Sasiharan Sithamparanathan1, Arun Nair2, Logan Thirugnanasothy1, J Gerry Coghlan3, Robin Condliffe4, Konstantinos Dimopoulos5, Charlie A Elliot4, Andrew J Fisher1, Sean Gaine6, J Simon R Gibbs7, Michael A Gatzoulis5, Clive E Handler3, Luke S Howard7, Martin Johnson8, David G Kiely4, James L Lordan1, Andrew J Peacock8, Joanna Pepke-Zaba9, Benjamin E Schreiber3, Karen K K Sheares9, Stephen J Wort5, Paul A Corris10. 1. National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom. 2. National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom. 3. Pulmonary Hypertension Unit, Royal Free Hospital, London, United Kingdom. 4. Sheffield Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom. 5. Royal Brompton Pulmonary Hypertension and Adult Congenital Heart Centre, Imperial College, London, United Kingdom. 6. Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland. 7. National Heart and Lung Institute, Imperial College, London and Hammersmith Hospital, London, United Kingdom. 8. Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Glasgow, Scotland. 9. Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom. 10. National Pulmonary Hypertension Service (Newcastle), The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom. Electronic address: paul.corris@ncl.ac.uk.
Abstract
BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service. METHODS: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010. RESULTS: Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, with a balanced distribution in gender. Alcohol (n = 57) and hepatitis C (n = 10) were the most common causes of portal hypertension. Phosphodiesterase V inhibitors were the most frequently used targeted therapy, in 63.6% (n = 70) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, or year of diagnosis on survival. CONCLUSIONS: Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.
BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition associated with poor survival, and the effect of modern therapies that target pulmonary arterial hypertension (PAH) on long-term outcome is unknown. This study investigated the baseline characteristics and survival in the cohort of patients diagnosed with PoPH in the United Kingdom National Pulmonary Hypertension Service. METHODS: A retrospective review was conducted of all incident treatment-naïve patients with PoPH within the United Kingdom national registry diagnosed between January 2001 and December 2010. RESULTS:Patients with PoPH (n = 110) had survival rates of 85%, 60%, and 35% at 1, 3, and 5 years. The prevalence of PoPH was 0.85 cases/1 million. Mean age at diagnosis was 53 ± 12 years, with a balanced distribution in gender. Alcohol (n = 57) and hepatitis C (n = 10) were the most common causes of portal hypertension. Phosphodiesterase V inhibitors were the most frequently used targeted therapy, in 63.6% (n = 70) of patients, endothelin receptor antagonists were used in 10% (n = 11) and prostacyclin analogs in 12.7% (n = 14). Univariate and multivariate analysis of baseline characteristics did not demonstrate a significant influence of severity of portal hypertension or liver cirrhosis, World Health Organization Functional Class, cardiopulmonary hemodynamics, or year of diagnosis on survival. CONCLUSIONS: Survival of patients with PoPH remains poor despite targeted therapy and worse than patients with idiopathic PAH. The benefit of PAH therapies in PoPH on long-term morbidity and mortality outcomes needs further consideration and study.
Authors: Manik Aggarwal; Manshi Li; Abhishek Bhardwaj; William D Wallace; Xiaofeng Wang; William D Carey; Raed A Dweik; Gustavo A Heresi; Adriano R Tonelli Journal: Eur J Gastroenterol Hepatol Date: 2022-04-01 Impact factor: 2.566
Authors: Michael B Fallon; Steven M Kawut; Nadine Al-Naamani; Michael J Krowka; Kimberly A Forde; Karen L Krok; Rui Feng; Gustavo A Heresi; Raed A Dweik; Sonja Bartolome; Todd M Bull; Kari E Roberts; Eric D Austin; Anna R Hemnes; Mamta J Patel; Jae K Oh; Grace Lin; Margaret F Doyle; Nina Denver; Ruth Andrew; Margaret R MacLean Journal: Hepatology Date: 2020-09-29 Impact factor: 17.425
Authors: Hilary M DuBrock; Reena J Salgia; Norman L Sussman; Sonja D Bartolome; Zakiyah Kadry; David C Mulligan; Sarah Jenkins; Kandace Lackore; Richard N Channick; Steven M Kawut; Michael J Krowka Journal: Transplant Direct Date: 2019-05-22
Authors: Hilary M DuBrock; Charles D Burger; Sonja D Bartolome; Jeremy P Feldman; D Dunbar Ivy; Erika B Rosenzweig; Jeffrey S Sager; Kenneth W Presberg; Stephen C Mathai; Matthew R Lammi; James R Klinger; Michael Eggert; Teresa De Marco; Jean M Elwing; David Badesch; Todd M Bull; Linda M Cadaret; Gautam Ramani; Thenappan Thenappan; H James Ford; Nadine Al-Naamani; Marc A Simon; Sula Mazimba; James R Runo; Murali Chakinala; Evelyn M Horn; John J Ryan; Robert P Frantz; Michael J Krowka Journal: Pulm Circ Date: 2021-05-17 Impact factor: 3.017
Authors: Michael Krowka; Emmanuelle Cottreel; Marius M Hoeper; Nick H Kim; Nicolas Martin; Olivier Sitbon; Jaume Bosch Journal: Liver Transpl Date: 2020-04-30 Impact factor: 5.799