Echocardiographic evidence of an intrapulmonary shunt in a patient with severe liver cirrhosis.
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ABSTRACT:
SUBMITTER: Dobbermann H
PROVIDER: S-EPMC8238719 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:We reported here four cases presenting with disturbance of consciousness over long periods of time and hyperammonemia. Two patients were on maintenance hemodialysis. Contrast-enhanced computed tomography (CT) of abdomen and balloon-occluded retrograde contrast venography revealed existence of a non-cirrhotic portosystemic shunt. Conservative treatment such as intravenous branched-chain amino acid administration and oral lactulose administration had only a modest effect in all patients. Improvements in symptoms were observed following the occlusion of the shunt path in three patients. Measurements of ammonia values would be the most important test for screening, but changes in Fischer's ratio or indocyanine green (ICG) test values were also correlated with clinical symptoms. Neurologists should keep in mind the possibility of non-cirrhotic portosystemic shunts when they encounter patients with disturbance of consciousness. They should also remember that occlusion of the shunt pathway is an effective treatment.
| S-EPMC8761820 | biostudies-literature
Project description:Sarcopenia is common in patients with liver cirrhosis and related to higher mortality. Implantation of a transjugular intrahepatic portosystemic shunt (TIPS) is a feasible method for reducing cirrhosis-related portal hypertension, but also possible improvement of the patient`s muscle status. We aimed to analyze changes in muscle quantity and prevalence of sarcopenia after TIPS. We retrospectively surveyed the muscle status in 52 patients (mean age 54.2 years) before and after TIPS by evaluating skeletal (SMI) and psoas muscle indices (PMI) in CT and MR images. Model for End-Stage Liver Disease (MELD), Freiburg index of post-TIPS survival (FIPS), and their underlying laboratory parameters (e.g., Albumin) were analyzed. Prevalence of sarcopenia was 84.6%. After a median follow-up of 16.5 months after TIPS, SMI (0.020) and PMI (p < 0.001) increased, and sarcopenia decreased by 14.8% (0.109). MELD and PMI after TIPS were negatively correlated (r = - 0.536, p < 0.001). Albumin levels increased in patients with increased SMI after TIPS (p = 0.022). Confirming the positive impact of TIPS implantation on muscle indices in patients with liver cirrhosis, we found indications for improved survival and possible indications for altered metabolism with increased albumin levels in patients with increased muscle quantity.
| S-EPMC10564886 | biostudies-literature
Project description:Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a lethal neonatal lung disease characterised by severe pulmonary hypertension, abnormal vasculature and intractable hypoxaemia. Mechanisms linking abnormal lung vasculature with severe hypoxaemia in ACD/MPV are unknown. We investigated whether bronchopulmonary anastomoses form right-to-left shunt pathways in ACD/MVP. We studied 2 infants who died of ACD/MPV postmortem with direct injections of coloured ink into the pulmonary artery, bronchial artery and pulmonary veins. Extensive histological evaluations included serial sectioning, immunostaining and 3-dimensional reconstruction demonstrated striking intrapulmonary vascular pathways linking the systemic and pulmonary circulations that bypass the alveolar capillary bed. These data support the role of prominent right-to-left intrapulmonary vascular shunt pathways in the pathophysiology of ACD/MPV.
| S-EPMC4405163 | biostudies-literature
Project description:Pulmonary arteriovenous malformations are common in patients with hereditary haemorrhagic telangiectasia and can be associated with significant hypoxia and intra-pulmonary shunt. We present a case of a young man with a known 57% calculated shunt requiring abdominal surgery and the multidisciplinary decisions required in the preoperative period to minimize post-operative complications.
| S-EPMC7060891 | biostudies-literature
Project description:INTRODUCTION:Muscle mass has been shown to be a prognostic marker in patients with liver cirrhosis. Transversal psoas muscle thickness normalized by height (TPMT/height) obtained by routine computed tomography is a simple surrogate parameter for sarcopenia. TPMT/height, however, is not sex specific, which might play a role in risk stratification. Its association with acute-on-chronic liver failure (ACLF) has not been established yet. ACLF is associated with systemic inflammatory dysregulation. This study aimed at evaluating the role of sarcopenia in ACLF development of patients with decompensated cirrhosis receiving transjugular intrahepatic portosystemic shunt (TIPS) using sex-specific TPMT/height. METHODS:One hundred eighty-six patients from the prospective Non-invasive Evaluation Program for TIPS and Follow Up Network cohort (observational, real-world TIPS cohort with structured follow-up) were analyzed. TPMT/height was measured from routine computed tomography. The sex-specific cutoff was determined to classify patients as sarcopenic and nonsarcopenic for 1-year mortality after TIPS. Clinical outcome was compared. Primary end points were ACLF and 1-year mortality after TIPS. Secondary end points were development of decompensations (hepatic encephalopathy and ascites) after TIPS. RESULTS:The sex-specific cutoff increases the diagnostic accuracy with regard to primary and secondary end points compared with the unisex cutoff. Sex-specific sarcopenia classification is an independent predictor of 1-year mortality and ACLF development in patients with cirrhosis receiving TIPS. Patients in the sarcopenia group showed significantly higher rates of mortality, ascites, overt hepatic encephalopathy, and ACLF after TIPS compared with the nonsarcopenia group. The Chronic Liver Failure Consortium Acute Decompensation score as a marker of systemic inflammation was significantly higher in sarcopenic patients. CONCLUSIONS:This study demonstrates for the first time that sarcopenia is related to ACLF development and systemic inflammation. The prognostic value of TPMT/height can be improved by using sex-specific cutoffs. ClinicalTrials.gov identifier: NCT03584204.
| S-EPMC6602782 | biostudies-literature
Project description:Strong gut microbiome biomarkers for Liver cirrhosis
| PRJEB6336 | ENA
Project description:Sitosterolemia (MIM 210250) is a rare genetic disorder caused by disruption of the normal mechanisms that regulate dietary cholesterol absorption and prevent the accumulation of noncholesterol sterols. As a result of this defect, affected individuals accumulate high concentrations of plant sterols in plasma and tissues. They present clinically with tendon or tuberoeruptive xanthomas, premature coronary artery disease, and/or hemolytic anemia. Two genes, ABCG5 and ABCG8, compose the STSL locus, and complete mutation in either, but not both, results in disease. The expression of these genes is confined to the intestine and liver. They are thought to function as sterol efflux pumps. It is not clear which organ, liver or intestine, is of greater importance in maintaining sterol balance with respect to noncholesterol sterols. We report here a case of a patient who presented with "chronic active liver disease" and was found to have sitosterolemia by chance and subsequently underwent orthotopic liver transplantation. Following transplantation, the grossly elevated pretransplantation serum plant sterol levels decreased to values only slightly higher than those of the patient's heterozygous father. This case highlights 2 important features: (1) The liver functions as the predominant organ for maintaining noncholesterol sterol balance (because the intestinal defect was not altered), and (2) a new clinical feature of undiagnosed sitosterolemia may be "idiopathic" liver disease. Because the diagnosis of sitosterolemia is based on specialized plasma analyses, we would propose that some consideration to this diagnosis should be given in appropriate cases.
| S-EPMC1391914 | biostudies-literature
Project description:BACKGROUND:Unrepaired left to right atrial shunt lesions can cause significant right ventricular (RV) volume overload. The parameter pulmonary to systemic shunt volume ratio (Qp:Qs) has been shown to detect even small differences between left and right ventricular stroke volume; however, four parameters are needed for its calculation. This study was carried out to evaluate the accuracy of the single parameter right ventricular outflow tract (RVOT) velocity time integral (VTI) to identify atrial shunt lesions. METHODS:All patients who underwent transesophageal echocardiography (TEE) examination at this institution between 1 January 2013 and 1 January 2018 were retrospectively analyzed. The RVOT-VTI was measured in the transthoracic echocardiography performed immediately before each TEE. The diagnostic accuracy for detection of atrial shunt lesions was tested. RESULTS:A total of 2797 patients with a median age of 67 years (interquartile range, IQR 54-77 years) were included in the final analysis. A total of 113 (4%) patients had a relevant atrial shunt lesion. The mean RVOT-VTI of the shunt group was 25 cm (SD ± 8.1 cm) and was significantly higher than that of the non-shunt group with 17 cm (SD ± 4.8 cm) (p < 0.001). The area under the curve (AUC) was 0.81. A total of 106 patients (93.8%) of the shunt group had a VTI of ≥16 cm. If the RVOT-VTI was <16 cm, the negative predictive value was 99.3%. If the RVOT-VTI was ≥25 cm, 22% of patients proved to have a significant shunt lesion. CONCLUSION:In this large retrospective analysis it could be shown that a low RVOT-VTI predicted the absence of significant atrial shunt lesions, while a high RVOT-VTI predicted the presence. The parameter should be applied in all patients with suspected atrial shunt lesions where calculation of Qp:Qs is impossible.
| S-EPMC7297847 | biostudies-literature
Project description:BACKGROUND & AIMS:The management of patients with refractory ascites (RA) is challenging, particularly at higher age. Transjugular intrahepatic portosystemic shunt (TIPS) is an established treatment for RA, but safety data in elderly patients are rare. Our aim was to evaluate the safety and feasibility of TIPS in elderly patients with RA. METHODS:Overall, 160 consecutive cirrhotic patients receiving a TIPS for RA at Hannover Medical School between 2012 and 2018 were considered for this retrospective analysis. Periinterventional complications such as acute-on-chronic liver failure (ACLF) as well as survival were compared between patients <65 and ?65 years. Propensity score matching was conducted to match elderly TIPS patients and patients treated with paracentesis. RESULTS:A number of 53 out of the 160 patients were ?65 years (33%). Periinterventional course in those ?65 years appeared to be slightly more complicated than in <65 years as reflected by a significantly longer hospital stay (p = 0.030) and more ACLF-episodes (21% vs. 9%; p = 0.044). 28-day mortality was similar between both groups (p = 0.350), whereas survival of the younger patients was significantly higher at 90 days (p = 0.029) and numerically higher at 1 year (p = 0.171). In the multivariate analysis age ?65 years remained an independent predictor for 90-day mortality (HR: 2.58; p = 0.028), while it was not associated with 28-day and 1-year survival. Importantly, after matching for potential confounders 1-year survival was similar in elderly patients if treated with TIPS or paracentesis (p = 0.419). CONCLUSIONS:TIPS placement in elderly patients with RA appears to be slightly more complicated compared to younger individuals, but overall feasible and at least not inferior to paracentesis.
| S-EPMC7316253 | biostudies-literature
Project description:BackgroundTreatment of refractory ascites in liver cirrhosis is challenging. Transjugular intrahepatic portosystemic shunt and alfapump® have been proposed for the management, but few data comparing both exist.AimsThe aim of this study was to evaluate the characteristics and outcomes of patients treated with transjugular intrahepatic portosystemic shunt and alfapump® for refractory ascites at our centre.MethodsAll consecutive patients were retrospectively reviewed for baseline characteristics, efficacy of treatment, complications and survival.ResultsIn total, 19 patients with transjugular intrahepatic portosystemic shunt and 40 patients with alfapump® were included. Patients with transjugular intrahepatic portosystemic shunt had better liver function and less hepatic encephalopathy at baseline. Fifty-eight per cent of patients developed hepatic encephalopathy in the first six months after transjugular intrahepatic portosystemic shunt. In patients with alfapump®, renal function decreased and 58% developed prerenal impairment and 43% hepatorenal syndrome in the first six months. Alfapump® patients with new catheters required less reinterventions (26% versus 57% with old catheters, p = 0.049). Transplant-free survival at 1 year was 25% in alfapump® and 65% in transjugular intrahepatic portosystemic shunt. Hepatic encephalopathy predicted transplant-free survival in patients with alfapump® (hazard ratio 2.00, 95% confidence interval 0.99-4.02, p = 0.05). In a sensitivity analysis comparing patients with similar liver function, the rate of hepatorenal syndrome and prerenal impairment was higher in patients with alfapump® and these patients were hospitalised more frequently, whereas the rate of hepatic encephalopathy was similar in both treatment groups.ConclusionsBoth transjugular intrahepatic portosystemic shunt and alfapump® were effective treatments for refractory ascites in cirrhosis. Patients treated with transjugular intrahepatic portosystemic shunt had a better one-year transplant-free survival but had less negative prognostic factors at baseline. Selecting patients without hepatic encephalopathy prior to implantation of an alfapump® might improve transplant-free survival.
| S-EPMC7707873 | biostudies-literature