Severe Hepatopulmonary Syndrome Research Articles

Favorable Outcomes of Liver Transplantation for Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of chronic liver disease, which develops insidiously as a result of chronic liver disease. The prognosis for untreated patients with HPS is extremely poor, and liver transplantation (LT) serves as the only effective means for treating this condition. Here, we performed a retrospective analysis to evaluate the efficacy of LT on the survival and long-term prognosis of patients with HPS. Clinical data, including survival and postoperative efficacy, from patients with HPS from records as obtained over the period from January 1 to December 31, 2022. All records were from a waiting list for LT at the Beijing Friendship Hospital Affiliated with Capital Medical University. Among the 274 patients on the LT waiting list, 37 were diagnosed with HPS (13.50%) and were enrolled. Survival rates of patients with HPS receiving an LT were greater, whereas a statistically significant difference was obtained between patients with LT vs non-LT with moderate to severe HPS (P = .003). The overall time until death without LT was 4-72 days after their initial HPS diagnosis. Patients with HPS receiving an LT showed a significant improvement in the state of oxygenation after surgery (P = .001). Comprehensive preoperative screening of patients on the waiting list for LT is critical to identify those patients with HPS who would maximally benefit from LT. Survival rates of patients with moderate to severe HPS are significantly increased after LT, a procedure that should be performed as soon as possible in these patients with HPS.

Hepatopulmonary syndrome: diagnosis and treatment

Hepatopulmonary syndrome (HPS) is one of the lung diseases associated with liver cirrhosis and portal hypertension. The diagnosis is based on the triad: liver disease and portal hypertension, evidence of intrapulmonary vascular dilatation and impaired gas exchange. HPS impairs prognosis (23 % survival after 5 years) and patients’ quality of life, so early diagnosis and timely treatment are of great importance. Liver transplantation allows for regression of intrapulmonary vascular dilatation in almost 100 % of cases, normalization of gas exchange and improves a 5-year survival after transplantation from 76 to 87 %. This is the only treatment method indicated for patients with severe HPS, defined by an arterial partial pressure of oxygen (PaO2) below 60 mm Hg. However, in the face of a global shortage of transplants, it is necessary to develop medical therapies to delay or even defer liver transplantation. This goal seems possible due to the growing understanding of the HPS pathophysiology and the development of therapies targeting key mechanisms, mainly inflammatory and angiogenic. This article provides an overview of the clinical manifestations, diagnosis and treatment of HPS based on literature sources from the MEDLINE database on the PubMed platform.

Chronic Hypoxemia Inadvanced Liver Diseases: Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is one of the pulmonary complications from liver disease with or without cirrhosis. The prevalence of HPS varies from 4% to 47% in patients with liver cirrhosis. We present a case of chronic progressive dyspnea in an 18-year-old man with a history of chronic liver disease. Diagnosing can be particularly challenging, especially in cases involving prolonged dyspnea and comorbidities. Following thorough investigations, the patient's condition was attributed to a rarer condition: severe hepatopulmonary syndrome. Contrast-enhanced echocardiography with agitated saline is the gold standard for diagnosing pulmonary vascular dilatation which is the hallmark of HPS. There is currently no medical therapy approved for HPS. Oxygen therapy is recommended for patients with severe hypoxemia.

Vascular Extracellular Vesicles Indicate Severe Hepatopulmonary Syndrome in Cirrhosis

Hepatopulmonary syndrome (HPS) is a pulmonary vasculature complication in the setting of liver disease that is characterized by pathological vasodilation resulting in arterial oxygenation defects. We investigated the role of extracellular vesicles (EV) in cirrhosis patients with HPS, as well as the functional effect of EV administration in a common bile duct ligation (CBDL) HPS mouse model. A total of 113 cirrhosis patients were studied: 42 (Gr. A) with HPS and 71 (Gr. B) without HPS, as well as 22 healthy controls. Plasma levels of EV associated with endothelial cells, epithelial cells, and hepatocytes were measured. The cytokine cargoes were estimated using ELISA. The effect of EV administered intranasally in the CBDL mouse model was investigated for its functional effect in vascular remodeling and inflammation. We found endothelial cells (EC) associated EV (EC-EV) were elevated in cirrhosis patients with and without HPS (p < 0.001) than controls. EC-EV levels were higher in HPS patients (p = 0.004) than in those without HPS. The epithelial cell EVs were significantly high in cirrhosis patients than controls (p < 0.001) but no changes found in patients with HPS than without. There was a progressive increase in EC-EV levels from mild to severe intrapulmonary shunting in HPS patients (p = 0.02 mild vs. severe), and we were able to predict severe HPS with an AUROC of 0.85; p < 0.001. An inverse correlation of EC-EVs was found with hemoglobin (r = -0.24; p = 0.031) and PaO2 (r = 0.690; p = 0.01) and a direct correlation with MELD (r = 0.32; p = 0.014). Further, both TNF-α (p = 0.001) and IL-1β (p = 0.021) as cargo levels were significantly elevated inside the EVs of HPS patients than without HPS. Interestingly, upon administration of intranasal EVs, there was a significant decrease in Evans blue accumulation and lung wet-dry ratio (p = 0.042; 0.038). A significant reduction was also noticed in inflammation and cholestasis. High levels of plasma EC-EV levels were found in patients with HPS with elevated pro-inflammatory cytokine cargoes. EC-EVs were indicative of severe HPS condition. In the CBDL HPS model, we were able to prove the beneficial effects of improving vascular tone, inflammation, and liver pathogenesis.

Pulmonary vascular disease and exercise hemodynamics in chronic liver disease

Background & aimsPortopulmonary 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. MethodsWe 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. ResultsTwo-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. ConclusionIn 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.

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

To determine the prevalence and prognostic impact of hepatopulmonary syndrome (HPS) in patients with unresectable hepatocellular carcinoma (HCC) undergoing transarterial chemoembolization (TACE). 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. 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. Mild or moderate HPS is common in patients with unresectable HCC undergoing TACE, but it does not seem to have a significant prognostic impact.

211.7: Is Pulse Oximetry a Good Screening Test for Hepatopulmonary Syndrome in Liver Transplant Candidates?

Background: Pulse oximetry with oxygen saturation (SpO2) ≤96% is recommended as a screening test to identify patients (pts) with severe hepatopulmonary syndrome (HPS) to be eligible for MELD exceptions. However, diagnosis of milder forms requires contrast-enhanced echocardiography (CE-TTE) and arterial blood gas (ABG). Goals: 1) To investigate the prevalence of intrapulmonary vascular dilatations (IPVD) and HPS in pts with cirrhosis evaluated for liver transplantation (OLT) and 2) To compare the results of SpO2 with ABG measurements. Methods: The study included 972 consecutive pts (2013-2021) who completed pre-OLT evaluation including CE-TTE, SpO2 and ABG. IPVD was defined as the appearance of microbubbles in the left heart within 3-5 cardiac cycles and HPS by an alveolar arterial O2 gradient ≥ 15 mm Hg or ≥ 20 if age > 64 and absence of pulmonary chronic diseases. HPS was considered as mild, moderate or severe according to PaO2 (>80, 60-80 and <60 respectively). Results: The prevalence of IPVD was 15.4% (150/972) and of HPS 7.8% (76/972). Age of pts with HPS was 47±3 years (57% males) and MELD-Na 17± 1.HCV, alcoholism and autoimmune hepatitis were the most frequent etiologies of cirrhosis (22% each). Among pts with HPS 59 (78%) were mild (PaO2 108 ±22), 13 (17%) moderate (73 ±4) and 4 severe (55.5±3). SpO2 was ≤96% in 26% (20/76), 15% in mild (9/59), 54% in moderate (8/13) and 100% in severe (4/4) HPS. Unexpectedly, of 20 pts with Spo2 ≤96% only 4 had PaO2 <60 mmHg. Conclusions: Universal testing with CE-TTE followed by ABG in patients with cirrhosis evaluated for OLT resulted in a lower prevalence than reported of HPS, most likely due to the preponderance of mild clinical forms with normal SpO2. Using the recommended cut-off value of Spo2 ≤96% we found a poor correlation between pulse oximetry and ABG measurement.

P9.41: Outcomes of Live Donor Liver Transplantation in Adult Patients With HPS – 10-Year Experience From a Single Centre: A Retrospective Analysis

Introduction: Hepatopulmonary syndrome (HPS) is characterised by hypoxia and is associated with a worse prognosis in patients with cirrhosis. Liver transplantation is the only definitive treatment. We report our 10 year experience of live donor liver transplant in adult patients with HPS. Methods: Seventeen patients with HPS (age ≥ 18 years) and cirrhosis who underwent LDLT during the period 2012 – 2022 were retrospectively analysed. HPS was defined as PaO2 < 80 mmHg in presence of demonstrable macro-aggregated albumin (MAA) scan shunt fraction >6%. Results: The study group composed of 15 male and 2 female patients, mean age 51.05 ± 7.67 years. The most common presentations was dyspnoea, seen in 82.35% patients followed by ascites (76.47%) and gastrointestinal bleed (41.18%). Mean Child Pugh score was 10.52 ± 1.77, model for end-stage liver disease (MELD) score was 20.35 ± 7.38. One patient had very severe HPS (PaO2 <50 mmHg), four had severe HPS (PaO2 50 - 60 mmHg) and twelve had moderate HPS (PaO2 60 - 80 mmHg). The mean MAA shunt fraction was 24.05 ± 18.52% and mean PaO2 was 66.77 ± 10.63 mmHg. All patients underwent right lobe LDLT. The overall time to extubation was 1.23 ± 0.83 days and for ICU stay8.66 ± 0.55 days. Patients received oxygen for a median of 3 [2 – 5] days post-transplant. The main complications in post-LT course were sepsis and neurological, both seen in 3 patients each (17.64%). Overall survival at a median follow up of 54.5 [17 – 76] months was 82.35%. Conclusion: LDLT in HPS is feasible and is associated with good outcomes.

Liver histology in short telomere syndrome: A case report and review of the literature

Abstract Introduction/Objective Short telomere syndrome (STS) is a genetically inherited syndrome resulting in premature telomere shortening. STS encompasses a spectrum of clinical manifestations, including dyskeratosis congenita, premature hair graying, bone marrow failure, immunodeficiency, pulmonary fibrosis and liver disease. Liver histopathologic features in STS patients have not been well characterized. Methods/Case Report A 46-year-old man presented for dyspnea and cryptogenic cirrhosis. He had a complicated medical history significant for immune thrombocytopenic purpura and splenectomy, recurrent respiratory tract infections, pneumonia, sepsis, primary immunodeficiency, pulmonary mucosa-associated lymphoid tissue lymphoma, and severe hepatopulmonary syndrome. He and his brother had gray hair in their late 20s. He also had a long history of intermittently elevated liver enzymes starting at age 33. A liver biopsy performed 12 years before showed chronic portal inflammation with hepatocellular damage without significant fibrosis. These clinical manifestations prompted an evaluation for a possible telomere biology disorder, which revealed the telomere length was critically short and fell at or below the first percentile for age, supportive of the diagnosis. The most recent liver biopsy showed marked portal lymphocytic inflammation with interface hepatitis, bile ductular reaction and frequent foci of lobular inflammation with focal hepatocyte dropout. Some hepatocytes around the portal tracts were swollen with feathery degeneration and occasional Mallory-Denk bodies. A Rhodanine stain highlighted copper granules in the periportal hepatocytes, suggesting chronic cholestasis. Trichrome and reticulin stains demonstrated portal/periportal/pericellular/perisinusoidal fibrosis and focal bridging fibrosis. Results (if a Case Study enter NA) NA Conclusion Partly due to the rarity of STS and the risk of bleeding associated with biopsies, liver histology was described in only few limited studies with small samples of STS patients, including inflammation, nodular regenerative hyperplasia, steatohepatitis, hemosiderosis, cholestasis, cirrhosis, and large cell change of hepatocytes. Our case and others suggest liver disease associated with STS demonstrates a spectrum of histopathology. Being aware of these histomorphologic features in STS is important for establishing the correct diagnosis.

Clinical outcomes and risk factors of hepatopulmonary syndrome in children

Hepatopulmonary syndrome (HPS) is defined as three distinct features: liver disease, hypoxemia, and intrapulmonary vasodilation. The purpose of this study was to investigate the clinical outcomes of pediatric HPS and to identify the risk factors for HPS in children with biliary atresia (BA). We performed a retrospective cohort study of all children who were diagnosed with HPS between 2000 and 2018 at Seoul National University Hospital. The clinical features and outcomes of the 10 patients diagnosed with HPS were reviewed. To clarify the risk factors of HPS in patients with BA, we reviewed 120 patients diagnosed with BA. Underlying liver disease was BA in 8 patients, portal vein agenesis in 1 patient, and portal vein thrombosis in 1 patient. A total of 7 patients underwent liver transplantation (LT). Currently, all seven patients, including 3 patients with severe HPS, survived after LT. The prevalence of HPS in children with BA was 7%. Polysplenia/interrupted inferior vena was the only risk factor for HPS in BA patients in multivariate analysis. The Pediatric End-Stage Liver Disease score was not associated with the development of HPS. Children with severe HPS undergoing LT had excellent outcomes. Screening for HPS in children with BA is required regardless of the severity of liver diseases.

Pediatric liver transplantation in severe hepatopulmonary syndrome and use of inhaled nitric oxide for post-transplant hypoxemia-a single center experience.

Data on pediatric patients with HPS undergoing LT are limited. Our aim was to study the spectrum and outcomes of pediatric patients with HPS undergoing LDLT. The role ofiNO for post-LDLT refractory hypoxemia was also assessed. Patients (aged<18years) undergoing LT were retrospectively studied. HPS was diagnosed based on European Respiratory Society Taskforce 2004 criteria. HPS was graded based on oxygenation criteria and contrast-enhanced echocardiogram. Post-operative course was studied. Refractory post-operative hypoxemia was treated with iNO by institutionally developed protocol. 23/150 pediatric patients undergoing LDLT had HPS. BA was the most common underlying cause (52.2%). By oxygenation criteria, 6 (26.1%) had VS-HPS. VS-HPS was associated with longer LOS (p=.031) and prolonged oxygen requirement (p=.001) compared with other HPS patients. 4/6 patients with VS-HPS had pO2<45mmHg. Among these, 2 developed ICH post-operatively and 1 died. 3 developed refractory post-operative hypoxemia, successfully treated with iNO. Mean duration of iNO was 26.3days. In the group of patients with HPS, the incidence of HAT and portal vein thrombosis was 17.3% and 4.3%, respectively. One year post-LDLT survival of patients with HPS was similar to non-HPS patients (86.9% vs 94.4%; p=.88). We concluded that, pediatric patients with VS-HPS, especially those with pre-operative pO2<45mmHg, have long and difficult post-LT course. Refractory postoperative hypoxemia can be successfully overcome with strategic use of iNO. Vigilant monitoring and good intensive care support are essential.

Use of L-Arginine for inhaled Nitric Oxide (iNO) dependent hepatopulmonary syndrome (HPS) Post-Liver Transplant

Hepatopulmonary syndrome (HPS) is one of the recognised complications of liver cirrhosis which warrants urgent liver transplantation. We report a case of 7 year old girl who underwent orthotopic liver transplantation (OLT) for severe HPS. Post transplantation the patient had a stormy respiratory course and became severely hypoxemic. She was started on inhaled NO and showed a good response. However, the patient then became NO dependent and multiple attempts to wean iNO failed albeit minimal ventilatory requirements. Following IV L-Arginine infusion, iNO could be weaned within 6 hours without recurrence of hypoxemia. L-Arginine infusion should be considered as a treatment option when facing difficulties to wean iNO in an otherwise well responding patient.

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Management in Liver Transplantation in the Horizon 2020

Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary microvasculature dilatation that causes intrapulmonary shunting and leads to a gas exchange abnormality in the presence of liver diseases, which is the most common cause of respiratory insufficiency in these patients. HPS doubles the risk of death, and liver transplantation (LT) is the only curative therapeutic option so it should be considered in patients with severe HPS, with excellent survival rates post-LT. However, pretransplant Pao2 <45 mm Hg has been associated with an increase in post-transplant morbidity and mortality, but it does not imply a contraindication for LT. The resolution of HPS usually occurs within 6 months post-LT, but it can take 1 year.Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension with or without liver disease in the absence of other causes of PAH. The prevalence of PoPH is 5% to 10% among liver transplant (LT) candidates. The impact of LT on PoPH is unpredictable. Therefore, despite conferring a high morbidity and mortality, PoPH itself is not an indication for liver transplantation. It may be considered a contraindication for LT in severe cases.

Living donor liver transplantation: An answer to severe hepatopulmonary syndrome in cirrhosis

Living donor liver transplantation: An answer to severe hepatopulmonary syndrome in cirrhosis

RESOLUTION OF HEPATOPULMONARY SYNDROME WITH SUCCESSFUL TREATMENT OF CHRONIC HEPATITIS C INFECTION

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Hepatopulmonary syndrome (HPS) is a serious complication of liver disease where pulmonary vasculature dilates causing hypoxia and dyspnea. The following case describes a patient had reversal of their HPS after successful treatment of their chronic hepatitis C virus (HCV) infection. CASE PRESENTATION: A 49-year-old female presented to the St. Paul Hospital in Saskatoon, Canada for hypoxemia. Her past medical history includes untreated human immunodeficiency virus (HIV) and HCV genotype 3 with associated cirrhosis. At presentation, the patient had a SpO2 of 78% on room air which improved to 88% with administration of 5L supplemental oxygen. HPS was suspected and a thoracic CT was performed which demonstrated enlargement of the pulmonary arterioles in the lower lung zones. The diagnosis of HPS was confirmed with a contrast echo demonstrating bubbles in the left atrium after approximately 4 cardiac cycles suggesting a large shunt. For symptomatic management , the patient was started on 4L of home O2 and referred to infectious diseases for management of the patients HIV and HCV. The patient was started immediately on emtricitabine, tenofovir alafenamide and dolutegravir for her HIV, developing undetectable viral loads and a CD4 count of 80 within 3 months of treatment. For her HCV, the patient was later started on a 12-week course of sofosbuvir, velpatasvir and ribavarin, which was confirmed to have eliminated her HCV infection at 24 weeks post-treatment. Following eradication of the patients HCV infection, the patient's hypoxia had also resolved with a measured SpO2 of 94% on room air. A follow-up contrast echo demonstrated few bubbles in the left atrium following 10 cardiac cycles, therefore suggesting successful treatment of HPS. DISCUSSION: HPS is characterized by hypoxia secondary to intrapulmonary vascular dilatations which cause an apparent right to left intrapulmonary shunt1. The syndrome leads to progressive hypoxemia and significant morbidity. There is currently no effective medical therapy for HPS, and liver transplantation is the only definitive treatment available2,3. Globally HCV infection is a major contributor to chronic liver disease, and therefore HPS. There has also been evidence of HPS occurring in non-cirrhotic patients with chronic viral hepatitis 4. With the introduction of direct-acting antivirals (DAAs), treatment of HCV has become more efficacious and well tolerated. The potential for successful treatment with DAAs to resolve HPS could lead to significant medical advances for the treatment of HPS. CONCLUSIONS: In conclusion this case report describes the successful reversal of HPS following the treatment of chronic HCV infection. These findings suggests that further research may discover a role for the use of DAAs in treatment of HPS. Reference #1: Grilo-Bensusan, I., & Pascasio-Acevedo, J. M. (2016). Hepatopulmonary syndrome: What we know and what we would like to know. World journal of gastroenterology, 22(25), 5728. Reference #2: Gupta, S., et al. (2010). Improved survival after liver transplantation in patients with hepatopulmonary syndrome. American Journal of Transplantation, 10(2), 354-363. Reference #3: Tzovaras, N., et al. (2006). Reversion of severe hepatopulmonary syndrome in a non cirrhotic patient after corticosteroid treatment for granulomatous hepatitis: a case report and review of the literature. World journal of gastroenterology: WJG, 12(2), 336. DISCLOSURES: No relevant relationships by Adam Bloom, source=Web Response Advisory Committee Member relationship with Gilead Please note: $1001 - $5000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with Gilead Please note: $1001 - $5000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Honoraria Grant relationship with Gilead Please note: $20001 - $100000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Grant/Research Support Advisory Committee Member relationship with Abbvie Please note: $1001 - $5000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Consulting fee grant relationship with Abbvie Please note: $5001 - $20000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Grant/Research Support Grant relationship with Viiv Please note: $1001 - $5000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Grant/Research Support Advisory Committee Member relationship with Merck Please note: $1001 - $5000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Consulting fee Grant relationship with Merck Please note: $5001 - $20000 Added 03/23/2019 by Kris Stewart, source=Web Response, value=Grant/Research Support No relevant relationships by Chung Chun Tyan, source=Web Response

High-Dose Oral Garlic is Associated with a Decrease in Intrapulmonary Shunting in Severe Hepatopulmonary Syndrome.

High-Dose Oral Garlic is Associated with a Decrease in Intrapulmonary Shunting in Severe Hepatopulmonary Syndrome.

22. Living Donor Liver Transplantation for Moderate to Very Severe Hepatopulmonary Syndrome: An Experience of 24 Patients

Background and Aims: Hepatopulmonary syndrome (HPS) in patients with cirrhosis worsens the prognosis and liver transplantation is only definitive treatment. In countries with less cadaveric donation, patients with HPS have risk of significantly worsening and mortality while on waiting list. Living donor liver transplantation (LDLT) offers option of early transplantation. Methods: HPS was confirmed in presence of low PaO2 and macro-aggregated albumin (MAA) scan shunt fraction >6%; severity of HPS was defined as moderate (PaO2 >60 to < 80), severe (PaO2 >50 to < 60) and very severe (PaO2< 50). Twenty five patients were planned for LDLT; one surgery was abandoned due to persistent hypoxia, and 24 underwent LDLT for HPS and were included in retrospective analysis. Results: The study group comprised of 21 males and 3 females; mean age 50±8.7 years. Most common presentation was progressive dyspnea. Prior to LDLT, the mean model for end-stage liver disease score (without extra points) was 16.9±4.5 and Child's score was 9.2±2.1. The commonest etiology of cirrhosis was cryptogenic (n = 11,45.8%). The mean PaO2 was 59 mm Hg, and the mean shunt fraction was 22%. Seven patients (29.1%) had very severe while five (20.8%) had severe HPS. All patients underwent right lobe LDLT. The median overall time to extubation was 2 days (range 1–32) and ICU stay was 6 days (3–20). The main complications in post-LT course were bacterial infection in 6 (25%), CMV viremia in 5 (20.8%), acute cellular rejection in 2 (8.3%) and biliary leak/stricture in 1 (4%) patients. One patient had mortality in very severe group due to sepsis in first week after LDLT, rest are doing well at a follow up of 71±41 months. Seventeen LDLT recipients were off oxygen at the time of discharge from hospital. Conclusions: We report one of the largest series of LDLT in HPS with good results even in very severe cases. The authors have none to declare.

Ischemic Cholangiopathy: Insight From an Explant Liver Allograft Affected by Severe Post-transplant Hypoxemia Due to Hepatopulmonary Syndrome: A Case Report

Increased biliary complications in hepatopulmonary syndrome (HPS) have been hypothesized due to post-transplant hypoxemia. Supporting this hypothesis, we report histopathological findings from an explant liver allograft where the recipient suffered severe and prolonged post-operative hypoxemia. A 4-year-old child underwent liver transplantation (LT) for decompensated chronic liver disease complicated by severe HPS. The post-operative period was complicated by severe prolonged hypoxemia. HPS resolved completely 6 months after LT only to recur 3 months later due to graft dysfunction. The child underwent retransplantation 8 months after the first LT. The explant liver showed bile duct loss along with ulceration and fibrosis of large hilar bile ducts biliary, suggestive of ischemic cholangiopathy. Based on the histopathology findings, we suggest that severe prolonged hypoxemia during post-transplant period could cause ischemic cholangiopathy, which can lead to biliary complications.

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

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. 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. 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). HPS is a common complication of cirrhosis in children. A combination of clinical and imaging criteria should be used to diagnose HPS.

The Frequency and Significance of Portopulmonary Venous Anastomosis (PPVA) and Transvenous Obliteration for Gastric Varices

A Portopulmonary Venous Anastomosis (PPVA) is a direct vascular connection between the portal venous system and the pulmonary veins, close to the left atrium. We describe a patient with advanced cirrhosis and moderately severe Hepatopulmonary Syndrome (HPS) in whom (PPVA) was demonstrated by a high resolution computed tomographic scan. In order to reduce the symptoms of hypoxia in our patient, we sequentially performed Transjugular Intrahepatic Portosystemic Shunt (TIPS) followed by a catheter based obliteration of the PPVA. The case raised three questions which were addressed through an extensive literature review. Among patients with advanced cirrhosis, what is the frequency of PPVA? Our literature review suggests that the frequency of finding a PPVA in patients with portal hypertension depends on the imaging technique used. Transhepatic or transvenous portal venography may demonstrate PPVA in about 20% of patients with cirrhosis and varices. Limited published experience with contrast enhanced (bubble) echocardiography suggests that PPVA may be present in about 30% of such patients. An increasing number of case reports of PPVA have been published in recent years. This observation indicates that routine use of High Resolution Computed Tomography (HRCT) and magnetic resonance imaging is able to identify PPVA in cirrhotic patients. The visualization of PPVA with HRCT depends on the timing of the contrast injection and the expertise of the viewer. The present report describes three patients with portopulmonary venous anastomosis (PPVA) in whom balloon-occluded retrograde transvenous obliteration (B-RTO) of gastric varices was attempted. No patients had a gastrorenal shunt. In one patient, after an approach from the inferior phrenic vein (IPV), the PPVA was embolized with the use of microcoils. Portopulmonary Venous Anastomosis Detected at Balloon-occluded Retrograde Transvenous Obliteration for Gastric Varices among cirrhotic patients with PPVA, what is the clinical significance of the PPVA? There are multiple causes of clinically significant hypoxia in patients with cirrhosis. A PPVA is a right to left shunt that, theoretically, could be associated with clinically significant systemic arterial hypoxemia or emboli. There is not definitive evidence in the published literature that PPVA alone causes clinically significant hypoxia in cirrhotic patients. PPVA is, however, a documented important risk factor for systemic emboli when needle or catheter techniques are used to treat or prevent bleeding from gastroesophageal varices.