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Published online before print October 24, 2007
Eur Respir J 2007, doi:10.1183/09031936.00104807
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ORIGINAL ARTICLE

Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases

R. Souza 1, M. Humbert 1*, B. Sztrymf 1, X. Jaïs 1, A. Yaïci 1, J. Le Pavec 1, F. Parent 1, P. Hervé 1, F. Soubrier 2, O. Sitbon 1, G. Simonneau 1

1 Centre Thématique de Recherche et de Soins sur l'Hypertension Artérielle Pulmonaire, CTRS INSERM 062, UPRES EA2705, Service de Pneumologie et Réanimation respiratoire, Hôpital Antoine-Béclère, Université Paris-Sud, Assistance Publique - Hôpitaux de Paris, Clamart, France
2 Laboratoire d'Oncogénétique et d'Angiogénétique Moléculaire, Fédération de Génétique, Groupe Hospitalier Pitié-Salpêtrière, Université Paris VI, Assistance Publique - Hôpitaux de Paris, Paris, France

* To whom correspondence should be addressed. E-mail: marc.humbert{at}abc.aphp.fr.


  Abstract

The aim of this study was to describe a large cohort of fenfluramine associated pulmonary arterial hypertension(fen-PAH) and its possible prognostic markers.

We retrospectively studied the records of all patients with the diagnosis of fen-PAH evaluated at our center from 1986 to 2004. Baseline clinical and hemodynamic data were collected, as well as the survival time. Results: Median duration of exposure was of 6 months with 4.5 years between exposure and beginning of symptoms. Nine out of 40 patients (22.5%) evaluated for the presence of germline BMPR2 mutations resulted positive; in these patients, duration of exposure to fenfluramine was significantly lower than in patients without mutation (p=0.007). Median survival was of 6.4 years without significant difference between fen-PAH and a control group of idiopathic and familial PAH patients referred to our center over the same time frame and treated identically. Duration of fenfluramine exposure presented no relation to survival whilst cardiac index was the only independent predictor at multivariate analysis.

Fen-PAH shares clinical, functional, hemodynamic and genetic features with idiopathic PAH, as well as the same overall survival. We therefore conclude that fenfluramine exposure characterizes a potent trigger for PAH without influencing its clinical course.

Keywords:  BMPR2, Fenfluramine derivatives, Idiopathic pulmonary arterial hypertension, Pulmonary arterial hypertension, Survival




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