Gender Differences in Portopulmonary Hypertension.

Abstract Background Portopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, can lead to right heart failure and death. Female sex is a known risk factor for POPH, but little is known about the impact of gender on clinical manifestations, hemodynamics, treatment response and survival. Research Question We sought to characterize gender differences in clinical characteristics, pulmonary hemodynamics, treatment response and survival in patients with POPH. Study Design and Methods: We performed a retrospective cohort study of adult liver transplant (LT) candidates with POPH within the Organ Procurement Transplantation Network database. Females and males were compared. Multivariate regression was performed to assess the association between gender and pulmonary vascular resistance (PVR) and survival. Patients were also stratified by age (50 years) to determine how age modifies the relationship between gender and hemodynamics and survival. Results 190 adults (103 males, 87 females) were included. Compared to males, females had a lower Model for End Stage Liver Disease (MELD) score (12.1±4.2 versus 13.8±4.9, p=0.01) and were more likely to have autoimmune liver disease. Females had a higher baseline PVR (610.6±366.6 versus 461.0±185.3 dynes-s-cm-5, p 0.05). When stratified by age, female gender was independently associated with worse waitlist survival in younger patients (HR 6.61, 95%CI 1.25-35.08, p=0.03) but not older patients. Interpretation Compared to males, female LT candidates with POPH had a higher PVR, lower MELD score and were more likely to have autoimmune liver disease. Females and males had similar overall survival, but female gender was associated with worse survival in younger patients.