Diagnosis Of Hepatopulmonary Syndrome Research Articles

Diagnostic Challenge of Hepatopulmonary Syndrome in a Patient with Coexisting Structural Heart Disease

Hepatopulmonary syndrome (HPS) is a severe complication seen in advance liver disease. Its prevalence among cirrhotic patients varies from 4–47 percent. HPS exact pathogenesis remains unknown. Patient presents with signs/symptoms of chronic liver disease, and dypsnea of variable severity. Our patient is a 62 years old white male with a known history of chronic hepatitis C, cirrhosis, ascites, and hypothyroidism who presented to GI/liver clinic complaining of 1 episode BRBPR, and exacerbating dypsnea associated with nausea and few episodes of non-bloody vomit. Physical exam showed, icterus, jaundice, few small spider angiomas on the chest, decrease breath sounds bilateral right more than left, and mild tachycardic. Abdominal exam revealed mid-line scar, moderated size ventral hernia, distention, diffused tenderness, and dullness to percussion. Laboratory result: CBC 5.2/13.2/37.6/83, LFTs 83/217/125/5.2/4.7/7.4, Pt 22.6 INR 1.9 PTT35.4. CT scan showed liver cirrhosis, abdominal varices, and moderated ascites collection around ventral hernia. Calculated A-a gradient was 49.5. Echocardiography revealed patent foramen ovale (PFO) with predominant left to right shunt. In our case, existence of paten foramen ovale (PFO) and atelectasis precludes definitive diagnosis of HPS. Presence of cardiopulmonary shunt could be partially responsible for the patient's dypsnea exacerbation.

Hepatopulmonary syndrome in children - is conventional liver transplantation always needed?

Hepatopulmonary syndrome (HPS) is the association of liver disease, hypoxemia, and intrapulmonary vascular dilatations. There are little data on the management of HPS in children other than conventional orthotopic liver transplantation (OLT). To describe the patient characteristics, mode of diagnosis, treatment, and outcomes of children with HPS at our center. Retrospective review of patients diagnosed with HPS between 1997 and 2007 after IRB approval. There were 10 patients, six females; median age at diagnosis of HPS was 12 yr. Six with cirrhosis underwent OLT and had subsequent resolution of HPS and are stable at last follow-up. Of the remaining four, two had cirrhosis. HPS resolved without conventional OLT in the following four patients: hepatitis C after antiviral treatment, biliary atresia with portal hypertension after transjugular intrahepatic portosystemic shunting, Abernethy syndrome after auxiliary partial OLT, and in a child with splenic vein thrombosis after splenectomy. Our series shows resolution of HPS in all patients and 100% survival after conventional OLT. Four children had resolution of HPS after surgical or medical treatments other than conventional OLT. Careful review of clinical status and underlying pathophysiology and anatomy at diagnosis of HPS should inform treatment decisions.

Saline Contrast Echocardiography in Patients With Hepatopulmonary Syndrome Awaiting Liver Transplantation

Patients with hepatopulmonary syndrome (HPS) with end-stage liver disease (ESLD) have higher cardiorespiratory mortality than those without. The aims of this study were to determine whether echocardiography could distinguish patients with ESLD with and without HPS and whether the diagnosis of HPS by contrast echocardiography (CE) was altered by the performance of the test in a supine or standing position. Subjects were recruited prospectively from patients with end-stage liver disease undergoing assessment for liver transplantation. Hepatopulmonary syndrome was diagnosed on the basis of arterial blood gas analysis, lung function testing and agitated saline contrast echocardiography in the absence of primary cardiac or pulmonary disease. Bubble contrast injections were performed supine or standing in a randomised order and read by a blinded observer. CE showed late right-to-left shunting in 13 of 50 consecutive patients with cirrhosis (26%). Eight patients (16%) had definite diagnoses of HPS. CE in the standing position consistently increased both the number and the size of shunts compared with supine injection. CE detected intrapulmonary shunting before a change in arterial blood gases. Standard echocardiographic parameters did not distinguish between those with and without HPS. This study suggests that screening for HPS in patients with advanced cirrhosis should be done using CE with patients in the upright position.

Prevalence of hepatopulmonary syndrome in patients with decompensated chronic liver disease and its impact on short-term survival

Hepatopulmonary syndrome is reported to occur in 4% to 32% of the patients with chronic liver disease and is associated with poor liver function and shortened patient survival before and after liver transplantation. To assess the frequency of hepatopulmonary syndrome in Brazilian patients with decompensated chronic liver disease and to investigate its impact on patient survival. One hundred and thirty patients (101 males, mean age 61 +/- 12 years) with decompensated chronic liver disease were evaluated for the presence of hepatopulmonary syndrome. The diagnosis of hepatopulmonary syndrome was considered in the presence of alveolar arterial oxygen gradient of more than 15 mm Hg and of pulmonary vascular dilatation assessed by contrast enhanced echocardiography. Hepatopulmonary syndrome was observed in 21 (16%) patients. The presence of hepatopulmonary syndrome was significantly associated with severity of liver disease assessed by the MELD (Model for End-Stage Liver Disease) score, but not with in hospital mortality after admission due to decompensated chronic liver disease. Hepatopulmonary syndrome occurs in 16% of patients with chronic liver disease and is associated with disease severity according to the MELD score. Short term mortality following decompensation of chronic liver disease was not associated with hepatopulmonary syndrome.

Monitoring preload during liver transplantation

Liver transplantation is associated with major shifts incentral blood volume; therefore, monitoring preload is es-sential. Filling pressures (central venous pressure or pul-monary artery occlusion pressure) do not reflect preload[right ventricular end-diastolic volume (RVEDV) or leftventricular end-diastolic volume (LVEDV)] well becausethese pressures are affected not just by preload but alsoby changes in compliance of the heart. These compliancechanges can be quite significant because of the changesin chest volume that are the result of surgical retractionand variable pressure on the diaphragm. Then, what arethe alternative methods of preload monitoring in the op-erating room? There are only two: thermodilution deter-mination of RVEDV and transesophageal echocardio-graphic estimation of RVEDV and LVEDV. Each of thesetechniques has advantages and disadvantages.The clinical determination of the right ventricularejection fraction (RVEF) and RVEDV became possible inthe early 1980s when American Edwards Laboratoriesmarketed a pulmonary artery catheter with a fast-re-sponse thermistor and an accompanying computer.That technology was based on intermittent injection ofa cold solution as a dye, and the principle of conserva-tion of energy allowed the calculation of RVEF and rightventricular volumes.

Hepatopulmonary syndrome, an unusual cause of hypoxemia

A six-year-old boy presented with cough, cyanosis and clubbing. Investigations revealed hypoxia (PaO2 53 mm Hg on room air) which was only partially correctable (PaO2 73 mm Hg) with 100% oxygen administered through a non re breathing face mask. Liver function tests showed elevated total bilirubin, and transaminases, liver biopsy confirmed chronic hepatitis and endoscopy showed grade three varices. A contrast enhanced echocardiography (bubble study) revealed pulmonary arterio-venous communication. A diagnosis of hepatopulmonary syndrome was made based on the triad of hypoxemia, liver disease and intra pulmonary vascular communications.

Therapy Insight: hepatopulmonary syndrome and orthotopic liver transplantation

Hepatopulmonary syndrome (HPS)--a pulmonary complication observed in patients who have chronic liver disease and/or portal hypertension--is attributed to intrapulmonary vascular dilatation and induces severe hypoxemia. HPS is mainly detected when patients are included on the waiting list for orthotopic liver transplantation (OLT) and can be diagnosed by blood gas analysis, transthoracic contrast-enhanced echocardiography or body scan with (99m)Tc-labeled macroaggregated albumin perfusion. When the partial pressure of arterial oxygen (PaO(2)) is >or=80 mmHg, it is unlikely that the patient has HPS. When the PaO(2) is <80 mmHg, imaging techniques should be used to confirm or exclude pulmonary vascular dilatation. When a diagnosis of HPS is confirmed, knowing the degree of hypoxemia is crucial for optimum patient management. Patients who have a PaO(2) >or=50 mmHg but <60 mmHg should be prioritized for OLT. This procedure is not indicated for patients with a PaO(2) between 60 mmHg and 80 mmHg, although follow-up every 3 months is recommended to detect any deterioration of the PaO(2). A PaO(2) of <50 mmHg might preclude OLT, because mortality and morbidity after OLT are greatly increased in these patients.

Left Atrial Volume: A Novel Predictor of Hepatopulmonary Syndrome

We studied patients with hepato-pulmonary syndrome (HPS). We found that HPS is frequently present in patients with left atrial enlargement. The aim of this prospective study was to evaluate the possible correlation between left atrial volume and HPS. Adult patients (>18 yr old) with biopsy proven liver cirrhosis who were referred for liver transplantation were enrolled in the study. Diagnosis of HPS was established when the following points were fulfilled: (a) the presence of chronic liver disease, (b) increased alveolar-arterial difference (AaDO(2)), (c) intrapulmonary vascular dilatation, and (d) absence of primary cardiac or pulmonary disease. We enrolled 41 patients (mean age 47.1 +/- 10.6 yr) diagnosed with HPS. Also 108 Child-Pugh score matched cirrhotic patients (mean age 49.2 +/- 9.3 yr) who have negative contrast echocardiography and normal age-related AaDO(2) were selected as a control group for the purpose of comparison of left atrial volume (LAV). LAV was significantly greater in patients with HPS compared to the control group (55.1 +/- 7.5 mL vs 37.1 +/- 9.3 mL, P < 0.05). The area under the receiver-operating characteristic (ROC) curve for LAV was 0.903 (Cut point >/= 50 mL, sensitivity 86.3%, specificity 81.2%). In the context of liver cirrhosis, LAV >/= 50 mL is a simple and feasible parameter to detect HPS.

OUTCOME OF HEPATOPULMONARY SYNDROME IN CHILDREN

Methods: We identified children with hepatopulmonary syndrome (HPS) from 1990 to 2004. Inclusion criteria: liver disease or portal hypertension, hypoxemia (PaO2 < 70 mmHg or O2 saturation < 95%) and intrapulmonary shunting documented by macroaggregated albumin (MAA) scan ratio of > 4%. Patients were classified into three grades based on MAA shunt ratio; mild group (< 20%), moderate group (20%-40%) and severe group (> 40%). Resolution of HPS post liver transplant (LT) was defined as PaO2>70 mmHg or O2 saturation>95%. Results: Eighteen children were diagnosed to have HPS, underlying liver disease being biliary atresia (n = 8), cryptogenic biliary cirrhosis (4), progressive intrahepatic cholestasis (2), cystic fibrosis, alpha 1 antitrypsin deficiency, portal vein thrombosis and congenital portosystemic shunt in 1. There were 12 girls (66%) with a median (range) age at diagnosis of HPS of 8.6 years (1-15.5 yeas). The degree of shunting was mild in 8, moderate in 5 and severe in 4. Fourteen patients underwent LT with resolution of HPS in 13. Two patients developed hepatic artery thrombosis and 4 developed biliary complications. Four children died (28%), two pre LT and two post LT. The one-year survival rate post LT was 93%. Mean PaO2 was significantly lower in non-survivors compared to survivors (43 vs 55.2 mmHg) (P = 0.03). O2 saturation < 78% was the strongest predictor of mortality (P = 0.05, LR = 5). There was significant correlation between oxygen parameters pre LT and the time to HPS resolution post LT. Conclusions: HPS is reversible after LT, but the severe form is associated with increased mortality and morbidity.

Pulmonale Komplikationen von Lebererkrankungen

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two distinct pulmonary complications of liver disease, which seem pathogenetically linked to the presence of portal hypertension. The most common presenting symptom of both syndromes is dyspnoea, but HPS is more prevalent in 5-30% of patients. The diagnosis of HPS requires the documentation of arterial hypoxemia and intrapulmonary vascular dilatation with anatomical shunting. In contrast, POPH has a prevalence of 2-16% and is only considered proven, if other causes of the pulmonary hypertension than the high portal pressure are excluded. Moderate to severe HPS or POPH carry a poor prognosis and liver transplantation remains the only curative treatment, although POPH in particular is associated with high perioperative mortality.

Hepatopulmonary syndrome: an emerging complication in HIV-hepatitis C virus co-infected patients

Following the availability of highly active antiretroviral therapy (HAART), chronic liver disease now represents the major cause of morbidity and mortality for HIV-infected patients [1,2]. Among the wide spectrum of end-stage liver disease complications, hepatopulmonary syndrome (HPS) has not been described. We report a fatal case of HPS in a woman with HIV and Child–Pugh B cirrhosis due to hepatitis C virus (HCV). A 40-year-old psychotic woman, who had been followed up for a HIV/HCV co-infection over a period of 10 years, presented at our institution complaining of dyspnoea for 2 weeks. She had interrupted her antiretroviral therapy a year earlier. Her CD4 cell count was 22 cells/μl and her HIV-RNA viral load was 88 000 copies/ml. At presentation, with a temperature of 37°C and a respiratory rate of 25/min, she was cyanotic with clubbing of the fingers. She had hepatomegaly and stigmata of portal hypertension (spider angiomas, splenomegaly). Fine crackles could be heard over both lungs. Analysis (on room air) of arterial blood gases withdrawn while the patient was in a supine position showed hypoxemia (arterial partial pressure of oxygen (PaO2) was 50 mmHg and the arterial partial pressure of carbon dioxide (PaCO2) was 40 mmHg). The arterial blood gases worsened upon standing (PaO2, 42 mmHg; PaCO2, 39.3 mmHg) suggesting orthodeoxia. Blood testing showed the following values: haemoglobin, 12.6 g/dl and platelets, 40 000/μl. The serum transaminases were elevated; serum conjugated bilirubin was 30 μmol/l (normal range, < 17 μmol/l); albumin was low with a value 19 g/l (normal range, 35–45 g/l); and the prothrombin time was 51% (normal range, 75–100%). The serum brain natriuretic peptide level was normal. Microscopic examination and culture of sputum and broncho-alveolar lavage were negative for Pneumocystis jirovecii and other opportunistic pathogens. Chest radiographs showed bilateral basilar reticulonodular opacities and normal lung volumes. Pulmonary computerized tomographic angiography with contrast revealed no evidence of pulmonary emboli. Pulmonary function testing demonstrated a reduced carbon monoxide diffusion capacity. The presence of delayed contrast appearance in the left heart on contrast-enhanced echocardiocardiography confirmed the diagnosis of HPS. Continuous home oxygen therapy (6 l/min) and a new antiretroviral therapy regimen were initiated. After initial improvement of the dyspnoea, she died of a urinary sepsis 3 months later. Hepatopulmonary syndrome is defined as a triad of chronic liver diseases, hypoxemia < 70 mmHg and pulmonary vascular dilation [3]. The estimated prevalence of HPS in patients with chronic liver disease varies between 4 and 29% according to the heterogeneous criteria used for definition of the condition [3,4]. In a recent study among patients with hepatitis B or C infection, the reported prevalence was 1.1% [5]. Little data is available on HIV-infected patients as only two cases have previously been reported in the literature [6,7]. The mechanisms leading to pulmonary vasodilation are complex and multifactorial. There appears to be an imbalance between the vasodilating and vasoconstricting mediators (nitrogen monoxide and endothelin-1). Hypoxemia can be explained by anatomical shunting and a diffusion–perfusion abnormality [3,4,6]. HPS occurs mainly but not exclusively in cirrhotic patients with portal hypertension [3,4]. This increasingly frequent complication is characterized by a delayed diagnosis with a mean duration of respiratory symptoms of 4.8 years before diagnosis. The presence of hypoxemia associated with platypnea and orthodeoxia (decrease in PaO2 > 3 mmHg in the erect position) as in our observation is suggestive of clinically significant HPS [3,4,6,7]. When the diagnosis is suspected, analyses of arterial blood gases should be obtained in both erect and supine positions. Attention in daily practice should be drawn to the presence of basilar interstitial infiltrates on chest radiographs, mimicking interstitial lung diseases or opportunistic pulmonary infections. Moreover, a high-resolution computerized tomography scan of the chest is useful to rule out other causes of hypoxemia and confirm the vascular etiology (dilated lung vessels) of the opacities [8]. Contrast-enhanced echocardiography is the key diagnostic tool [3,4]. HPS represents a significant mortality risk factor (overall mortality of 41% at 3 years after onset of dyspnoea) among cirrhotic patients, even after transplantation [9]. The most relevant prognosis factor is severe hypoxemia, with a baseline PaO2 < 50 mmHg associated with high mortality [10]. Currently, there is no effective medical treatment (methylene blue, inhaled NO, garlic, norfloxacin) for HPS [4,6,9,11]. Orthotopic liver transplantation (OLT), an emerging practice in selected HIV/HCV co-infected patients, is the only efficient treatment since it can reverse oxygenation abnormalities [11–13]. No data is available yet in this group. Thus, HPS should be considered in every case of a HIV patient with associated liver disease, presenting with an unexplained dyspnoea and hypoxemia. Early detection of ‘subclinical HPS’ by systematic arterial blood gas measurements in HIV-associated liver disease improves the prognosis.

Hepatopulmonary Syndrome in Patients With Hypoxic Hepatitis

The hepatopulmonary syndrome (HPS) is defined as the triad of liver disease, arterial deoxygenation, and widespread pulmonary vasodilatation. Hypoxic hepatitis, also known as ischemic hepatitis, is the leading cause of acute liver impairment in hospitals. It is unknown whether HPS occurs in hypoxic hepatitis. We assessed the prevalence and clinical consequences of HPS in patients with hypoxic hepatitis. Forty-four patients with hypoxic hepatitis were screened prospectively for HPS using established criteria: (1) presence of hepatic disease, (2) increased alveolar-arterial difference for the partial pressure of oxygen greater than the age-related threshold, and (3) intrapulmonary vasodilatation detected via contrast-enhanced echocardiography. Sixty-two critically ill patients with different cardiopulmonary diseases but without hepatic disease were screened for prevalence of intrapulmonary vasodilatation as a control group. Criteria of HPS were fulfilled in 18 patients with hypoxic hepatitis. HPS-positive patients had a significantly decreased partial pressure of arterial oxygen (P = .001) and partial pressure of arterial oxygen/fraction of inspired oxygen ratio (P = .034) at the time of diagnosis of HPS, a significant decreased area under the curve of the partial pressure of arterial oxygen/fraction of inspired oxygen ratio during the first 48 hours after diagnosis of hypoxic hepatitis (P = .009), and a significantly increased peak serum aspartate transaminase level (P = .028), compared with patients without HPS. Complete resolution of intrapulmonary vasodilatation was observed during follow-up evaluation. Contrast-enhanced echocardiography was negative for intrapulmonary vasodilatation in all 62 control patients. Intrapulmonary vasodilatation indicating HPS frequently occurs in patients with hypoxic hepatitis. It is reversible after normalization of the hepatic dysfunction. Clinicians should consider intrapulmonary vasodilatation and HPS in patients with hypoxic hepatitis.

Significance of Hypoxemia Screening in Candidates for Liver Transplantation: Our Experience

Hepatopulmonary syndrome (HPS) is recognized as one of the causes of hypoxemia in patients with chronic liver disease. This complication is responsible for increased mortality and increased perioperative risk in liver transplantation candidates. Recent data from the literature suggest extending the screening for HPS to all candidates for liver transplantation. The aim of this retrospective study was to evaluate the incidence of hypoxemia among a population of patients awaiting liver transplantation. Using pulse oximetry as a screening tool for hypoxemia, 39 of 198 patients (20%) were hypoxemic. The results of this study confirmed the importance of screening for hypoxemia among patients awaiting liver transplantation. In these patients, a more accurate evaluation of respiratory function should be performed to confirm or exclude the diagnosis of HPS.

Model for end-stage liver disease (MELD) exception for hepatopulmonary syndrome

Hepatopulmonary syndrome (HPS) occurs when intrapulmonary vascular dilatation leads to abnormal arterial gas exchange in the setting of liver disease and/or portal hypertension. Over the past 15 years, HPS has emerged as a relatively common complication of cirrhosis, occurring in 15 to 20% of cases, and is an indication for liver transplantation (LT) in patients with significant hypoxemia. However, many clinical features of HPS that may influence exception to the Model for EndStage Liver Disease (MELD) scoring system, including standardized diagnostic criteria, preand post-LT mortality rates, and rate of progression of hypoxemia, are not fully characterized. The diagnosis of HPS is based on the presence of hypoxemia on room air due to intrapulmonary vascular dilatation in the setting of liver disease and/or portal hypertension. The prevalence of HPS (range, 12 to 32%) varies depending on whether abnormalities in arterial gas exchange are defined by an abnormal alveolar-arterial oxygen gradient or arterial hypoxemia (PaO2). 2-7 For the purpose of considering HPS for MELD exception, arterial hypoxemia (PaO2 60 mm Hg) detected in the sitting position (to avoid effects of positional changes on PaO2) is a reasonable standard. In a large prospective study of 200 LT candidates, pulse oximetry was found to be a useful screening technique for hypoxemia in patients with cirrhosis. The use of a screening oximetry threshold of 96% as a trigger for obtaining an arterial blood gas would have detected all patients with a PaO2 60 mm Hg and resulted in screening of 14% of the cohort. A proposed screening algorithm is provided in Figure 1. The most sensitive and appropriate screening test to detect intrapulmonary vascular dilatation is microbubble transthoracic contrast echocardiography. Although this is a qualitative test, it can also be used to screen for pulmonary hypertension and frequently distinguishes between intracardiac and intrapulmonary shunting. Radionuclide scanning with Tc99m-macroaggregated albumin (MAA), although quantitative, should not be used as a screening test because the MAA scanning technique is not standardized across centers, it is less sensitive than transthoracic contrast echocardiography, and it cannot distinguish between intracardiac and intrapulmonary shunting. In addition, up to 20 to 30% of patients with HPS have comorbidities that may contribute to hypoxemia (i.e., ascites, hydrothorax, pneumonia, chronic obstructive pulmonary disease). Therefore, it is appropriate to review cardiopulmonary data (i.e., chest x-ray, pulmonary function tests, computed tomographic scan of the chest) in all patients being considered for MELD exception. The major diagnostic use of the MAA scan, if performed and interpreted by means of standardized methodology, is in defining the contribution of HPS to hypoxemia in patients with comorbidities and PaO2 60 mm Hg. 8 If the MAA scan is positive for increased shunting, HPS is likely an important component of hypoxemia. The natural history and outcomes before and after LT in patients with HPS are incompletely characterized. Two recent single-center studies suggest that mortality in well-characterized patients with cirrhosis not undergoing LT is significantly increased in those with HPS compared with those without HPS, and that the degree of hypoxemia and severity of liver disease adversely influence outcome. However, the numbers of patients are small, and in one study, a subset of patients with HPS did not undergo LT because of comorbidities

Evaluation of Intrapulmonary Vascular Dilatations With High-Resolution Computed Thorax Tomography in Patients With Hepatopulmonary Syndrome

We aimed to determine the role of thorax high-resolution computed tomography (HRCT) in demonstrating the pulmonary vasodilatation in patients with hepatopulmonary syndrome (HPS). Traditionally, the presence of intrapulmonary vascular dilatations can be detected by using one of the three diagnostic modalities: contrast-enhanced echocardiography, technetium 99 m-labeled macroaggregated albumin scan, and pulmonary angiography. The study group included 10 patients with HPS (Group 1), 12 patients with normoxemic cirrhosis (Group 2), and 12 healthy controls (Group 3). All of the subjects underwent conventional and HRCT of thorax. The diameters of pulmonary trunk, main pulmonary arteries, and right lower lobe basal segmental arteries were measured. The ratios of right lower lobe basal segmental pulmonary artery to bronchus diameter were calculated. The mean diameters of the main pulmonary trunk, right and left main pulmonary arteries were not different between the groups. Mean diameters of right lower lobe basal segmental pulmonary arteries were significantly higher in Group 1 compared with Group 2 (P=0.01) and Group 3 (P=0.002). Mean right lower lobe basal segmental pulmonary artery to bronchus ratios were significantly higher in Group 1 compared with Group 2 (P=0.03) and Group 3 (P<0.001). Group 2 had significantly higher pulmonary artery to bronchus ratios than Group 3 (P<0.001). Thorax HRCT may be helpful in the diagnosis of HPS by demonstrating the dilated peripheral pulmonary vessels or increased pulmonary artery to bronchus ratios in patients with liver disease and hypoxemia.

An unusual cause of dyspnoea complicating right upper abdominal swelling

A middle aged, non-addict male presented with right upper abdominal pain and swelling with respiratory distress. Examination revealed central cyanosis, bipedal pitting edema with prominent epigastric and back veins. Liver was enlarged, tender, spanned 20 cm without any splenomegaly or ascites. Other systems were clinically normal. Laboratory investigations showed polymorphonuclear leucocytosis with slightly deranged liver function. Abdominal ultrasonography showed an abscess in the right lobe of the liver with compressed inferior vena cava (IVC), middle and left hepatic veins. Arterial blood gas (ABG) documented hypoxia with orthodeoxia and air-contrast echocardiography was suggestive of an intrapulmonary shunt. A diagnosis of hepato-pulmonary syndrome (HPS) was made with near normal liver function secondary to amebic liver abscess. It reversed completely following successful treatment of the liver abscess.

Hepatopulmonary syndrome can show spontaneous resolution: Possible mechanism of portopulmonary hypertension overlap?

Hepatopulmonary syndrome (HPS) (hypoxaemia due to intrapulmonary vasodilation and a right-to-left shunt associated with liver disease) resolves after liver transplantation. The authors describe a case of spontaneous resolution of HPS prior to liver transplantation. This patient was diagnosed with HPS associated with extra-hepatic biliary atresia when she was 10 years old. She exhibited digital clubbing, facial vascular dilation, cyanosis, and suffered from dyspnoea during exercise. The patient's PaO(2) at rest was 53.8 mm Hg in room air and a Technetium-99m macro-aggregated albumin lung perfusion scan demonstrated a right-to-left shunt. Although her symptoms and laboratory data supported a diagnosis of HPS, she nevertheless showed spontaneous resolution within 2 years. When she was 14 years old, pulmonary hypertension was evident upon examination of her echocardiogram. HPS may be improved or masked by an accidental overlap with pulmonary hypertension in the terminal stage of liver disease.

Intrapulmonary shunting and hepatopulmonary syndrome

A57-year-old woman was referred for assessment of progressive dyspnoea, decreasing exercise tolerance and worsening bluish facial discolouration. She had congenital cirrhosis complicated by portal hypertension aged 4 years, for which she underwent a portacaval shunt. She was an exsmoker of 30 years. On examination, she was centrally cyanosed and clubbed. Her jugular venous pressure was not elevated, cardiac examination was normal and there were no focal chest signs. Spider naevi were noted, but no palpable organomegaly or ascites. Her oxygen saturation, however, was 88% breathing room air, which did not alter when recumbent or supine. A chest radiograph showed normal lung fields and vasculature, and no effusions. Renal and liver profile blood tests and international normalized ratio were normal. Arterial blood gas analysis revealed: pH 7.43 (normal 7.35–7.45), Pa∘2 7.21 kPa (normal &gt;10.6 kPa), PaC∘2 3.96 kPa (normal 4.6–6.0 kPa) breathing room air. Her AaPa∘2 was 7.8 kPa (predicted agecorrected AaPa∘2 2.10 kPa). Pa∘2 increased to 69.8 kPa breathing 100% inspired oxygen delivered using continuous positive airway pressure. Her calculated intrapulmonary shunt fraction was 7% (normal &lt;5%). Pulmonary function tests showed normal spirometry and preserved lung volumes, however, carbon monoxide diffusing capacity was only 38% predicted, adjusted for haemoglobin 17.8 g/dl. Transthoracic echocardiography demonstrated normal structure and function of all cardiac chambers. Contrast echocardiography established the presence of intrapulmonary shunting (Figure 1) and confirmed the diagnosis of hepatopulmonary syndrome. She was established on long-term oxygen therapy and is currently under consideration for a liver transplant.

Natural history of hepatopulmonary syndrome

Few data exist concerning survival after the diagnosis of hepatopulmonary syndrome (HPS). Although orthotopic liver transplantation (OLT) frequently results in complete resolution of HPS, the relationship between transplantation and survival has not been described. The study rationale was to describe long-term survival in patients with HPS. Data were derived from patients diagnosed with HPS at Mayo Clinic (n = 61) between 1985 and 2002, including those undergoing OLT (n = 24) and those who did not (n = 37). A case-control, Kaplan-Meier survival analysis between HPS patients and 77 patients without HPS matched for liver disease cause, model for end-stage liver disease (MELD), severity of liver disease by the Child classification, and age was described for OLT and non-OLT groups. Patients with HPS had a mean partial pressure of arterial oxygen (PaO(2)) decline of 5.2 + 2.3 mm Hg per year awaiting OLT. For HPS patients, despite similar baseline PaO(2), brain uptake of technetium macroaggregated albumin ((99m)TcMAA), or measures of hepatic dysfunction, 5-year survival associated with OLT was 76% versus 23% who did not undergo transplantation (P < .0001). Comparing those who did not undergo transplantation, HPS patients had worse 5-year survival than matched controls (P = .0003). However, reasons to deny OLT (comorbidity) in the setting of HPS may well have contributed to observed survival differences. Baseline PaO(2) </=50 mm Hg was associated with worse survival irrespective of the decision to perform OLT. In conclusion, hypoxemia of HPS is frequently progressive. As OLT outcome relates to pretransplantation PaO(2), additional MELD points should advance the priority for OLT in HPS.

P0377 HEPATOPULMONARY SYNDROME IN CHILDREN: A SINGLE CENTRE EXPERIENCE

Introduction: Hepatopulmonary syndrome (HPS) is a rare complication of chronic liver disease or portal hypertension. The clinical presentation is dyspnoea on exertion or overt cyanosis. In the past, this complication contraindicated liver transplantation (LT). However, good outcome with reversal of symptoms after LT has been described. Methods: Single centre experience of management and outcome of HPS in 12 children. Results: Twelve children were diagnosed to have HPS on macroaggregated albumin (MAA) lung scintigraphy over a 15 year period (1988–2003), underlying liver disease being biliary atresia (BA) in 6, progressive intrahepatic cholestasis in 2, cryptogenic cirrhosis, billiary cirrhosis, portal vein sclerosis and alpha 1 antitrypsin deficiency in 1 each. Biliary atresia splenic malformation (BASM) syndrome was present in 3. There were 8 girls (65%) with a median (range) age at diagnosis of 6.6 years (1.4–15.4 years). All patients had clinical evidence of portal hypertension with 4 having a history of gastrointestinal bleeding. The presenting symptoms were dyspnoea (7), cyanosis (9)and clubbing (10). All children with BA had cleared their jaundice. Median (range) oxygen saturation by pulse oxymetry was 90% (56–95%) with mild increase on supplemental facial oxygen. MAA scintigraphy revealed median pulmonary shunting of 22% (range 4–49%). The degree of shunting was mild (<20%) in 5, moderate (20–40%) in 4 and severe (>40%) in 3. Cardiac echography revealed normal function of the heart in all. All children were listed for LT. Six children required oxygen therapy prior to LT. One child with BASM and a moderate shunt died of sepsis while awaiting LT. One child is currently listed for LT. Ten children underwent LT 6–20 months (median 12 months) after diagnosis of HPS. Six children were extubated after 24 hours with the longest period of ventilation being 10 days. Median (range) oxygen requirement after LT was 21 days (1–45 days). Two children died 1 week and 3.2 years post LT of multi-organ failure and post transplant lymphoproliferative disorder respectively. Nine children are alive and well 5.25 years (range 1 month-12.8 years) after diagnosis of HPS. Conclusion: HPS is reversible after LT and does not appear to complicate the postoperative course. Female preponderance in our study group remains unexplained but may be an effect of female sex hormones on pulmonary circulation.