Cardiac index and hepatopulmonary syndrome in liver transplantation candidates: The pulmonary vascular complications of liver disease study.

BACKGROUND AND AIMS: Hepatopulmonary syndrome (HPS) and a hyperdynamic circulation are common complications of advanced liver disease, but the relationship between HPS and cardiac index (CI) is poorly understood. We sought to compare CI in patients with and without HPS and to assess the relationship between CI and symptoms, quality of life, gas exchange, and exercise capacity among liver transplantation (LT) candidates. We performed a cross-sectional analysis within the Pulmonary Vascular Complications of Liver Disease 2 study, a multicenter prospective cohort study of patients being evaluated for LT. We excluded patients with obstructive or restrictive lung disease, intracardiac shunting, and portopulmonary hypertension. We included 214 patients (81 with HPS and 133 controls without HPS). Compared with controls, patients with HPS had a higher CI (least square mean 3.2 L/min/m 2 , 95% CI 3.1-3.4 vs. 2.8 L/min/m 2 , 95% CI 2.7-3.0, p < 0.001) after adjustment for age, sex, Model for End-stage Liver Disease-Sodium (MELD-Na) score and beta-blocker use, and a lower systemic vascular resistance. Among all LT candidates, CI was correlated with oxygenation (Alveolar-arterial oxygen gradient r =0.27, p < 0.001), intrapulmonary vasodilatation severity ( p < 0.001), and biomarkers of angiogenesis. Higher CI was independently associated with dyspnea and worse functional class and physical quality of life after adjusting for age, sex, MELD-Na, beta-blocker use, and HPS status. HPS was associated with a higher CI among LT candidates. Independent of HPS, higher CI was associated with increased dyspnea and worse functional class, quality of life, and arterial oxygenation.

Prevalence and prognostic impact of hepatopulmonary syndrome in patients with unresectable hepatocellular carcinoma undergoing transarterial chemoembolization: a prospective cohort study.

BACKGROUND: To determine the prevalence and prognostic impact of hepatopulmonary syndrome (HPS) in patients with unresectable hepatocellular carcinoma (HCC) undergoing transarterial chemoembolization (TACE). METHODS: Fifty-four patients with unresectable HCC undergoing TACE between December 2014 and December 2015 were prospectively screened for HPS and were followed up for a maximum of 2 years or until the end of this prospective study. RESULTS: Nineteen of the 54 (35.2%) patients were considered to have HPS, including one (5.3%) with severe HPS, nine (47.4%) with moderate HPS, and nine (47.4%) with mild HPS. The median overall survival (OS) was 10.1 (95% confidence interval [CI], 3.9-16.3) months for patients with HPS and 15.1 (95% CI, 7.3-22.9) months for patients without HPS, which is not a significant difference ( P  = 0.100). The median progression-free survival was also not significantly different between patients with and without HPS (5.2 [95% CI, 0-12.8] vs. 8.4 [95% CI, 3.6-13.1] months; P  = 0.537). In the multivariable Cox regression analyses, carbon monoxide diffusing capacity (hazard ratio [HR] = 1.033 [95% CI, 1.003-1.064]; P  = 0.028) and Child-Pugh class (HR = 1.815 [95% CI, 1.011-3.260]; P  = 0.046) were identified to be the independent prognostic factors of OS. CONCLUSION: Mild or moderate HPS is common in patients with unresectable HCC undergoing TACE, but it does not seem to have a significant prognostic impact.

Pulmonary vascular disease and exercise hemodynamics in chronic liver disease.

BACKGROUND & AIMS: Portopulmonary hypertension (POPH) and hepatopulmonary syndrome (HPS) are severe pulmonary vascular complications of chronic liver disease and strongly associated with morbidity and mortality. The prevalence of these complications is relatively high in patients evaluated for liver transplantation, however it is virtually unknown in patients with stable chronic liver disease. METHODS: We assessed the pulmonary hypertension (PH) and HPS prevalence in a prospective registry study of our liver out-patient clinic in a tertiary center. Between 2011 and 2016, consecutive patients with cirrhosis or non-cirrhotic portal hypertension were prospectively enrolled after written informed consent. We excluded patients with acute decompensation of liver disease and other causes of PH like severe chronic heart or lung diseases and chronic thromboembolic PH. HPS was diagnosed using contrast enhanced echocardiography and blood gas analysis. Patients were screened for PH using an algorithm implementing severity of dyspnea, echocardiography, cardiopulmonary exercise testing and exercise echocardiography employing a threshold of systolic pulmonary arterial pressure (SPAP) = 50 mmHg at peak exercise. If the algorithm indicated an increased PH risk, patients were invited for invasive investigations by means of right heart and hepatic vein catheter. We defined POPH as resting mPAP≥21 mmHg and PVR>3WU and PAWP<15 mmHg, mild PH as resting mPAP = 21-24 mmHg, and exercise PH as mPAP>30 mmHg and TPR >3 WU at peak exercise. RESULTS: Two-hundred-five patients were enrolled (male 75%; cirrhosis 96%; median age 57 yrs). Sixty-seven patients (33%) fulfilled HPS criteria but only two (1.0%) for severe (PaO2:50-60 mmHg) or very severe HPS (PaO2<50 mmHg). In 18/77 patients (23%) undergoing exercise echocardiography, SPAP at peak exercise exceeded 50 mmHg. Finally, n = 3 (1.5%) patients were invasively diagnosed with POPH, n = 4 (2.9%) with mild PH and n = 2 with exercise PH. CONCLUSION: In chronic liver disease, excluding acute decompensation and other causes of PH, POPH and severe HPS are rare findings while mild to moderate HPS and mild PH or exercise PH are more frequent.

[Hepatopulmonary syndrome: Prevalence, pathophysiology and clinical implications]. / Syndrome hépatopulmonaire : prévalence, physiopathologie et implications cliniques.

Hepatopulmonary syndrome (HPS) is a pulmonary vascular disease induced by portal hypertension that is characterized by dilation of the pulmonary capillaries and right-to-left shunting, leading to gas exchange abnormalities. While dysregulation of several endothelial cell (EC)-derived angiocrine factors and the development of HPS have been associated, the field is currently lacking in mechanistic understanding on how they contribute to disease initiation, perpetuation, and worsening. Although substantial progress has been made in the description of clinical characteristics and outcomes, no specific targeting therapy has been shown to have a long-term effect on the evolution of HPS; only liver transplantation can lead full or partial reversibility of the syndrome. This article aims to provide a comprehensive review of the clinical and epidemiological definitions of HPS and to describe its experimental models as well as recent advances in understanding the pathophysiology of the disease, with specific focus on BMP-9 and its signaling pathway.

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.

Pulmonary Abnormalities in Liver Disease: Relevance to Transplantation and Outcome.

Pulmonary disease in liver cirrhosis and portal hypertension (PH) constitutes a challenging clinical scenario and may have important implications with regard to prognosis, liver transplantation (LT) candidacy, and post-LT outcome. Pre-LT evaluation should include adequate screening for pulmonary diseases that may occur concomitantly with liver disease as well as for those that may arise as a complication of end-stage liver disease and PH, given that either may jeopardize safe LT and successful outcome. It is key to discriminate those patients who would benefit from LT, especially pulmonary disorders that have been reported to resolve post-LT and are considered "pulmonary indications" for transplant, from those who are at increased mortality risk and in whom LT is contraindicated. In conclusion, in this article, we review the impact of several pulmonary disorders, including cystic fibrosis, alpha 1-antitrypsin deficiency, hereditary hemorrhagic telangiectasia, sarcoidosis, coronavirus disease 2019, asthma, chronic obstructive pulmonary disease, pulmonary nodules, interstitial lung disease, hepatic hydrothorax, hepatopulmonary syndrome, and portopulmonary hypertension, on post-LT survival, as well as the reciprocal impact of LT on the evolution of lung function.

Prevalence and determinants of hepatopulmonary syndrome in decompensated chronic liver disease.

BACKGROUND: One of the severe complications of liver disease is hepatopulmonary syndrome (HPS). There is paucity in literature regarding the various factors associated with the development of HPS. This study was conducted to analyze the prevalence and determinants of HPS among patients with decompensated chronic liver disease (CLD). METHODS: This study was a cross-sectional-observational study conducted in a tertiary care center. Decompensated CLD patients admitted for pre-liver transplant workup were included in the study. Demographic data, clinical findings, and biochemical and hematologic parameters were collected. Pulse oximetry, arterial blood gas analysis, bubble contrast echocardiogram, diffusion capacity of the lungs for carbon monoxide (DLCO), and spirometry were done to get the needed parameters. All data were entered into a Microsoft Excel sheet and analyzed using the statistical software SPSS for Windows, version 22.0. RESULTS: Among 64 subjects, 58 were men (90.6%). Mean age was 54.5 years. HPS was present in 26 (40.6%) patients. Platypnea and orthodeoxia were present more often in HPS patients. DLCO was significantly impaired among patients with HPS. Portopulmonary hypertension was seen in 8 (12.5%) subjects with no difference between HPS and non-HPS patients. Subjects with HPS had more severe liver disease. A model for end-stage liver disease (MELD)-Na score > 19 was associated with HPS (sensitivity 73.08%, specificity 65.79%, PPV 59.4%, and NPV 78.1%). Multivariate analysis (binary logistic regression) revealed that a higher MELD-Na score, hepatic encephalopathy, and impaired DLCO were independently associated with HPS. CONCLUSIONS: HPS is associated with more severe liver disease (as per Child-Turcotte-Pugh [CTP] stage and MELD-Na score). There was no relation between HPS and causes of CLD. Higher MELD-Na score, hepatic encephalopathy, impaired DLCO, clubbing, and spider naevi were independently associated with HPS.

Endothelin-1 level as a predictor of hepatopulmonary syndrome in liver cirrhosis.

Aim To determine the role of endothelin (ET)-1 in predicting hepatopulmonary syndrome (HPS) in patients with liver cirrhosis. Methods A cross sectional study involving 80 liver cirrhosis patients aged 18 years or older was conducted in Adam Malik General Hospital Medan, Indonesia between July 2017 and June 2018. HPS diagnosis was confirmed from the presence of liver cirrhosis, abnormal oxygenation, and intrapulmonary vascular dilatations (IPVD). ET-1 level was measured from serum sample using ELISA method. Patients with coexisting primary pulmonary pathology and intrinsic heart disease were active smokers, and those who declined to participate were excluded. Statistical analysis was conducted at 95% confidence interval. p<0.05 was considered significant. Results Majority the patients were male (56.3%) and had higher educational background (62.5%). Mean age of the patients was 51.3 (SD=12.6) years. The prevalence of HPS was 21.2%. The patients with HPS had higher ET-1 level compared to those without HPS (p<0.001). The patients with hepatic encephalopathy had 2.917 times higher risk for suffering from HPS, while the patients with Child Pugh score A had lower risk (0.738 times) for having HPS compared to subjects with Child Pugh score B and C. ET-1 level >187.5 mg/L had sensitivity and specificity for predicting HPS in subjects with liver cirrhosis of 82.35% and 81.25%, respectively. Conclusion ET-1 could be used as a promising marker for HPS in patients with liver cirrhosis. ET-1 level of >187.5 mg/l had a good accuracy in predicting HPS in liver cirrhosis patient.

Guidelines for the Management of Adult Acute and Acute-on-Chronic Liver Failure in the ICU: Cardiovascular, Endocrine, Hematologic, Pulmonary, and Renal Considerations.

OBJECTIVES: To develop evidence-based recommendations for clinicians caring for adults with acute or acute on chronic liver failure in the ICU. DESIGN: The guideline panel comprised 29 members with expertise in aspects of care of the critically ill patient with liver failure and/or methodology. The Society of Critical Care Medicine standard operating procedures manual and conflict-of-interest policy were followed throughout. Teleconferences and electronic-based discussion among the panel, as well as within subgroups, served as an integral part of the guideline development. SETTING: The panel was divided into nine subgroups: cardiovascular, hematology, pulmonary, renal, endocrine and nutrition, gastrointestinal, infection, perioperative, and neurology. INTERVENTIONS: We developed and selected population, intervention, comparison, and outcomes questions according to importance to patients and practicing clinicians. For each population, intervention, comparison, and outcomes question, we conducted a systematic review aiming to identify the best available evidence, statistically summarized the evidence whenever applicable, and assessed the quality of evidence using the Grading of Recommendations Assessment, Development, and Evaluation approach. We used the evidence to decision framework to facilitate recommendations formulation as strong or conditional. We followed strict criteria to formulate best practice statements. MEASUREMENTS AND MAIN RESULTS: In this article, we report 29 recommendations (from 30 population, intervention, comparison, and outcomes questions) on the management acute or acute on chronic liver failure in the ICU, related to five groups (cardiovascular, hematology, pulmonary, renal, and endocrine). Overall, six were strong recommendations, 19 were conditional recommendations, four were best-practice statements, and in two instances, the panel did not issue a recommendation due to insufficient evidence. CONCLUSIONS: Multidisciplinary international experts were able to formulate evidence-based recommendations for the management acute or acute on chronic liver failure in the ICU, acknowledging that most recommendations were based on low-quality indirect evidence.

Comparison of Two Diagnostic Criteria for Hepatopulmonary Syndrome-High Prevalence in Biliary Atresia.

OBJECTIVES: There is lack of clarity regarding the exact prevalence of hepatopulmonary syndrome (HPS) in pediatric liver diseases owing to lack of standardized diagnostic criteria. Thus, we aimed to do a comparative study of HPS with respect to its prevalence using the available diagnostic criteria. METHODS: All consecutive children with biliary atresia (BA) and other chronic liver diseases (CLDs) were studied. Prevalence of HPS was compared using the 2 available criteria: demonstration of intrapulmonary vascular dilatation along with either alveolar-arterial oxygen difference (P [A-a] O2) on arterial blood gas analysis of more than 15 mmHg (criteria 1), or higher than age-appropriate calculated value for P (A-a) O2 (criteria 2). RESULTS: A total of 42 children in BA group and 62 in the non-BA CLD group were included. Using the criteria 1, the prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group, whereas using criteria 2, the prevalence was 48.1%: 61.9% in the BA group and 38.7% in the CLD group. Criteria 2 diagnosed 6 additional patients with HPS compared to criteria 1 (P value 0.405). BA subjects had higher risk (2.9-3 folds) of developing HPS compared to other CLDs. CONCLUSION: There is high prevalence of HPS in pediatric liver disease subjects. Age-appropriate formula for HPS diagnosis may be better applicable in pediatric population. BA subjects have a higher risk of developing HPS compared to other CLDs overall, irrespective of the severity of liver disease and/or portal hypertension.

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study.

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

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.

[Hepatopulmonar syndrome and liver transplantation: experience in the transplantation department of the Guillermo Almenara Irigoyen National Hospital - EsSalud]. / Síndrome hepatopulmonar y trasplante hepático: experiencia en el departamento de trasplantes del Hospital Nacional Guillermo Almenara Irigoyen - EsSalud.

INTRODUCTION: Hepatopulmonary syndrome (HPS) is a serious complication of liver disease, which is characterized by the presence of intrapulmonary vasodilation and progressive hypoxemia. Liver transplantation is the only effective treatment. OBJECTIVE: To show our results of patients with hepatopulmonary syndrome undergoing liver transplantation. MATERIALS AND METHODS: Retrospective, descriptive and cross-sectional study. From March 2000 to December 2016; 226 liver transplants were performed. Of the total, 25 patients were excluded: 12 retransplantation, 9 liver-kidney combined transplants, 2 transplants for acute liver failure, 2 transplants in non-cirrhotic patients. Of the 201 patients with pretransplant diagnosis of liver cirrhosis, 19 filled criteria for SHP; who were distributed according to age, sex, hypoxemia level (pO2), Child-Pugh score and MELD score. The reversibility hypoxemia after liver trasplantation was measured with a cut-off of p02 >75 mmHg. RESULTS: The prevalence of SHP in our series was 9.45%. The average age was 41 years (14-65); the M / F ratio of 1.65. The 78.94% (15/19) were adults. 89.5% (17/19) were Score of Child-Pugh B and C, and 68.4% had severe and very severe SHP. In 94.11% of patients, reversibility SHP founded. The early mortality rate (30 days) in patients with SHP was 10.4%. CONCLUSIONS: The prevalence of HPS in our series was 9.45%. Transplanted patients with and without SHP had similar survival.

[Liver diseases and pulmonary vascular disorders]. / Hépatopathies et maladies vasculaires pulmonaires.

About 70% patients waiting for liver transplantation have a dyspnea. Two pulmonary vascular disorders can be associated with portal hypertension or chronic liver diseases: portopulmonary hypertension (PoPH) related to pulmonary small arteries remodeling and obstruction and hepatopulmonary syndrome (HPS) characterized by pulmonary capillaries dilatations and proliferations. PoPH is defined by the combination of pulmonary arterial hypertension (PAH) (mean pulmonary artery pressure [PAP]≥25mmHg, with normal pulmonary artery wedge pressure≤15mmHg and pulmonary vascular resistance [PVR]>3 Wood units [WU]) and portal hypertension. HPS is a triad of intrapulmonary vascular dilatations, hypoxemia (increased alveolar-arterial oxygen gradient) and liver disease or isolated portal hypertension. The pathophysiology of both syndromes is complex and poorly understood. PoPH and HPS have a negative impact on functional and vital prognosis in patients with portal hypertension. Liver transplantation is the established treatment standard in HPS. PoPH treatment is improved over the years with the use of specific PAH treatment despite the lack of randomized assay in this indication. Liver transplantation could be considered in PoPH leading to stabilization, improvement or recovery in selected patients (mean PAP<35mmHg without severe right ventricular dysfunction and PVR<4 WU).

Síndrome hepatopulmonar y trasplante hepático: experiencia en el departamento de trasplantes del Hospital Nacional Guillermo Almenara Irigoyen - EsSalud / Hepatopulmonar syndrome and liver transplantation: experience in the transplantation department of the Guillermo Almenara Irigoyen National Hospital - EsSalud

Introducción: El síndrome hepatopulmonar (SHP) es una complicación grave de la enfermedad hepática, la cual se caracteriza por la presencia de vasodilatación intrapulmonar e hipoxemia progresiva, siendo el trasplante de hígado el único tratamiento efectivo. Objetivo: Mostrar nuestros resultados de los pacientes con síndrome hepatopulmonar sometidos a trasplante hepático. Materiales y métodos: Estudio retrospectivo, descriptivo y trasversal. Desde marzo del 2000 a diciembre del 2016 se realizaron 226 trasplantes de hígado. Del total, se excluyeron a 25 pacientes: 12 retrasplantes, 9 trasplantes dobles higadoriñon, 2 trasplantes con falla hepática aguda, 2 trasplantes en pacientes no cirróticos. De los 201 pacientes con diagnóstico pretrasplante de cirrosis hepática, 19 tuvieron criterios de SHP; quienes fueron distribuidos según edad, sexo, nivel de hipoxemia (pO2), score CHILD, score MELD. La reversibilidad de la hipoxemia post trasplante se midió con una cutt off de p0(2) >75 mmHg. Resultados: La prevalencia del SHP en nuestra serie fue 9,45%. La edad promedio fue 41 años (14-65); la relación M/F de 1,65. El 78,94% (15/19) fueron adultos. 89,5% (17/19) fueron score de CHILD B y C, y el 68,4% tuvieron SHP severo y muy severo. En el 94,11% de los pacientes se demostró reversibilidad del SHP. La tasa de mortalidad temprana en los pacientes con SHP fue 10,4%. Conclusiones: La prevalencia del SHP fue del 9,45%. Los pacientes trasplantados con y sin SHP tuvieron similar sobrevida.

Introduction: Hepatopulmonary syndrome (HPS) is a serious complication of liver disease, which is characterized by the presence of intrapulmonary vasodilation and progressive hypoxemia. Liver transplantation is the only effective treatment. Objective: To show our results of patients with hepatopulmonary syndrome undergoing liver transplantation. Materials and methods: Retrospective, descriptive and cross-sectional study. From March 2000 to December 2016; 226 liver transplants were performed. Of the total, 25 patients were excluded: 12 retransplantation, 9 liver-kidney combined transplants, 2 transplants for acute liver failure, 2 transplants in non-cirrhotic patients. Of the 201 patients with pretransplant diagnosis of liver cirrhosis, 19 filled criteria for SHP; who were distributed according to age, sex, hypoxemia level (pO2), Child-Pugh score and MELD score. The reversibility hypoxemia after liver trasplantation was measured with a cut-off of p0(2) >75 mmHg. Results: The prevalence of SHP in our series was 9.45%. The average age was 41 years (14-65); the M / F ratio of 1.65. The 78.94% (15/19) were adults. 89.5% (17/19) were Score of Child-Pugh B and C, and 68.4% had severe and very severe SHP. In 94.11% of patients, reversibility SHP founded. The early mortality rate (30 days) in patients with SHP was 10.4%. Conclusions: The prevalence of HPS in our series was 9.45%. Transplanted patients with and without SHP had similar survival.

Review article: Update on current and emergent data on hepatopulmonary syndrome.

Hepatopulmonary syndrome (HPS) is a frequent pulmonary complication of end-stage liver disease, characterized by impaired arterial oxygenation induced by intrapulmonary vascular dilatation. Its prevalence ranges from 4% to 47% in patients with cirrhosis due to the different diagnostic criteria applied among different studies. Nitric oxide overproduction and angiogenesis seem to be the hallmarks of a complicated pathogenetic mechanism, leading to intrapulmonary shunting and ventilation-perfusion mismatch. A classification of HPS according to the severity of hypoxemia has been suggested. Contrast-enhanced echocardiography represents the gold standard method for the detection of intrapulmonary vascular dilatations which is required, in combination with an elevated alveolar arterial gradient to set the diagnosis. The only effective treatment which can modify the syndrome's natural history is liver transplantation. Although it is usually asymptomatic, HPS imparts a high risk of pretransplantation mortality, independently of the severity of liver disease, while there is variable data concerning survival rates after liver transplantation. The potential of myocardial involvement in the setting of HPS has also gained increasing interest in recent research. The aim of this review is to critically approach the existing literature of HPS and emphasize unclear points that remain to be unraveled by future research.

Chronic liver disease is universal in children with biliary atresia living with native liver.

AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers. METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices. RESULTS: Fifty-two children [females = 32, 62%; median age 7.4 years, n = 35 (67%) older than 5 years] with BA (median age at surgery 60 d, range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension (40%, n = 21; 2 younger than 5 years), cholangitis (36%) and bleeding varices (25%, n = 13; 1 younger than 5 years). Fifteen (29%) had no clinical complications of CLD and three (6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient (2%). CONCLUSION: Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age, underscoring the importance of medical surveillance for complications of BA starting at a young age.

Late complications of biliary atresia: hepatopulmonary syndrome and portopulmonary hypertension.

Children with biliary atresia (BA) following Kasai portoenterostomy have a high risk for portal hypertension, however, while variceal and hemorrhagic complications have been more commonly studied, less frequent but no less possibly devastating complications of hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) remain less well understood. HPS and PPH both occur in a setting of portal hypertension, however, paradoxically patients with HPS develop pulmonic vasculature dilation leading to shunting and hypoxia, while those with PPH develop an opposite progression of pulmonary vasoconstriction eventually leading to cor pulmonale and decompensation. Given the near diametric evolution of diseases, HPS and PPH differ widely in therapy, though liver transplantation can have a role for treatment in either disease state. We reviewed our series of 320 pediatric patients with biliary atresia treated at our institution over 44 years, highlighting two cases that developed HPS and PPH, respectively, using these cases in further discussion of hepatopulmonary syndrome and portopulmonary hypertension regarding disease etiology, diagnosis, management, and prognosis. The complicated nature of these processes demand a careful multidisciplinary approach to optimize patient outcomes, including mindful evaluation for when transplantation may offer benefit.

Prevalencia y severidad del síndrome hepatopulmonar en pacientes con cirrosis hepática del Hospital Nacional Cayetano Heredia, entre enero y diciembre del 2015 / Prevalence and severity of the hepatopulmonar syndrome in patients with liver cirrhosis attended at Cayetano Heredia Hospital, between January and December 2015

Introducción: El sindrome hepatopulmonar (SHP) es una complicación poco frecuente de la cirrosis hepática (CH) que disminuye considerablemente la calidad de vida de las personas que la padecen. Objetivos: Determinar la prevalencia y severidad del SHP en los pacientes con CH atendidos en el Hospital Nacional Cayetano Heredia (HCH) en el periodo comprendido entre enero a diciembre del 2015. Material y métodos: Estudio transversal, con tamaño de muestra necesario para determinar la prevalencia puntual calculado en 297 pacientes. Resultados: La prevalencia del SHP fue de 0,7% y los casos identificados se clasificaron como SHP leve y severo. Conclusión: La prevalencia del SHP es muy baja en la población de pacientes con cirrosis hepática atendidos en el Hospital Nacional Cayetano Heredia

Introduction: The hepatopulmonary syndrome (HPS) is a rare complication of liver cirrhosis (LC) which significantly diminishes the quality of life for people who suffer. Objectives: To determine the prevalence and severity of HPS in patients with CH treated at the Cayetano Heredia (HCH) Hospital in the period from January to December 2015. Materials and methods: Cross-sectional study with sample size needed to determine the point prevalence calculated in 297 patients. Results: The prevalence of HPS in 0.7% and the identified cases were classified as mild and severe SHP. Conclusion: The prevalence of HPS is very low in the population of patients with liver cirrhosis treated at the Cayetano Heredia Hospital

The multiple dimensions of Platypnea-Orthodeoxia syndrome: A review.

Platypnea-Orthodeoxia syndrome (POS) is a rare clinical entity characterized by dyspnea and arterial desaturation while in the upright position. The various pathophysiologic mechanisms leading to POS has puzzled clinicians for years. The hypoxia in POS has been attributed to the mixing of the deoxygenated venous blood with the oxygenated arterial blood via a shunt. The primary mechanisms of POS in these patients can be broadly classified based on intracardiac abnormalities, extracardiac abnormalities and miscellaneous etiologies. A Patent Foramen Ovale (PFO) was the most common reported site of an intracardiac shunt. In addition to PFO, intracardiac shunt leading to POS has been reported from either an Atrial Septal Defect (ASD) or an Atrial Septal Aneurysm (ASA). Most patients with an intracardiac shunt also demonstrated a secondary anatomic or a functional defect. Extracardiac causes of POS included intra-pulmonary arteriovenous malformations and lung parenchymal diseases. A systematic evaluation is necessary to identify the underlying cause and institute an appropriate intervention. We conducted a review of literature and reviewed 239 cases of POS. In this article, we review the etiology and pathophysiology of POS and also summarize the diagnostic algorithms and treatment modalities available for early diagnosis and prompt treatment of patients presenting with symptoms of platypnea and/or orthodeoxia.