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28-09-2014 | Cardiology | Article

Targeted treatments may improve idiopathic PAH survival


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medwireNews: Patients with idiopathic pulmonary arterial hypertension (IPAH) may live longer if they are given targeted treatments than if they receive conventional management, researchers suggest.

Survival rates for targeted versus conventionally treated patients were 92.3% and 72.9%, respectively, at 1 year, 82.3% and 49.9% at 3 years, 64.4% and 42.8% at 5 years, and 42.9% and 22.0% at 10 years.

“We believe targeted therapies would improve survival benefits in IPAH patients”, say Jae Seung Lee and colleagues from the University of Ulsan College of Medicine in Seoul, South Korea, in Heart & Lung.

Conventional treatments, such as oxygen therapy, digoxin, diuretics and anticoagulation, are used to relieve symptoms and aim to improve patients’ quality of life, but there are limited data on potential survival benefits.

Novel and targeted treatments include prostanoids, endothelin receptor agonists and phosphodiesterase type 5 inhibitors, the researchers add, and data on the associated survival benefits have been reported in retrospective, nonrandomised studies in mainly Western populations.

Their study compared the survival benefits of conversional versus targeted treatments in patients diagnosed with PAH at the Asan Medical Center, a tertiary hospital affiliated to the Ulsan College of Medicine, between 1994 and 2013.

They considered 71 patients (50 female), with a median age of 30 years, and PAH diagnosed by right heart catheterisation and transthoracic echocardiography findings. The majority (63%) received conventional therapies; the remainder received targeted treatments alone or in addition to conventional treatments.

Lee et al note that 31.1% of patients in the conventional treatment arm were also treated with targeted agents after 1 year of conventional therapy and almost a quarter of patients continued this additional targeted treatment for more than a year.

Survival rates were higher in patients receiving combined therapy than in those who only received conventional treatments. There was also a trend for better survival with combined therapy over targeted therapy alone, although the researchers say this could be due to selection bias.

Nevertheless, they conclude that survival of patients “improved after the introduction of targeted agents and recommend that “further well-conducted, randomized controlled studies are necessary to evaluate the efficacy of targeted therapies.”

medwireNews ( is an independent clinical news service provided by Springer Healthcare Limited. © Springer Healthcare Ltd; 2014

By Sara Freeman, medwireNews Reporter