Logan Thirugnanasothy

Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry

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.

The good the bad and the ugly

We were, of course, very interested to read the manuscript by Saggar et al ,1 and the accompanying editorial by Nathan2 relating to the use of parenteral treprostinil therapy in patients referred for lung transplantation with pulmonary hypertension (PH) in association with pulmonary fibrosis (PF). It is good that there is continued interest in finding a clinical phenotype of patient with PF who may benefit on both symptomatic and prognostic grounds from targeted PH therapy. It is also good because there is a great clinical need to help this group of desperate …

Surgical embolectomy is underused

Pulmonary embolism remains a major healthcare burden and some patients still die from this preventable disease, perhaps because of limited knowledge about available treatments for massive pulmonary embolism.1 Systemic thrombolysis has been the mainstay for massive pulmonary embolism. Other options include transcatheter clot removal and locally directed thrombolysis, but their application is …