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

Abstract

Abstract 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.