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Pulmonary hypertension: lessons from contemporary registries

ESC Congress 2010

Pulmonary hypertension: lessons from contemporary registries

In this session, world-renowned experts in pulmonary hypertension reported the preliminary results of ongoing registries in pulmonary arterial hypertension.
Pulmonary Hypertension

Prof Beghetti from Geneva described the inclusion criteria of the TOPP registry, including pediatric patients (age 3 months – 18 years) with pulmonary arterial hypertension. More than 500 cases have been enrolled so far and the predominant etiology is idiopathic pulmonary arterial hypertension. This initiative will give us multiple diagnostic, prognostic and therapeutic insights in the field of pediatric pulmonary arterial hypertension, which is still without evidence-based information.

Professor Marc Humbert from Paris illustrated the results of the ongoing French registry on pulmonary arterial hypertension, focusing on the issue of incident vs prevalent cases. The prognosis of incident cases is worse as compared to that of prevalent cases (diagnosis > 6 months before inclusion in the registry). In fact, the population of prevalent cases is constituted by subjects who have survived the short term mortality and represent more stable patients. It was suggested to take into consideration this aspect in both prognostic analysis and in enrollment in clinical trials.

Dr Badesch from Denver reported the last update of the USA REVEAL registry, in which more than 3000 pulmonary arterial hypertension patients have been included. The initial results of this study have made it possible to propose a risk score calculator based on multiple clinical and haemodynamic variables. The application of this score to the existing population is able to stratify different populations with increasing mortality. The additional validation by independent populations is ongoing.

Prof. Sean Gaine from Dublin compared the patient populations enrolled in randomized clinical trials and in registries, and found that there were no major differences. Therefore, the favorable effects observed in randomized clinical trials with the approved drugs for pulmonary arterial hypertension can reliably be transferred to “real life” patients.




Pulmonary hypertension: lessons from contemporary registries
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.