Liver abnormalities in pulmonary arterial hypertension

Nickel, Nils; Galura, Gian; Zuckerman, Marc J.; Hakim, M. Nawar; Alkhateeb, Haider; Mukherjee, Debabrata; Austin, Eric D.; Heresi, Gustavo A.

doi:10.1177/20458940211054304

Cited by 17 publications

(21 citation statements)

References 105 publications

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“…The lung-liver relationship is reciprocal, and liver disease clearly has implications on the anatomy and function of the pulmonary circulation, as seen in porto-pulmonary hypertension, hepato-pulmonary syndrome (5) and Fontan physiology(6). Vice versa, right heart strain has signi cant clinical implications on liver function and liver dysfunction is common in PAH patients (4). The exact pathophysiology of liver dysfunction in PAH is poorly understood: hepatic congestion from right heart pressure overload and alteration in in ammatory, hormonal and genetic pathways have been implicated (4).…”

Section: Resultsmentioning

confidence: 99%

“…Vice versa, right heart strain has signi cant clinical implications on liver function and liver dysfunction is common in PAH patients (4). The exact pathophysiology of liver dysfunction in PAH is poorly understood: hepatic congestion from right heart pressure overload and alteration in in ammatory, hormonal and genetic pathways have been implicated (4). Non-alcoholic fatty liver disease (NAFLD) is a clinicopathologic liver disease with a wide histological spectrum ranging from simple steatosis to cirrhosis and is associated with increased morbidity and mortality(8) .…”

Section: Resultsmentioning

confidence: 99%

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The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

Kalas

Khatab

Galura

et al. 2022

Preprint

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Background:Pulmonary arterial hypertension (PAH) is a deadly cardiopulmonary disease with multi-organ involvement including impaired liver function. Liver dysfunction in PAH is poorly understood but significantly associated with morbidity and mortality. Hispanics have a significantly higher prevalence of non-alcoholic fatty liver disease (NAFLD) and evidence of more advanced disease in comparison to other ethnic groups. The clinical impact of NAFLD in Hispanic PAH patients is unknown. We aimed to investigate the impact of a validated scoring system, non-alcoholic fatty liver disease fibrosis (NFS) score, to predict the degree of liver fibrosis in a Hispanic PAH population and its relationship to hemodynamics, functional class, and outcomes. Methods: A retrospective review of all treatment naïve Hispanic patients with group I WHO pulmonary hypertension (PH) at a single academic center between February 2016 and March 2021 was performed. Patients with history of substance or alcohol abuse, non-group I WHO PH, pre-existent liver disease, chronic kidney disease, atrial fibrillation, thyroid disease, and warfarin use were excluded from the study. The diagnosis of group I WHO PH was determined by cardiac catheterization after the exclusion of other etiologies. NFS scores were calculated for each patient and correlated with functional capacity, hemodynamics, NT-proBNP, and survival.Results:A total of 96 Hispanic patients were included in our study. The median age of patients in our cohort was 49 (IQR 15) and 69% of our cohort were females. Higher NFS scores indicating advanced hepatic fibrosis (F3-F4) were found to correlate with elevated right-sided cardiac filling pressures, elevated levels of NT-proBNP, lower functional capacity, and worse 5-year survival rates. Conclusion:In Hispanic patients with PAH, NFS scores correlate with the degree of right sided pressure overload. In addition, advanced fibrosis scores were independently associated with lower 5-year survival rates and added prognostic information to other established risk parameters in PAH. This study suggests that screening for liver disease in this vulnerable patient population can aid in earlier detection and possible intervention, thus leading to potential improvement in survival rates.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

Kalas

Khatab

Galura

et al. 2022

Preprint

Self Cite

View full text Add to dashboard Cite

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Section: Discussionmentioning

confidence: 99%

“…Due to the elevations in right ventricular (RV) pressures in PH, upstream clinical and biochemical effects are expected. The liver is one of the rst organs affected by elevated RV pressures resulting in abnormalities in liver biochemical tests and ascites (4). The lung-liver relationship is reciprocal: elevated right-sided lling pressures from PH are associated with congestive hepatopathy.…”

Section: Introductionmentioning

confidence: 99%

The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

Kalas

Khatab

Galura

et al. 2023

Preprint

Self Cite

View full text Add to dashboard Cite

Background: Pulmonary arterial hypertension (PAH) is a deadly cardiopulmonary disease with multi-organ involvement including impaired liver function. Liver dysfunction in PAH is poorly understood but significantly associated with morbidity and mortality. Hispanics have a significantly higher prevalence of non-alcoholic fatty liver disease (NAFLD) and evidence of more advanced disease in comparison to other ethnic groups. The clinical impact of NAFLD in Hispanic PAH patients is unknown. We aimed to investigate the impact of a validated scoring system, non-alcoholic fatty liver disease fibrosis (NFS) score, to predict the degree of liver fibrosis in a Hispanic PAH population and its relationship to hemodynamics, functional class, and outcomes. Methods: A retrospective review of all treatment naïve Hispanic patients with group I WHO pulmonary hypertension (PH) at a single academic center between February 2016 and March 2021 was performed. Patients with history of substance or alcohol abuse, non-group I WHO PH, pre-existent liver disease, chronic kidney disease, atrial fibrillation, thyroid disease, and warfarin use were excluded from the study. The diagnosis of group I WHO PH was determined by cardiac catheterization after the exclusion of other etiologies. NFS scores were calculated for each patient and correlated with functional capacity, hemodynamics, NT-proBNP, and survival. Results: A total of 96 Hispanic patients were included in our study. The median age of patients in our cohort was 49 (IQR 15) and 69% of our cohort were females. Higher NFS scores indicating advanced hepatic fibrosis (F3-F4) were found to correlate with elevated right-sided cardiac filling pressures, elevated levels of NT-proBNP, lower functional capacity, and worse 5-year survival rates. Conclusion: In Hispanic patients with PAH, NFS scores correlate with the degree of right sided pressure overload. In addition, advanced fibrosis scores were independently associated with lower 5-year survival rates and added prognostic information to other established risk parameters in PAH. This study suggests that screening for liver disease in this vulnerable patient population can aid in earlier detection and possible intervention, thus leading to potential improvement in survival rates.

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“…In recent years, PAH has shown to be a multiorgan systemic disorder hallmarked by anomalies in the immune system, skeletal muscle, kidneys, peripheral and central nervous system, as well as systemic circulation ( 2 , 3 ). Due to the liver’s close physiological and anatomical link to the RV, it is among the vulnerable organs to be affected by RV failure from PAH, which can lead to a variety of liver abnormalities ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

Abnormal Liver Function Tests Were Related to Short- and Long-Term Prognosis in Critically Ill Patients With Primary Pulmonary Hypertension

Wang

Huang²,

Xu³

et al. 2022

Front. Cardiovasc. Med.

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AimThe aim of this study was to examine the utility of liver function tests (LFTs) in predicting the prognosis of critically ill patients with primary pulmonary hypertension (PPH) with/without liver disease.MethodsWe retrieved the Medical Information Mart for Intensive Care III (MIMIC-III) database to acquire clinical data. From the database, we recruited adult patients that were equal to or older than 18 years with primary pulmonary hypertension (PPH) discharge from intensive care unit (ICU). Then, the relationship between LFTs and duration of hospitalization and ICU stays was examined based on the Spearman correlation. The chi-square assessment was conducted to examine the correlation between LFTs and death rates. Survival curves were plotted with the aid of the Kaplan-Meier technique, and the curves were subsequently compared utilizing the log-rank test. The LFTs were identified as independent predictive variables of death according to the results of multivariable logistic regression. The specificity and sensitivity for mortality were calculated utilizing receiver operating characteristic (ROC) curves and the area under the curve (AUC).ResultsIn total, 198 patients satisfying the inclusion criteria were recruited, among which there were 23 patients with liver disease. Only ALB was correlated with the length of ICU stay in the total PPH group. ALB independently served as a risk variable for hospital mortality and 90-day mortality and was significantly associated with 90-day and 4-year survival rates in both total PPH and PPH without liver disease. AST was correlated with hospital mortality and 90-day survival curves in both total PPH and PPH without liver disease and independently served as a risk factor for hospital and 90-day mortality only in the total PPH group. ALT independently acted as a risk variable for hospital mortality and total bilirubin was correlated with hospital mortality in the total group. The diagnostic performance of the predictive model combining the LFTs was moderately good for the hospital, 90-day, and 4-year mortality. Both Modelı End-Stage ıLiverı Disease (MELD) score and albumin-bilirubin (ALBI) score were independent risk factors for short- and long-term prognosis. And they were also significantly associated with short- and long-term prognosis.ConclusionAmong critically ill patients with PPH and with or without liver illness, aberrant LFT was linked to short- and long-term prognoses.

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Liver abnormalities in pulmonary arterial hypertension

Cited by 17 publications

References 105 publications

The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

The association between non-clinically apparent liver fibrosis and pulmonary arterial hypertension in Hispanic patients

Abnormal Liver Function Tests Were Related to Short- and Long-Term Prognosis in Critically Ill Patients With Primary Pulmonary Hypertension

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