Portopulmonary hypertension in the current era of pulmonary hypertension management.

Journal of Hepatology
L SavaleO Sitbon

Abstract

Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current era of pulmonary hypertension management. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, survival and predictors of death in a large contemporary cohort of patients with PoPH. Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected. The effect of initial treatment strategies on functional class, exercise capacity and cardiopulmonary haemodynamics were analysed. Survival and its association with PAH- and hepatic-related characteristics were also examined. Six hundred and thirty-seven patients (mean age 55 ± 10 years; 58% male) were included. Fifty-seven percent had mild cirrhosis, i.e. Child-Pugh stage A. The median model for end-stage liver disease (MELD) score was 11 (IQR 9-15). Most patients (n = 474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 inhibitor (n = 336) or with an endothelin-receptor antagonist (n = 128); 95 (15%) were initiated on double oral combination therapy and 5 (1%) on triple therapy. After a median treatment time of 4.5 months, there were significant improvements in functional ...Continue Reading

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Citations

Jun 10, 2020·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Hilary M DuBrock, Michael J Krowka
Nov 24, 2020·Frontiers in Medicine·Christopher ThomasSandeep Sahay
Mar 18, 2021·BMC Pulmonary Medicine·Yukiko TakahashiKoichiro Tatsumi
Jun 5, 2021·Advanced Drug Delivery Reviews·Enis KostallariPierre-Emmanuel Rautou
May 9, 2021·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Arun JoseJean M Elwing
Jun 15, 2021·Transplantation Direct·Sandeep SahayAdriano R Tonelli
Aug 25, 2021·Pulmonary Pharmacology & Therapeutics·Rosario RossiGiuseppe Boriani
Sep 5, 2021·Mayo Clinic Proceedings·Charles D BurgerRobert P Frantz
Aug 16, 2021·Liver Transplantation : Official Publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society·Sarah Raevens, Michael B Fallon
Sep 18, 2021·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Takumi KawaguchiYoshihiro Fukumoto
Oct 17, 2020·Transplantation·Rodrigo Cartin-CebaMichael J Krowka
Jun 30, 2021·American Journal of Respiratory and Critical Care Medicine·Athénaïs BouclyOlivier Sitbon

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