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Congenital portosystemic shunts may result in the development of hepatopulmonary syndrome, typically presenting with progressive hypoxemia in later childhood. We describe a case of a 5-month-old male with heterotaxy with polysplenia presenting with new onset hypoxemia. Subsequent evaluation identified an extrahepatic portosystemic shunt arising from the confluence of the main portal and superior mesenteric veins draining into the left renal vein. To treat his hypoxemia and prevent future complications of shunting, the patient underwent a successful single-stage endovascular closure., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The hepatopulmonary syndrome (HPS) is characterized by arterial oxygenation defect induced by intrapulmonary vascular dilatations in the setting of liver disease. We report a 57-year-old woman with a history of liver cirrhosis presented with progressive cyanosis, exertional dyspnea and a dry cough. Oxyhemoglobin saturation was 88.5% on room air. Contrast transthoracic echocardiography (cTTE) and technetium-99m-macroaggregated albumin ( 99m Tc-MAA) scintigraphy showed an intrapulmonary shunting and confirmed HPS.

A 60-year-old lady with alcoholic liver disease developed central cyanosis and orthodeoxia. A technetium-99m macro-aggregated albumin lung perfusion scan and contrast echocardiogram were performed. A 13% right to left shunt was calculated from the macro-aggregated albumin scan. There were more bubbles in the left heart than the right at the end of the contrast echocardiogram. Hepatopulmonary syndrome was therefore diagnosed. The patient had a liver transplant five days after these investigations. Further discussion about hepatopulmonary syndrome will be provided. Normally, macro-aggregated albumin scans are performed in few centers, however as this was at the height of the coronavirus pandemic, the scan needed to be performed locally to reduce the chance of the patient getting coronavirus. Local radiographers were remotely instructed on conducting the macro-aggregated albumin scan by a larger center to provide a timely and important investigation in a logistically difficult scenario., Competing Interests: DISCLOSURES: Neither author has any interest to declare. No funding was received for this work., (Copyright Journal of Radiology Case Reports.)

Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on 99m Tc-macroaggregated albumin (MAA) lung perfusion scintigraphy ( 99m Tc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on 99m Tc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on 99m Tc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background and Aims: Long-term prospective data on hepatopulmonary syndrome (HPS) from a large number of patients, especially in Asian patients, are lacking. We evaluated the long-term prognosis of HPS and the development of acute-on-chronic liver failure (ACLF), and related factors., Methods: A total of 142 patients with cirrhosis who underwent saline-agitated contrast echocardiography for the diagnosis of HPS were enrolled and observed prospectively from 2014 to 2019., Results: A total of 59 patients (41%) were diagnosed with HPS (24 grade 1, 23 grade 2, 12 grade 3). Thirty-eight and 37 patients died in the HPS and non-HPS groups, respectively (p < 0.01). The 5-year survival rate was 47% in the HPS group and 62% in the non-HPS group. In the Cox proportional hazards model, HPS and Model for End-stage Liver Disease (MELD) score ≥ 18, and Child-Turcotte-Pugh (CTP) class B/C were significant risk factors for mortality after adjusting for other risk factors (HPS hazard ratio [HR] = 1.9, p = 0.01; MELD score ≥ 18 HR = 2.3, p < 0.01; CTP class B/C HR = 2.9, p < 0.01). Compared to that in non-HPS group, the HPS group had a significantly higher incidence of ACLF during follow-up (p < 0.01) and more frequently presented with lung involvement of ACLF (p = 0.03)., Conclusions: In the long-term follow-up cohort, patients with HPS showed poorer prognosis than that of patients without HPS. HPS was a risk factor for ACLF development independent of hepatic dysfunction, and lung involvement was significantly common than without ACLF., (© 2021. Asian Pacific Association for the Study of the Liver.)

Cardiac function impairment in the setting of hepatopulmonary syndrome (HPS) in patients with end stage liver disease remains an issue of debate. The current study evaluated possible correlations between HPS and biventricular systolic function in patients with decompensated cirrhosis. Consecutive liver transplantation candidates with stable decompensated cirrhosis were prospectively evaluated. HPS was defined as the presence of an elevated alveolar-arterial oxygen gradient and intrapulmonary vasodilatation, detected by contrast enhanced echocardiography. HPS severity was determined based on arterial blood oxygen pressure values, while shunt size was assessed with a semi-quantitative method. Demographic, clinical and laboratory parameters were also prospectively collected. In total, 130 patients (mean age 56.5, M/F: 94/36, MELD score 14.6 ± 5.6) were enrolled, of whom 45 (34.6%) fulfilled the criteria for HPS diagnosis (mild: 57.7%, moderate: 33.3%, severe 4.4% and very severe 4.4%). Significantly lower absolute left ventricular (LV) global longitudinal strain (GLS) values (- 21.6 ± 2.3 vs. - 22.6 ± 2.5%, p = 0.041) were measured in patients with HPS compared to cirrhotic patients without HPS, while there was no statistically significant difference regarding right ventricular GLS (- 22.1 ± 3.3 vs. - 23.2 ± 3.5%, p = 0.061) between the two groups. Lower LV ejection fraction values were also recorded in the HPS group (53.9 ± 3.5 vs. 56.3 ± 4.5%, p < 0.01). No other echocardiographic parameter was correlated to HPS. Intrapulmonary shunt grading was correlated to HPS classification (χ 2  = 19.8, p < 0.01), with lower arterial oxygen values being recorded in higher stages of intrapulmonary shunt. In patients with cirrhosis, the presence of HPS is associated with worse LV contractile performance., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.)

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%-47%. Chronic liver disease has been described in patients with hypothalamic-pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

A 64-year-old woman with a history of cirrhosis and progressive difficulty breathing underwent pulmonary ventilation/perfusion SPECT to evaluate possible pulmonary embolism. The images demonstrated multiple mismatched ventilation/perfusion defects in both lungs, suggesting pulmonary embolism. However, there was also Tc-MAA radioactivity in the brain and bilateral kidney, with a right-to-left shunting rate of 8.8%. In addition, CT pulmonary angiography did not demonstrate embolus. The findings indicated that perfusion defects were caused by hepatopulmonary syndrome.

Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis., Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS., Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05)., Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09)., Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Background: Abernethy malformation is an extremely rare congenital malformation characterised by an extrahepatic portosystemic shunt. Children with Abernathy malformation can develop hepatopulmonary syndrome (HPS) with pulmonary arteriovenous fistulas (PAVF) or pulmonary hypertension. PAVF manifests as central cyanosis with effort intolerance. We report a case of PAVF in a Ten-year-old Boy. Persistent symptoms identified Abernathy malformation as the cause of progressive symptoms and current understanding of this rare malformation is reviewed., Case Presentation: A case of 10-year-old boy with Abernethy malformation complicated with HPS initially managed as PAVF was presented. Selective lung angiography showed a typical diffuse reticular pattern on right lower lung, which suggested PAVF. However, cyanosis was not improved post transcatheter coil embolization. Then, liver disease was considered although the patient had normal aspartate aminotransferase and alanine aminotransferase. The significantly elevated serum ammonia was attracted our attention. Abdominal computed tomography also exhibited enlarged main portal vein (MPV), cirsoid spleen vein, and superior mesenteric vein (SMV). Angiography with direct opacification of the SMV with a catheter coming from the inferior vena cava (IVC) and going to the SMV via the shunt vessel (SHUNT) between the MPV and IVC. Occlusion the IVC with an inflated balloon, injection of contrast medium via a catheter placed in the SMV, MPV was showed and absence of intrahepatic branches. Abernethy malformation IB type is finally confirmed., Conclusions: Abernethy malformation is an unusual cause for development of PAVF and cyanosis in children. Clinicians must be suspicious of Abernethy malformation complicated with HPS. If patients have abnormal serum ammonia and enlarged MPV in abdominal CT, cathether angiography should be done to rule out Abernethy malformation.

Purpose: To evaluate effect of transjugular intrahepatic portosystemic shunt (TIPS) creation on pulmonary gas exchange in patients with hepatopulmonary syndrome (HPS)., Materials and Methods: All patients with cirrhosis or Budd-Chiari syndrome undergoing elective TIPS creation at a single institution between June 2014 and June 2015 were eligible for inclusion. Twenty-three patients with HPS (age 55.0 y ± 14.4; 11 men; Model for End-Stage Liver Disease score 10.2 ± 2.7) who achieved technical success were included in the analysis. Diagnosis of HPS was established by contrast-enhanced echocardiography demonstrating intrapulmonary vascular dilatation and arterial blood gas analysis demonstrating arterial oxygenation defects., Results: Mean portosystemic gradient was reduced from 21.7 mm Hg ± 8.3 before TIPS creation to 10.8 mm Hg ± 5.1 after TIPS creation. Among the 5 (21.7%) patients who experienced dyspnea, 4 (80.0%) reported improvement after TIPS creation. This improvement was not maintained at 3 months after TIPS creation in 2 (50.0%) patients. Compared with before TIPS creation, mean change in alveolar-arterial oxygen gradient for patients with HPS was statistically significant at 1 month (-9.2 mm Hg ± 8.0; P < .001) after TIPS creation, but not at 2-3 days (-0.9 mm Hg ± 10.5; P = .678) or 3 months (-3.4 mm Hg ± 11.8; P = .179) after TIPS creation., Conclusions: TIPS creation can transiently improve pulmonary gas exchange in patients with HPS., (Copyright © 2018 SIR. Published by Elsevier Inc. All rights reserved.)

Background: Hepatopulmonary syndrome (HPS) is defined as a triad characterized by arterial deoxygenation, intrapulmonary vascular dilatations (IPVDs), and liver disorder. The aims of this study were to assess the prevalence of HPS in children with cirrhosis, the clinical characteristics of patients with HPS, and the tests used for the diagnosis of IPVD., Patients and Methods: This was a prospective, cross-sectional study of 40 children with cirrhosis (median age: 44 months). Investigations of HPS included arterial blood gas analysis, contrast-enhanced transthoracic echocardiography (CE-TTE), and perfusion lung scanning using technetium-99m-labeled macroaggregated albumin (Tc-MMA). Patients' clinical characteristics (age, etiology of cirrhosis, and severity of hepatopathy) were assessed. HPS was defined as liver disease; alveolar-arterial oxygen gradient of at least 15 mmHg and/or partial pressure of arterial oxygen less than 80 mmHg; and detection of IPVD by CE-TTE or Tc-MMA scanning. Statistical significance was indicated by a P value less than 0.05., Results: The prevalence of HPS was 42.5% (17/40). Eight patients had moderate HPS (47%) and two patients had severe HPS (12%). In bivariate analysis, biliary atresia (P=0.033) and median age (10 months; P=0.005) were associated with HPS. In multivariate analysis, only age remained statistically significant (prevalence ratio=0.99; 95% confidence interval=0.98-0.99; P=0.010). Sixteen patients with HPS had IPVD detected by CE-TTE (94.1%) and six patients had IPVD detected by Tc-MMA scanning (35.3%), with no significant agreement between these methods (κ=-0.12; P=0.163)., Conclusion: HPS is a common complication of cirrhosis in children. A combination of clinical and imaging criteria should be used to diagnose HPS.

We report an unusual case of severe hepatopulmonary syndrome with previously unrecognized cirrhosis, presenting with acute on chronic dyspnoea, extreme hypoxemia, secondary polycythemia as well as direct identification of arteriovenous communications on computed tomography angiography. Hepatopulmonary syndrome, defined as the combination of hepatopathy, arterial deoxygenation and pulmonary vascular dilatation, is increasingly recognized as a life-threatening complication in advanced liver disease and transplant candidacy. It is usually diagnosed in chronic liver disease patients following pre-transplant evaluation or mild dyspnea investigation. Diagnosis relies on the indirect evidence of pulmonary arteriovenous communications suggested by echocardiography with a bubble study. Clinicians need to be aware of this rare but potential acute presentation at the emergency room., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background & Aims: Pulmonary transit time (PTT) is the transit time of blood from the right side of the heart to the left side of the heart. The aim of the present study was to evaluate the role of the PTT derived from pulmonary angiography in the diagnosis of hepatopulmonary syndrome (HPS)., Methods: From December 2014 to September 2015, all patients with chronic liver disease and/or portal hypertension undergoing a venous interventional radiologic procedure at our institution were eligible for inclusion in this prospective study. Pulmonary angiography was performed in all patients, and the PTT, which was defined as the time between opacification of the pulmonary trunk and the right border of the left atrium, was determined., Results: A total of 53 patients were included, 20 of whom had a positive contrast-enhanced echocardiography result and an elevated alveolar-arterial oxygen gradient were considered to have HPS. PTT was significantly shorter in patients with HPS than in those without [median, 3.34 (interquartile range, 3.01-3.67) seconds vs 4.0 (interquartile range, 3.67-4.17) seconds; P < .001]. The area under the receiver operating characteristic curve of PTT for diagnosing HPS was 0.83 (95% confidence interval, 0.70-0.92). The optimal cut-off value of PTT for diagnosing HPS, based on Youden's index, was 3.55 seconds. The sensitivity, specificity and accuracy of PTT < 3.55 seconds for diagnosing HPS were 70%, 85% and 79% respectively., Conclusions: Pulmonary transit time derived from pulmonary angiography is useful for diagnosing HPS, especially for patients with intracardiac shunts and inadequate echocardiographic windows., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background and Aims: The aims of this study were to prospectively screen cirrhotic patients with arterial blood gas test and albumin perfusion scan, identify those fulfilling the classic hepatopulmonary syndrome (HPS) criteria, correlate with clinical parameters, and evaluate the survival of patients with HPS compared with those without HPS in a genetically homogenous Cretan cirrhotic population., Materials and Methods: Data on consecutive 102 patients within 1 year were collected and analyzed. All patients underwent a technetium 99m-macroaggregated albumin perfusion lung scan (Tc-MAA). Diagnosis of HPS was based on the presence of the quantitative index Tc-MAA≥6% and a [P(A-a)O2]≥15 mm Hg (≥20 mm Hg for patients over >64 y)., Results: In 94/102 patients, complete scintigraphic data were available. In total, 24 (26%) patients fulfilled the diagnostic criteria of HPS; 95.8% of them had mild-to-moderate HPS. In 8 patients the Tc-MAA scintigraphy could not be interpreted. There was no difference in HPS between decompensated (24.6%) and compensated cirrhosis (27.3%). In the multivariate analysis only the quantitative index was significant for the diagnosis of HPS (P=0.001, odds ratio; 95% confidence interval, 7.05; 2.27-21.87). Kaplan- Meier survival curves indicated a similar overall prognosis for patients diagnosed with HPS (P=0.105)., Conclusions: HPS is a frequent complication of cirrhosis. Mild-to-moderate HPS has no significant effect on survival of cirrhotic patients. The quantitative Tc-MAA test is a reliable tool for diagnosis.

Introduction: Different blood gas criteria have been used in the diagnosis of hepatopulmonary syndrome (HPS)., Patients and Methods: Arterial blood gases were prospectively evaluated in 194 cirrhotic candidates for liver transplantation (LT) in the supine and seated position. Three blood gas criteria were analyzed: classic (partial pressure of oxygen [PaO2] < 70 mmHg and/or alveolar-arterial gradient of oxygen [A-a PO2] ≥ 20 mmHg), modern (A-a PO2 ≥ 15 mmHg or ≥ 20 mmHg in patients over 64) and the A-a PO2 ≥ threshold value adjusted for age., Results: The prevalence of HPS in the supine and seated position was 27.8% and 23.2% (classic), 34% and 25.3% (modern) and 22.2% and 19% (adjusted for age), respectively. The proportion of severe and very severe cases increased in a seated position (11/49 [22.4%] vs 5/66 [7.6%], p = 0.02). No difference was observed in the pre-LT, post-LT and overall mortality in patients with HPS, regardless of the criteria used., Conclusion: Obtaining blood gas measurements in the supine position and the use of modern criteria are more sensitive for the diagnosis of HPS. Blood gas analysis with the patient seated detects a greater number of severe and very severe cases. The presence of HPS was not associated with an increase in mortality regardless of blood gas criterion used.

Despite the importance of haemodynamic management in patients undergoing liver transplantation, there is currently no consensus on the most appropriate type of monitoring to use. In this context, transoesophageal echocardiography can provide useful information to professionals, although their use constraints prevent further spread today., (Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: The natural history of intrapulmonary vascular dilations (IPVD) and their impact on patient outcomes in the setting of portal hypertension has only been described in small series., Aims: To assess the development of hepatopulmonary síndrome (HPS) in patients with isolated IPVD and to evaluate outcomes of IPVD and HPS among patients evaluated for liver transplantation (LT)., Material and Methods: Data from a prospective cohort of patients evaluated for LT with standardized screening for HPS were analyzed. IPVDs were defined as the presence of microbubbles in the left atrium > 3 cycles following right atrial opacification. HPS was defined as the presence of IPVD and hypoxemia (Alveolar-arterial gradient ≥ 15 mmHg) in the absence of concomitant cardiopulmonary disease., Results: A total of 104 patients with negative contrast-enhanced echocardiogram (CE) were compared to 63 patients with IPVD and 63 patients with HPS. Only four patients were categorized as ‘severe’ HPS based on degree of hipoxemia (defined as PaO2 < 60 mmHg). Twenty IPVD patients were followed with ABG over a mean duration of 21 months (range 9-43), of whom 7 (35%) subsequently met HPS criteria. Overall unadjusted survival from the time of LT evaluation using multi-state survival models that accounted for pre- and post-LT time was not statistically different among the three groups (negative CE, IPVD, and HPS; p > 0.5)., Conclusions: Patients with IPVD appear to have a substantial risk of developing oxygenation impairment over time and progress to HPS. In our cohort, survival in patients with HPS and isolated IPVD is not different when compared to those without IPVDs.

We present a case of a 54-year-old patient with cirrhosis, progressive dyspnea, and platypnea. Thoracic computed tomography (CT) showed multiple pulmonary arteriovenous malformations (PAVM), confirming the diagnosis of hepatopulmonary syndrome (HPS). Besides precisely identifying the number and location of PAVM, CT also demonstrated a striking mosaic pattern of the lung parenchyma, characterized by the presence of alternating geographic areas of low attenuation (showing pulmonary vessels with a decreased diameter) with regions of relatively increased attenuation (showing pulmonary vessels with a normal diameter). This mosaic pattern of the lung parenchyma has scarcely been described in patients with HPS since it is not always present and usually requires a post-processing of the CT images in order to increase the contrast between the low attenuation areas (representing hypoperfused regions) and the areas with a relatively increased attenuation (representing better perfused regions). The decision was made to embolize the major PAVM, achieving an improvement of both the oxygen partial pressure and the patient's symptoms. This improvement allowed the patient to become an acceptable candidate for liver transplantation. We believe that, unlike other radiological signs of HPS, the mosaic pattern has not been sufficiently described in the scientific literature. If the association of the mosaic pattern on CT with HPS is confirmed in larger studies, it could become a useful sign for detecting hypoperfused pulmonary areas related to small nonvisible PAVM.

Background: The macro-aggregated albumin lung perfusion scan (99mTc-MAA) is a diagnostic method for hepatopulmonary syndrome (HPS)., Goal: To determine the sensitivity of 99mTc-MAA in diagnosing HPS, to establish the utility of 99mTc-MAA in determining the influence of HPS on hypoxemia in patients with concomitant pulmonary disease and to determine the correlation between 99mTc-MAA values and other respiratory parameters., Methods: Data from 115 cirrhotic patients who were eligible for liver transplantation (LT) were prospectively analyzed. A transthoracic contrast echocardiography and 99mTc-MAA were performed in 85 patients, and 74 patients were diagnosed with HPS., Results: The overall sensitivity of 99mTc-MAA for the diagnosis of HPS was 18.9% (14/74) in all of the HPS cases and 66.7% (4/6) in the severe to very severe cases. In HPS patients who did not have lung disease, the degree of brain uptake of 99mTc-MAA was correlated with the alveolar-arterial oxygen gradient (A-a PO2) (r = 0.32, p < 0.05) and estimated oxygen shunt (r = 0.41, p < 0.05) and inversely correlated with partial pressure of arterial oxygen (PaO2) while breathing 100% O2 (r = -0.43, p < 0.05). The 99mTc-MAA was positive in 20.6% (7/36) of the patients with HPS and lung disease. The brain uptake of 99mTc-MAA was not associated with mortality and normalized in all cases six months after LT., Conclusions: The 99mTc-MAA is a low sensitivity test for the diagnosis of HPS that can be useful in patients who have concomitant lung disease and in severe to very severe cases of HPS. It was not related to mortality, and brain uptake normalized after LT.

This paper presents a case of a young patient with cyanosis and digital clubbing, until then an active, sporty person. He sought medical assistance due to the growing dyspnoea and the drop of effort tolerance. Initially the diagnostic process focused on the confirmation of the suspicion of pulmonary fibrosis or another interstitial lung disease as causes of the respiratory failure. Due to the atypical presentation of the symptoms, reaching the final diagnosis of digestive system disease with lung involvement required a more thorough multifaceted diagnostics of a number of systems and organs.

Clinical Introduction: A 60-year-old man had a long history of chronic hepatitis C. He presented to the emergency department with 2 days of progressive dyspnoea. Clinical manifestations included respiratory distress, cyanosis, digital clubbing, spider naevi on the upper chest and shifting dullness in the abdomen (see online supplementary figure S1). The ECG showed sinus tachycardia and left axis deviation. The chest radiography depicted blunting of right costophrenic angle with small pleural effusions and bilateral prominent pulmonary vascular markings (see online supplementary figure S2). The dyspnoea was exacerbated by upright posture and improved with recumbency. The hypoxaemia could not be corrected with administration of 100% oxygen. Contrast-enhanced transthoracic echocardiography was performed with injection of agitated saline (see figure 1 and online supplementary video). CT scan of the chest revealed some vascular abnormalities (see online supplementary figure S3-S6)., Question: Which of the following is the next best step in management?Catheter-based closure of intracardiac shuntLiver transplantationPulmonary angiography and embolisationThoracentesisTransjugular intrahepatic portosystemic shunt., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Hepatopulmonary syndrome (HPS) is characterized by abnormalities in blood oxygenation caused by the presence of intrapulmonary vascular dilations (IPVD) in the context of liver disease, generally at a cirrhotic stage. Knowledge about the subject is still only partial. The majority of the information about the etiopathogenesis of HPS has been obtained through experiments on animals. Reported prevalence in patients who are candidates for a liver transplantation (LT) varies between 4% and 32%, with a predominance of mild or moderate cases. Although it is generally asymptomatic it does have an impact on their quality of life and survival. The diagnosis requires taking an arterial blood gas sample of a seated patient with alveolar-arterial oxygen gradient (AaO2) ≥ 15 mm Hg, or ≥ 20 mm Hg in those over 64 years of age. The IPVD are identified through a transthoracic contrast echocardiography or a macroaggregated albumin lung perfusion scan ((99m)Tc-MAA). There is currently no effective medical treatment. LT has been shown to reverse the syndrome and improve survival rates, even in severe cases. Therefore the policy of prioritizing LT would appear to increase survival rates. This paper takes a critical and clinical look at the current understanding of HPS, as well as the controversies surrounding it and possible future research.

Background: Hepatopulmonary syndrome (HPS) is defined by liver dysfunction, intrapulmonary vascular dilatations, and impaired oxygenation. The gold standard for detection of intrapulmonary vascular dilatations in HPS is contrast echocardiography. However, two small studies have suggested that patients with HPS have larger segmental pulmonary arterial diameters than both normal subjects and normoxemic subjects with cirrhosis, when measured by CT. We sought to compare CT imaging-based pulmonary vasodilatation in patients with HPS, patients with liver dysfunction without HPS, and matching controls on CT imaging., Methods: We performed a retrospective cohort study at two quaternary care Canadian HPS centers. We analyzed CT thorax scans in 23 patients with HPS, 29 patients with liver dysfunction without HPS, and 52 gender- and age-matched controls. We measured the artery-bronchus ratios (ABRs) in upper and lower lung zones, calculated the "delta ABR" by subtracting the upper from the lower ABR, compared these measurements between groups, and correlated them with clinically relevant parameters (partial pressure of arterial oxygen, alveolar-arterial oxygen gradient, macroaggregated albumin shunt fraction, and diffusion capacity). We repeated measurements in patients with post-transplant CTs., Results: Patients had significantly larger lower zone ABRs and delta ABRs than controls (1.20 +/- 0.19 versus 0.98 +/- 0.10, p<0.01; and 0.12 +/- 0.17 versus -0.06 +/- 0.10, p<0.01, respectively). However, there were no significant differences between liver disease patients with and without HPS, nor any significant correlations between CT measurements and clinically relevant parameters. There were no significant changes in ABRs after liver transplantation (14 patients)., Conclusions: Basilar segmental artery-bronchus ratios are larger in patients with liver disease than in normal controls, but this vasodilatation is no more severe in patients with HPS. CT does not distinguish patients with HPS from those with uncomplicated liver disease.

Hepatopulmonary syndrome is one of the possible complications of chronic liver disease, defined clinically by impaired oxygenation. The underlying cause of the respiratory failure is the presence of intrapulmonary shunting, as a result of abnormal vascular dilatations in the lungs. We report the case of 52-year-old male, exsmoker, with a history of pulmonary TB and also of heavy drinking, who was admitted to the pulmonology ward for dyspnea at rest and limb cyanosis. His clinical exam was suggestive of liver cirrhosis, with signs of pneumonia, but also chronic lung disease. Variations in SaO2 with posture were noted: platypnea and orthodeoxia. Arterial gas assessment revealed severe hypoxemia, only partially corrected by high-flow oxygen therapy, while plethysmography showed only a mild obstructive syndrome, but with severely impaired alveolar-capillary diffusion. The suspicion of a hepatopulmonary syndrome was raised and a contrast echocardiography confirmed the diagnosis by revealing the presence of an intrapulmonary shunt. Although it is believed to be a fairly common complication of chronic liver disease, it is possible for a case of hepatopulmonary syndrome to be admitted solely for respiratory symptoms. The patient’s poor socio-economic status is the main reason for both the lack of proper followup for his liver disease and the limited therapeutic options. Keywords: Hepatopulmonary syndrome, liver cirrhosis, respiratory failure, contrast echocardiography

Hepatopulmonary syndrome (HPS) is a respiratory complication of hepatic disease, that is well recognized in humans and defined by the presence of 1) liver disease, 2) hypoxemia and/or high alveolar-arterial oxygen gradient (AaDO2) and 3) intrapulmonary vasodilatation. The present report describes a similar case of HPS in a dog. A six-month-old Papillon was diagnosed with ductal plate malformation with chronic active hepatitis and showed progressive increases in AaDO2 over the course of the following six months. The presence of intrapulmonary vasodilatation was confirmed by agitated saline contrast transthoracic echocardiography. Also, the absence of congenital cardiac defect was confirmed by transthoracic echocardiography. From these results, we suspected that this dog had HPS. This is the first description of suspected canine HPS.

A 42-year-old man with a medical history of liver cirrhosis and portal hypertension was admitted to the hospital because of slowly progressive shortness of breath and hypoxemia. The diagnosis hepatopulmonary syndrome was confirmed by a pulmonary perfusion scan with 99m Tc-albumin. The scan showed a right-left shunt, because the 99m Tc-albumin transited the lungs and appeared in the brain, the thyroid gland, the kidneys and the spleen. The patient received a liver transplantation, which is considered the only definitive treatment.

Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are pulmonary vascular complications of portal hypertension with divergent clinicopathologic features and management. The presence of intrapulmonary vascular dilatations (IPVDs), detected by agitated saline contrast-enhanced transthoracic echocardiography (cTTE), is an essential feature of HPS but is not typically characteristic of POPH. Although IPVDs have been reported rarely in POPH, the prevalence and significance of this finding have not been systematically studied. We conducted a retrospective chart review of 80 consecutive patients diagnosed with POPH from January 1, 2002 to June 30, 2014 with documentation of cTTE findings, pulmonary hemodynamics, oxygenation, and survival. A total of 34 of the 80 patients (42%) underwent cTTE during initial diagnosis of POPH. IPVDs were detected in 20/34 patients (59%); intracardiac shunting was detected in 9/34 patients (26%; 4 also had IPVDs); and 9 patients (26%) had negative cTTE with no evidence of IPVD or intracardiac shunting. Patients with IPVD had decreased survival as compared to those without IPVD (P = 0.003), a trend that persisted after exclusion of liver transplant recipients (P =  0.07). The IPVD group had a trend toward higher Model for End-Stage Liver Disease score with and without incorporating sodium (MELD or MELD-Na; P = 0.05 for both). The right ventricular index of myocardial performance (RIMP) was lower in the IPVD group (median, 0.4 versus 0.6; P = 0.006). Patients with moderate or large IPVDs (n = 6) had worse oxygenation parameters (partial pressure of arterial oxygen, diffusing capacity of the lung for carbon monoxide, and alveolar-arterial oxygen gradient) as compared to the rest of the cohort. Unexpectedly, IPVDs were frequently documented in POPH and associated with decreased survival. To further understand this observation, we recommend screening for IVPD in all patients with POPH., (© 2015 American Association for the Study of Liver Diseases.)

Introduction: Intrapulmonary vascular dilatations (IPVD) are considered a complication of cirrhosis. The technique of choice for their diagnosis is contrast-enhanced echocardiography (CEE). The aim of this study was to determine the usefulness of contrast-enhanced transcranial Doppler (CETD) in the diagnosis of IPVD., Method: We consecutively included patients evaluated for liver transplantation. A cross-sectional study was conducted. The investigator interpreting CETD was blind to the results of the gold standard (CEE). The accuracy of the diagnostic test was evaluated through sensitivity, specificity, positive and negative predictive values, and likelihood ratio., Results: CETD (n=43) showed a right-to-left shunt in 23 patients (62.2%): 4 early, 2 indeterminate and 17 late. Nineteen (51,4%) cases were classified as IPVD. With CEE (n=37), 10 procedures (27%) were negative for shunt, 27 (73%) were positive, and 21 (56.8%) were compatible with IPVD. Patients with and without IPVD showed no differences in age, sex, etiology, severity, or MELD score, independently of the diagnostic test. In the diagnostic validity study (n=37) of CETD versus CEE, the AUC for diagnostic yield was 0.813% (95%CI: 0.666-0.959; P=.001), sensitivity was 76.2% (95%CI: 54.9-89.4) and specificity was 90% (95%CI: 63.9-96.5). The positive likelihood ratio was 6.095., Conclusions: We found a high prevalence of IPVD in candidates for liver transplantation. When a late right-to-left shunt with recirculation is observed, CETD has a high probability of detecting IPVD, with few false-positive results. Because this technique has not previously been described in this indication, similar studies are needed for comparison., (Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.)

Hepatopulmonary syndrome is characterized by the presence of liver disease, pulmonary vascular dilatations, and arterial hypoxemia. It is usually associated with cirrhosis of any origin, but has been described in other liver diseases, both acute and chronic, and not always associated with portal hypertension. The gold standard method to detect pulmonary vascular dilations is contrast enhancement echocardiography with saline and is essential for the diagnosis of hepatopulmonary syndrome. These dilatations reflect changes in the pulmonary microvasculature (vasodilatation, intravascular monocyte accumulation, and angiogenesis) and induce a ventilation/perfusion mismatch, or even true intrapulmonary shunts, which eventually trigger hypoxemia. This syndrome worsens patients' prognosis and impairs their quality of life and may lead to the need for liver transplantation, which is the only effective and definitive treatment. In this article, we review the etiological, pathophysiological, clinical and therapeutic features of this syndrome., (Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.)

Hepatopulmonary syndrome is a serious pulmonary vascular complication in patients with chronic liver disease. It constitutes a triad of chronic liver disease, increased alveolar-arterial oxygen gradient, and evidence of intrapulmonary shunt (IPS). The diagnosis of IPS can be made by bubble echocardiography, technetium-99m-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy or pulmonary arteriography. Though echocardiography is a sensitive screening test, MAA scintigraphy can also quantify the extent of shunting.

Background & Aims: Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome., Methods: Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia/orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra- and intergroups with and without hepatopulmonary syndrome., Result: Of 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) (P = 0.01). Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, P < 0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, P = 0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both paediatric end-stage liver disease score and Child-Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%)., Conclusions: Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pulmonary right-to-left shunting can be encountered using transthoracic contrast echocardiography (TTCE) with agitated saline. Diseases associated with pulmonary shunting on saline TTCE include hereditary hemorrhagic telangiectasia (HHT), hepatopulmonary syndrome, and some congenital heart defects after partial or complete cavopulmonary anastomosis. Furthermore, small pulmonary shunts on saline TTCE are also documented in a proportion of healthy individuals. Pulmonary shunting carries the risk for severe neurologic complications due to paradoxical embolization. In HHT, additional chest computed tomography is recommended in case of any pulmonary shunt detected on saline TTCE, to evaluate the feasibility for transcatheter embolotherapy of pulmonary arteriovenous malformations. Furthermore, antibiotic prophylaxis is advised in case of any pulmonary shunt on saline TTCE to prevent brain abscesses after procedures with risk for bacteremia. The present review provides an overview of important aspects of pulmonary shunting and its detection using saline TTCE. Furthermore, advances in understanding the clinical implications of different pulmonary shunt grades on saline TTCE are described. It appears that small pulmonary shunts on saline TTCE (grade 1) lack any clinical implication, as these shunts cannot be used as a diagnostic criterion for HHT, are not associated with an increased risk for neurologic complications, and represent pulmonary arteriovenous malformations too small for subsequent endovascular treatment. This implies that additional chest computed tomography could be safely withheld in all persons with only small pulmonary shunts on saline TTCE and sets the stage for further discussion about the need for antibiotic prophylaxis in these subjects. Besides further optimization of the current screening algorithm for the detection of pulmonary arteriovenous malformations in HHT, these observations can be of additional clinical importance in other diseases associated with pulmonary shunting and in those healthy individuals with documented small pulmonary shunts on saline TTCE., (Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Hepatopulmonary syndrome, also known as hepatorenal syndrome, is a triad of liver disease, impaired oxygenation, and intrapulmonary vascular abnormalities. Forty-seven percent of patients with end-stage liver disease may have hepatopulmonary syndrome, an independent predictor of poor prognosis. Gross dilatation of pulmonary precapillary and capillary vessels, as well as an absolute increase in the number of dilated vessels, is the classic pathological description. We report a young man with cirrhosis demonstrating extrapulmonary shunting of 99mTc-MAA to kidneys with extensive peripheral arteriovenous (cutaneous) vasodilation, which we prefer to name as the "mosaic sign" in the absence of spider nevi or erythema.

Purpose: To identify liver tumor characteristics associated with low (<10%), intermediate (10%-20%), and high (>20%) lung shunt fraction (LSF) at technetium 99m ((99m)Tc) macroaggregated albumin (MAA) imaging performed before yttrium 90 ((90)Y) radioembolization (RE)., Materials and Methods: In this single-center retrospective study, 141 patients (70 with hepatocellular carcinoma [HCC], 71 with other tumors; 95 men, 45 women; median age, 61 years) underwent mapping arteriography with (99m)Tc-MAA LSF calculation before (90)Y RE from 2006 to 2012. Tumor characteristics, including tumor type, index lesion size and morphologic structure (circumscribed, infiltrative), focality (solitary oligonodular, multinodular), disease distribution (unilobar, bilobar), tumor burden (≤50%, 50%), portal vein invasion (present, absent), and arterioportal shunting (present, absent) were correlated with (99m)Tc-MAA imaging-calculated LSFs at univariate and multivariate analysis., Results: Median LSF was 8.4% (HCC, 9.0%; other tumors, 8.3%). LSF greater than 20% occurred in 14% of HCCs, but only in 3% of other tumors (P = .004). For HCC, tumor morphologic structure (P = .022), tumor burden (P < .001), main portal vein invasion (P = .033), and arterioportal shunting (P < .001) were significantly associated with different LSF categories at univariate analysis; infiltrative morphologic structure, tumor burden greater than 50%, portal vein invasion, and shunting had confirmed association with high LSF at multivariate analysis. For other liver tumors, tumor size (P = .001) and tumor burden (P = .003) were significantly associated with different LSF categories at univariate analysis. Multivariate confirmation was precluded by small sample size. Patients underwent a median of one (90)Y RE session (range, one to six), with median per-treatment and cumulative lung doses of 6.0 Gy and 8.5 Gy, respectively., Conclusion: LSF greater than 20% periodically occurs in HCC but is uncommon in other liver tumors. Specific tumor characteristics are associated with LSF greater than 20% and may indicate need for interventions to reduce LSF.

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

Background and Aim: The prevalence of hepatopulmonary syndrome (HPS) in the setting of cirrhosis ranges between 4%-47%. We aimed to detect a correlation between heart and lungs findings on contrast-enhanced ultrasound (CEUS)and pulse-oximetry, in order to early detect HPS and thus to improve patients referral to orthotopic liver transplantation(OLT)., Methods: We considered at risk for HPS all patients with delayed left ventricle (LV) enhancement of the contrast agent,at least 3 systolic beats after it appears in the right ventricle(RV). We correlated CEUS results with pulse-oximetry findings,considering to have a positive HPS diagnosis in all patients with both CEUS findings and SaO2 95%., Results: From 186 patients diagnosed with liver cirrhosis, 56 patients (30.10%) had delayed LV enhancement of the contrast agent. Pulse-oximetry showed alterations, such as SaO2 95%and PaO2 70 mmHg in 62 patients (33.33%). Pearson index showed a good correlation between lung and heart CEUS findings and pulse-oximetry (r=0.99) in HPS diagnosis. CONCLUSIONS. Two non-invasive, simple and rapid methods such as CEUS and pulse-oximetry can easily diagnose HPS, a highly fatal complication of liver cirrhosis, and can also guide the future treatment by speeding up OLT recommendations., (Celsius.)

Hepatopulmonary syndrome (HPS) is of prognostic value in patients awaiting for orthotopic liver transplantation (OLT), but little is known about the effect of cirrhotic cardiomyopathy (CCM). The aim of the present study was to estimate the prevalence and possible relation between respiratory and cardiac abnormalities in a same series of patients awaiting OLT. Special attention was paid to the prognostic value of CCM in comparison to HPS. Eighty-three patients were included (19 females, 64 males; 52.1 ± 10.0 yrs). All had lung function testing with arterial blood gases and echocardiographic evaluation at rest with a contrast echocardiography in case of arterial oxygenation defect. To estimate the presence of CCM, patients underwent a complete left and right echocardiography and Doppler examination. Complete echocardiographic assessment could be obtained in 64 of the 83 patients of the study. HPS was observed in 16.9% (14/83) and CCM in 23.4% (15/64) of patients. There was a tendency of more serious adverse events before and after OLT in patients with HPS in comparison to others but CCM was not of prognostic value. HPS and CCM were frequent in these patients awaiting OLT but both abnormalities were not found in the same patients. CCM was neither related to death before OLT nor to death or serious adverse events after OLT., (Copyright © 2013. Published by Elsevier Ltd.)

Background and Study Aims: Intrapulmonary vascular dilatations (IPVDs) are a criterion for the diagnosis of hepatopulmonary syndrome in patients with liver cirrhosis. We aimed to show that IPVDs are more common than suspected in a heterogenous cirrhotic population and to identify new diagnostic parameters., Patients and Methods: Forty-three consecutive patients with cirrhosis admitted to our Gastroenterology department were included in this prospective study. History, physical examination, ECG and, when warranted, pulmonary function tests and chest radiograph were used to exclude patients with significant cardiac or pulmonary disease. Contrast enhanced transthoracic echocardiography (CEE) was used to determine the presence of IPVDs. Pulse oximetry readings were taken in the supine and standing positions., Results: We found 12 patients with IPVDs. Statistical analysis proved the correlation between IPVDs and systolic pulmonary artery pressure (sPAP) (p= .049), right ventricle wall width (RVW) (p = .013) and E/A ratio (p = .034) but not left atrial or ventricular diameter. Orthodeoxia was also present more frequently in patients with positive CEE. The difference between supine and standing oxygen saturation (changeSat) proved a fair diagnostic test for detecting IPVDs, with an area under the receiver operated curve (AUROC) of 0.823., Conclusions: Our study shows that RVW, sPAP, E/A and orthodeoxia determined by pulse oximetry are valuable novel predictors of IPVDs, encouraging the routine use of pulse oximetry and echocardiography in cirrhotic patients.

Unlabelled: Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless of the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5, and 10 years) did not differ between groups based on baseline partial pressure of arterial oxygen (PaO2 ) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3, and 5 years post-LT in those HPS patients transplanted after January 1 2002 (n = 28) (92%, 88%, and 88%, respectively) as compared with those transplanted prior to that time (n = 21) (71%, 67%, and 67%, respectively) did not reach statistical significance (5-year P = 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since January 1 2002 with post-LT survival of 19/21 patients and one wait-list death., Conclusion: Long-term outcome after LT in HPS is favorable, with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO2 levels at the time of HPS diagnosis. (HEPATOLOGY 2012)., (Copyright © 2012 American Association for the Study of Liver Diseases.)

Yttrium-90 ((90)Y) radioembolization represents an emerging transcatheter treatment option for the management of hepatocellular carcinoma (HCC). Elevation of the hepatopulmonary shunt fraction risks nontarget radiation to the lungs and may limit the use of (90)Y therapy in patients with locally advanced disease with vascular invasion, who often demonstrate increased shunting. We present two cases in which patients with HCC and portal vein invasion resulting in elevated hepatopulmonary shunt fractions underwent chemoembolic shunt closure to allow safe (90)Y radioembolization. Both patients demonstrated excellent tumor response and patient survival. On this basis, we propose a role for chemoembolic reduction of the lung shunt fraction before (90)Y radioembolization in patients with extensive tumor-related hepatopulmonary shunting.