity through diaphragmatic defects.7–10Although there are no studies specifically addressing the exact area of leakage of fluid into the pleural cavity, this likely occurs because of the close anatomical relationship of bare areas of the liver with the diaphragm. Hepatic hydrothorax occurs when the accumulation of ascites in the pleural cavity surpasses the absorptive capacity of the pleura. Diaphragmatic defects represent small holes, usually <1 cm, in the tendinous portion of the diaphragm. These defects can be either microscopic or macroscopic. The negative intrathoracic pressure favours the transfer of fluid across these defects and hence patients usually have mild ascites.2, 4, 11 In most cases (85%) hepatic hydrothorax develops on the right side with 13% of cases occurring on the left side and 2% being bilateral.11, 12 Although diaphragmatic defects occur in the normal population and autopsy series report such defects in up to 20% of cases, they seem to only rarely result in pneumothorax following laparoscopic procedures.13–15 These defects may rupture, and therefore facilitate passage of fluid. This mechanism has been corroborated with nuclear medicine studies using [99Tcm]-human albumin or [99Tcm]-sulphur colloid and dye studies that show a unidirectional passage of these markers from the abdominal to the pleural cavity in the first 24 h after administration.16–18
Other proposed mechanisms, which are mostly speculative, include an increase in azygous vein pressure and flow leading to leakage of plasma,19 fluid traversing from the abdominal cavity to pleural space across transdiaphragmatic lymphatics,20 hypoalbuminaemia with decreased colloid osmotic pressure21 and leakage of the thoracic duct.22 Although not specifically addressed in hepatic hydrothorax, the rate of reabsorption of ascites back into the intravascular compartment could also play an important role in the pathogenesis of hepatic hydrothorax.23
Clinical features and diagnosis
The diagnosis of hepatic hydrothorax is usually suspected in a patient with advanced cirrhosis presenting with a unilateral pleural effusion, most commonly in the right side. Patients usually present with symptoms related to shortness of breath, cough, hypoxaemia and/or chest discomfort. Ascites is not always present. Several reports describe flow of [99Tcm]-sulphur colloid and radioisotopes after injection into the peritoneal cavity into the pleural cavity in the absence of overt ascites16, 24–26 Hydrothoraces that accumulate slowly and in debilitated patients with chronic liver disease can be relatively asymptomatic even when large. On rare occasions patients present with an acute tension hydrothorax, manifesting as severe dyspnoea and hypotension.27, 28
As in any patient with a significant pleural effusion, a diagnostic thoracentesis (approximately 40–50 mL of pleural fluid) should be performed for confirmation of diagnosis and to exclude alternate diagnoses. Diagnostic tests to be ordered include cell count, Gram stain and culture of the pleural fluid in blood culture bottles and serum and fluid protein, albumin, lactate dehydrogenase (LDH) and bilirubin.2, 11 Other tests in pleural fluid that would be appropriate depending on the clinical circumstance include triglyceride, pleural pH, adenosine deaminase and polymerase chain reaction (PCR) for mycobacterium, amylase, and cytology to exclude chylothorax, empyema, tuberculosis, pancreatitis, and malignancy respectively. The composition of pleural effusions deriving from portal hypertension are transudative in nature and therefore similar to the ascitic fluid, but some differences may be evident because of the different mechanisms of fluid absorption from the pleural space compared with those present in the peritoneal cavity.2
Total protein and albumin, may be slightly higher in hepatic hydrothorax compared with levels in the ascitic fluid.29 The characteristics and diagnostic tests and the interpretation of pleural fluid in hepatic hydrothorax are described in Table 1. In uncomplicated hepatic hydrothorax the cell count is <500 cells/mm,3 and the total protein concentration is <2.5 g/dL.2, 4 Although the measurement of the serum albumin – pleural gradient for determining a portal hypertension-related cause has not been studied in hepatic hydrothorax, in our experience the gradient is usually >1.1 g/dL, similar to that seen in cirrhotic ascites.6
Pleural fluid/serum lactate dehydrogenase (LDH) ratio (>0.6)
Pleural fluid/serum albumin gradient (>1.1)
Pleural fluid/serum bilirubin ratio (<0.6)
Glucose level similar to that of serum
As with ascites, infectious complications of hydrothoraces are associated with deteriorating prognosis and frequently few localizing signs. Spontaneous infection within the pleural fluid is known as spontaneous bacterial empyema (SBEM),30 although this term may be confusing because in most cases there is no evidence of pus or abscess in the thoracic cavity and some authors have proposed it be called spontaneous bacterial pleuritis.31 However, this name has not gained acceptance and most published studies referring to infections of the pleural fluid in cirrhotics use the term SBEM. Regardless of the name used, infection of the pleural fluid must be considered in any patient with hydrothorax who develops fever, pleuritic pain, encephalopathy or unexplained deterioration in renal function. Therefore, a high index of suspicion is essential for its diagnosis. SBEM is defined as pleural fluid with a polymorphonuclear (PMN) cell count >500 cells/mm3 or positive culture with PMN cell count >250 cells/mm3 with exclusion of a parapneumonic effusion.4, 30 In a study from Spain, Xiol et al. studied the clinical course of 120 cirrhotics with hydrothorax and reported that SBEM was present in as many as 13% on admission and had an associated mortality as high as 20% during treatment.30 The aetiology in most cases was due to Escheichia coli, Streptococcus species, Enterococcus, Klebsiella and Pseudomonas. In that study nearly 45% of episodes were not associated with spontaneous bacterial peritonitis (SBP).30 The risk factors for developing SBEM in patients with cirrhosis have only been addressed in one study by the same group in Spain.32 Sese et al. followed a cohort of patients with pleural effusions of different aetiologies; of these, 48 were cirrhotic patients with pleural effusions and 15 had SBEM on admission. The pleural fluid of cirrhotics had lower levels of total protein, complement, and opsonic activity when compared with other non-SBEM effusions. This study concluded that risk factors for developing SBEM include low levels of pleural fluid C3, low pleural fluid total protein, and a high Child-Pugh score.32
A computerized tomographic (CT) scan of the chest should be obtained to exclude mediastinal, pulmonary, or pleural lesions. In addition, detailed information of the diaphragm may be obtained with CT scan or magnetic resonance imaging, permitting recognition of the responsible small diaphragmatic defects.10 Thoracoscopy may also reveal the defects, but this procedure is invasive and carries significant morbidity in patients with advanced liver disease. Hence, it is rarely performed. If malignancy is strongly suspected despite negative cytology, thoracoscopy and pleural biopsy is indicated particularly if the patient is being considered for liver transplantation. Echocardiography is indicated if there is a suspicion of pericarditis or right heart failure. An abdominal ultrasound with Doppler study must be performed to examine the liver, rule out liver masses, and study the portal and hepatic veins and to determine the presence of ascites.
In cases where the diagnosis is uncertain, in particular when ascites is not detected or the hydrothorax is present on the left side; an intraperitoneal injection of [99Tcm]-sulphur colloid or [99Tcm]-human serum albumin may be helpful. With this test the radioisotopes migrate from the peritoneal cavity into the pleural space therefore establishing a communication between both spaces and confirming that the ascites is the source of the effusion.16, 17. Failure of the marker to show up in the pleural space indicates an alternate diagnosis for the pleural effusion.
The first and most important aspect in the management of all patients with cirrhosis and ascites is evaluation for candidacy for liver transplantation.6, 33 The main goal of treatment is relief of symptoms and prevention of pulmonary complications and infections until liver transplantation can be performed.
Sodium restriction and diuretics
Sodium restriction with diuretics is the first-line of therapy. A low sodium diet of 70–90 mmol/day is indicated and in general is well-tolerated. This restriction causes a negative sodium balance and loss of ascites and oedema in those patients with high urinary sodium excretion (>100 mmol/day as measured by a 24-h urine collection). In patients with marked sodium retention (urinary sodium excretion <10 mmol/day) such restriction is not sufficient by itself to achieve negative sodium balance, but it may slow further accumulation of fluid. These patients need diuretic therapy. The best initial regimen is the combination of furosemide 40 mg/day and spironolactone 100 mg/day.33 If there is no response, compliance with diet and medications should be addressed, and then diuretics may be increased in a stepwise fashion every 3–5 days by doubling the doses; spironolactone up to 400 mg/day and furosemide up to 160 mg/day. The goal is to achieve an average weight loss of 0.5 kg/day in patients without oedema and 1 kg/day in those with peripheral oedema.6, 34
Patients with persistent hydrothorax despite fluid and sodium restriction and use of maximal tolerable doses of diuretics are considered to have refractory hydrothorax.35 Although there is no data on how often this occurs, it is our experience that approximately 10% of patients either do not respond to diuretic therapy or develop diuretic-induced complications that prevent the use of high doses of these drugs. In these patients other options such as repeated thoracentesis, transjugular intrahepatic portosystemic shunt (TIPS), pleurodesis, and repair of defects in the diaphragm may be considered as a bridge to liver transplantation.35–40
In patients who develop SBEM, therapy with an intravenous third generation cephalosporin antibiotic such as ceftriaxone 1 g every 24 h for 7–10 days should be commenced immediately after the diagnosis is made.30 Given that patients that develop SBEM have approximately a 20% mortality during therapy30 and that a beneficial effect on mortality has been demonstrated with albumin infusion in the setting of SBP, we also use albumin therapy at 1.5 g/kg on day 1 and 1.0 g/kg on day 3 in the setting of SBEM,41 although albumin infusion has not been specifically studied in the setting of hepatic hydrothorax and SBEM. In cases where there is slow clinical recovery we recommend a repeat thoracentesis to document that the patient is responding to treatment.
Therapeutic thoracentesis is the best way of reducing a large effusion.2 It is a simple and effective procedure indicated for relief symptoms of dyspnoea in patients with large effusions (1.5–2 L) and those with reccurent or refractory hydrothorax. Contrary to what occurs in the peritoneal cavity where 2 L do not cause discomfort, this amount of fluid collection in the pleural cavity causes a significant amount of symptoms, such as shortness of breath, cough or chest pain and therefore these patients experience rapid relief of symptoms after thoracentesis. It is our experience that if these patients have adequate natriuresis (urine sodium >30 mmol/day) a therapeutic thoracentesis followed by diuretics leads to complete resolution of the effusion. However, in those patients with severe sodium retention (urine sodium <10 mmol/day) this measure may need to be repeated very often (about every 1–2 weeks).
Chest X-rays and CT scan of the chest help to define the size of the effusion. In general it is recommended that no more than 2 L be removed because there is a risk of re-expansion pulmonary oedema or hypotension.11, 42 Other complications of thoracentesis may include pain at puncture site, pneumothorax, empyema or soft tissues infection, vasovagal episodes, bleeding (haematoma, haemothorax, or haemoperitoneum), haemoptysis, air embolism, laceration of the liver or spleen, and subcutaneous empysema.11, 43 Coagulopathy of cirrhosis is not considered contraindication to therapeutic thoracenthesis unless there is disseminated intravascular coagulation. Nonetheless there are no guidelines regarding the administration of coagulation products. The incidence of significant bleeding or haemothorax has not been specifically addressed in any study. We recommend administration of fresh frozen plasma when the prothrombin time is prolonged more than 21 s, international normalized ratio (INR) >1.6, and platelet infusion when the platelet count is <50 000/mm.3 Given the relatively small volume of fluid removed at thoracentesis we do not administer intravenous albumin to avoid circulatory dysfunction unlike its routine use with large volume paracentesis.
Thoracentesis for hydrothorax in clinical practice is both useful and safe. However, thoracentesis dependence may be associated with deteriorating clinical status and impaired quality of life. When thoracentesis is required every 2–3 weeks alternative strategies need to be considered.
The TIPS is a non-surgical method of portal decompression, acting as a side-to-side portocaval shunt that reduces portal pressure and decreases the amount of ascites or pleural effusions in patients with hepatic hydrothorax.35–38 It is recommended for patients with hepatic hydrothorax who require repeated thoracentesis for control of symptoms.44 TIPS is probably the most effective option for refractory hepatic hydrothorax with response rates ranging from 70 to 80% in most studies.35–38 Although not specifically investigated in comparative studies, TIPS seems to be safer and less invasive and associated with less mortality and morbidity than surgical portosystemic shunts. The main disadvantage with TIPS is frequent obstruction of the prosthesis.35, 37, 38 In one study, 50% of patients with hepatic hydrothorax developed shunt insufficiency within 7 ± 9 months when treated with TIPS.37 Frequent obstruction precipitates rapid reaccumulation of effusions in some patients, however, newer polytetrafluoroethylene-covered prostheses improve TIPS patency and decrease the number of clinical relapses and re-interventions without increasing the risk of encephalopathy.45 Other major side-effects associated with TIPS include a 30–40% chance of hepatic encephalopathy, congestive heart failure, haemolytic anaemia and impairment in liver function.44
The TIPS for refractory hepatic hydrothorax has been reported to be effective, however, the experience with this procedure derives from relatively small case series. Initially reported in 1994, Strauss et al. treated five patients with advanced cirrhosis and documented symptomatic hepatic hydrothorax. Once TIPS patency was secured, all patients had resolution of hydrothorax or significant improvement as evidenced by reduced need for thoracentesis.36 In a larger series Gordon et al., treated 24 consecutive patients (five patients were Child-Pugh class B and 19 were Child-Pugh class C) with refractory hepatic hydrothorax.35 Patients were followed for a mean of 7.2 months (0.25–49 months). In 14 (58%) there was complete relief of symptoms with no need for additional thoracentesis, five additional patients required less frequent thoracentesis and in seven the Child's-Pugh score improved. Unfortunately in some, TIPS did not result in favourable outcome as hepatic encephalopathy developed in nine patients (37.5%) and five patients developed worsening liver function and died within 45 days of TIPS placement. During the study, seven patients that received liver transplantation had responded well with complete resolution in four and partial resolution in three. A small drawback of this study was lack of a control group of patients receiving the maximum tolerated dose of diuretics and a sodium restricted diet.
Siegerstetter et al. studied 40 patients (24 patients were Child-Pugh class B and 16 were Child-Pugh class C), with refractory hydrothorax requiring TIPS. Patients were followed for 16 ± 14 months.37 Seventeen patients were followed for at least 1 year and improvement in Child-Pugh score, serum albumin concentration and urinary sodium excretion were noted in all cases. Hydrothorax improved in 82% of patients and resolved in 71% of patients, however, two patients developed severe hepatic encephalopathy which required the shunt to be occluded. In these patients, shunt revision resulted in a secondary response rate of 82.3%. Although the survival rate at the end of 1 year was 64%, patients older than 60 years had a poor response and shortened survival. Unfortunately this study did not have a control group and not all patients were followed prospectively over a long period of time, therefore, it is difficult to interpret and translate these results of a small group of patients (n = 17), into clinical practice. Finally the safety and efficacy of TIPS was also evaluated by Spencer et al. in a group of 21 patients with refractory hepatic hydrothorax (seven, Child-Pugh B and 14, Child-Pugh C) that were followed for a mean of 223 days.38 Six patients died within 30 days of TIPS placement, two underwent liver transplantation within 30 days and 13 survived over 1 month. There was complete clinical response in 63%, partial response in 11% and no response in 26%. One of the troublesome aspects of this study was a high mortality (29%) within the first month of TIPS placement. Unfortunately these patients had severe coagulopathy, cardiac disease; requirements of ventilator and hepatic encephalopathy at the time of TIPS insertion; factors that in most centres would preclude a TIPS insertion.
The prognosis of patients receiving TIPS is related to the severity of the underlying liver disease. In fact, mortality rates are the same for patients with similar Child-Pugh scores irrespective of the indication for the procedure.46 Therefore, to better determine which patients are at risk of doing poorly after a TIPS two prognostic models have been proposed.46, 47 The most utilized model is that from which the model of end-stage liver disease (MELD) was created.47 In this model, INR, total serum bilirubin level, serum creatinine level, and aetiology of cirrhosis were used to predict survival following placement of a TIPS for any cause. The formula developed [3.8 loge (bilirubin mg/dL) + 11.2 loge (INR) + 9.6 loge (creatinine mg/dL) + 6.4 (aetiology: 0 if cholestatic or alcoholic and one otherwise)] was found to accurately predict survival (particularly at 3 months) after TIPS was placed.47 This prognostic index was modified and implemented in the United States as the MELD model to establish priority of patients awaiting liver transplantation.48 In the other model, variables including bilirubin level >3.0 mg/dL, alanine aminotransferase level >100 IU/L, pre-TIPS encephalopathy, and urgency of TIPS were independent predictors of survival.46 Neither of these two models has been specifically validated for patients with refractory hepatic hydrothorax.
In summary, 70–80% of patients with refractory hydrothorax have a good response with TIPS placement but serious adverse outcomes occur particularly in those who are most debilitated. Mortality of 20–25% can be expected in the first 45 days. Therefore, patient selection should be carefully selected avoiding patients over the age of 60, those with hepatic encephalopathy and/or those with Child C cirrhosis.
Pleurodesis is a technique that consists of the ablation of the space between the parietal and visceral pleura with a sclerosing agent injected by tube thoracostomy. Although some case reports have reported success, rapid fluid reaccumulation prevents the visceral and parietal pleural surfaces from approximating and adhering.49, 50 Milanez de Campos et al. performed thoracentesis followed by talc poudrage under thoracoscopy and repair of diaphragmatic defects if seen in 18 patients with persistent hepatic hydrothorax.51 A total of 21 procedures were performed and only in 10 (47.6%) the aerosolized talc was effective in preventing recurrence of the effusion. Patients were followed for 3 months. In the series, 43.7% cases recurred and in addition there were several complications noted following the procedure including fever, chest pain, empyema, incomplete re-expansion, pneumonia and wound infection. Diaphragmatic defects were localized and repaired in only five cases, and of these one developed an empyema and the other pneumonia and liver failure. Patients had a high morbidity (57.1%) and mortality (38.9%) during a follow-up period of 3 months making this option very unattractive for hepatic hydrothorax.51
A smaller study by Moroux et al. utilized video-assisted thoroscopy (VATS) to repair diaphragmatic defects in addition to pleurodesis in eight patients with refractory hepatic hydrothorax.8 Hydrothorax resolution occurred in six of eight patients that underwent repair. There was no recurrence of pleural effusion in any of the six cases that responded (mean follow-up, 7–36 months). Although encouraging, these results were obtained in a very small number of patients and therefore recommendations based on such studies are difficult to make. In a similar study, Ferrante et al. performed VATS and talc pleurodesis in 15 patients (six Child-Pugh class B and nine Child-Pugh class C) with refractory hepatic hydrothorax. Control of symptoms and resolution of effusion were achieved in 11 patients (73%) in the first 30 days after the procedure with eight patients remaining asymptomatic for a median follow-up of 5.5 months and three experiencing recurrence of the effusion between 45 and 60 days after the VATS.39 Unfortunately complications included pain around the chest tube site, low-grade fever with leucocytosis, pleurocutaneous fistula and empyema. There was no increased mortality observed between responders and non-responders. Finally, Takayama et al. reported a new technique of thoracoscopic pleurodesis using argon beam coagulation, fibrin glue and minocycline in nine patients with refractory hydrothorax. In this small study all patients showed clinical improvement with two recurrences.40
Although VATS with pleurodesis seems to be an encouraging alternative for patients with refractory hepatic hydrothorax, there are serious limitations to this therapeutic approach. First, the low number of patients treated in uncontrolled pilot studies does not permit adequate assessment of response. Secondly, the significant amount of complications and increased mortality are concerning. Thirdly, as with every technique, success depends on the operator. Although we do not recommend that patients undergo pleurodesis as a first line therapy for refractory hepatic hydrothorax, it may be a reasonable option for patients in who TIPS is contraindicated.
Chest tubes should not be placed under any circumstance as several complications such as subcutaneous empysema; lung, spleen, liver, and stomach lacerations; haemothorax resulting from intercostal artery laceration; unilateral pulmonary oedema from rapid removal of fluid; and placement of the tube into the abdominal cavity may occur. In addition massive protein and electrolyte depletion, infection, and bleeding have been reported.52, 53
Peritoneovenous shunts although very effective for refractory ascites, have not been specifically studied in refractory hepatic hydrothorax. Of note, these shunts were abandoned nearly a decade ago because of significant complications that caused increased mortality when compared with paracentesis in the setting of refractory ascites.54
Summary and treatment algorithm
Hepatic hydrothorax refers to a pleural effusion, usually >500 mL, present in a patient with cirrhosis without accompanying cardiac and/or pulmonary disease (Figure 1). The most accepted theory explaining its pathophysiology involves the direct passage of ascitic fluid from the peritoneal cavity into the pleural space through diaphragmatic defects. It occurs in approximately 5–10% of patients with advanced cirrhosis. The first step in the diagnostic work up a pleural effusion in a patient with cirrhosis is to perform a diagnostic thoracentesis (approximately 40–50 mL of pleural fluid) and to send the fluid for a cell count, Gram stain, and culture. In addition, determination of serum and pleural fluid protein, albumin, LDH, bilirubin should be performed. Other tests in pleural fluid may be indicated depending on the clinical circumstance.
We recommend that all patients with confirmed hepatic hydrothorax be referred for evaluation for liver transplantation. The first step in management is therapy with low sodium diet (70–90 mmol/day) and diuretics. Diuretic therapy should be commenced with furosemide 40 mg/day and spironolactone 100 mg/day. If there is no response, diuretics may be increased in a stepwise fashion every 3–5 days by doubling the doses (furosemide up to 160 mg/day and spironolactone up to 400 mg/day). The goal is to achieve an average weight loss of 0.5 kg/day in patients without oedema and 1 kg/day in those with peripheral oedema. With this measure, the majority of patients reduce their hydrothorax and ascites. If there is no response to diuretics, therapeutic thoracentesis of approximately 2 L can be attempted followed by diuretics. Patients that have refractory hydrothorax because of frequent re-accumulation of fluid or develop complications from diuretics that preclude their use should be considered for TIPS placement. This measure may help as a bridge to liver transplantation. For those patients that cannot undergo TIPS placement, consideration for pleurodesis or diaphragmatic repair by thoracoscopy should be considered. Chest tube placement should be avoided, as it is associated with severe complications.