Endovascular closure of a congenital extrahepatic portosystemic shunt for the treatment of hepatopulmonary syndrome in an infant.

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.

Imaging in hepatopulmonary syndrome-case report. A multicenter approach during the coronavirus pandemic.

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.

A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report.

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 99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (99mTc-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 99mTc-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 99mTc-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.

Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism.

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.

Hepatopulmonary syndrome is related to the development of acute-on-chronic liver failure and poor prognosis in cirrhotic patients.

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.

Assessment of biventricular function in patients with hepatopulmonary syndrome.

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 (χ2 = 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.

Hepatopulmonary Syndrome Mimics Pulmonary Embolism on Pulmonary Ventilation/Perfusion SPECT/CT Study.

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.

Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis / Valor do Ecocardiograma Transesofágico com Contraste no Diagnóstico da Dilatação Vascular Intrapulmonar na Esquistossomose Hepatoesplênica

Abstract 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.

Resumo Fundamento: A síndrome hepatopulmonar (SHP), presente em pacientes cirróticos, é pouco estudada na esquistossomose hepatoesplênica (EHE) e inclui a ocorrência de dilatações vasculares intrapulmonares (DVP). O ecocardiograma transesofágico com contraste (ETEc) de microbolhas é mais sensível que o ecocardiograma transtorácico com contraste (ETTc) de microbolhas na identificação de DVP na cirrose. Objetivo: Avaliar o desempenho do ETEc comparado ao ETTc na identificação de DVP para diagnóstico de SHP em pacientes com EHE. Métodos: Incluímos 22 pacientes com EHE submetidos a ETEc e ETTc para pesquisa de DVP, além de exames laboratoriais. Os ETEc e ETTc foram realizados empregando-se solução salina agitada, injetada em veia periférica. A visualização tardia das microbolhas em câmaras esquerdas indicava presença de DVP. Os resultados foram comparados entre os dois métodos pelos testes t de Stu dent e qui-quadrado (significância p < 0,05). Resultados: Todos os 22 pacientes realizaram ETEc sem intercorrências. Foram excluídos três pela presença de forame oval patente (FOP), e a análise final foi realizada nos outros 19. A DVP esteve presente ao ETEc em 13 pacientes (68%) e em apenas seis ao ETTc (32%, p < 0,01). Não houve diferenças significativas nos dados clínicos e laboratoriais entre os grupos com e sem DVP, incluindo a diferença alveoloarterial de oxigênio. O diagnóstico de SHP (presença de DVP com alterações gasométricas) ocorreu em cinco pacientes pelo ETEc e em apenas um pelo ETTc (p = 0,09). Conclusão: Em pacientes com EHE, o ETEc foi seguro e superior ao ETTc na detecção de DVP não identificada ao ETTc, o que possibilitou adicionalmente excluir FOP.

Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis.

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.

A 10-year-old boy with dyspnea and hypoxia: abernathy malformation masquerading as pulmonary arteriovenous fistula.

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.

Effect of Transjugular Intrahepatic Portosystemic Shunt Creation on Pulmonary Gas Exchange in Patients with Hepatopulmonary Syndrome: A Prospective Study.

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.

Prevalence and characteristics of hepatopulmonary syndrome in children with cirrhosis in southern Brazil.

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.

Hepatopulmonary syndrome-attributed extreme hypoxemia and polycythemia revealing liver cirrhosis.

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.

Síndrome hepatopulmonar y enfermedad pulmonar intersticial difusa: una asociación poco conocida / Hepatopulmonary Syndrome and Diffuse Interstitial Lung Disease: An Unusual Combination

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Fístula hepatopulmonar: Una complicación rara del absceso hepático / Hepatopulmonary fistula: a rare complication of hepatic abscess

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Pulmonary transit time derived from pulmonary angiography for the diagnosis of hepatopulmonary syndrome.

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.