• Maria Lazaro Salvador, Carlos A Quezada Loaiza, Rodríguez Padial Luis L Cardiology Department, Hospital Virgen de la Salud, Toledo, Spain., Joan A Barberá, Manuel López-Meseguer, Raquel López-Reyes, Ernest Sala-Llinas, Sergio Alcolea, Isabel Blanco, Pilar Escribano-Subías, and REHAP Investigators.
• Cardiology Department, Hospital Virgen de la Salud, Toledo, Spain.
• Intern Med J. 2021 Mar 1; 51 (3): 355-365.

BackgroundPortopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic/familial pulmonary arterial hypertension (IPAH/FPAH).AimsTo compare the characteristics, survival, prognostic factors and management of PoPH and IPAH/FPAH patients and to assess the impact of treatment on survival of PoPH patients.MethodsAnalysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first-line PAH-targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients).ResultsCompared to IPAH/FPAH patients (n = 678), patients with PoPH (n = 237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age- and sex-adjusted 5-year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (P < 0.001). Treated PoPH had better survival than non-treated. PAH- and liver-related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first-line PAH-targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first-line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation.ConclusionsPoPH patients are undertreated and show poorer survival than IPAH/FPAH patients. First-line treatment with PAH-targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.© 2020 Royal Australasian College of Physicians.

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