title: Primary pulmonary hypertension associated with the use of fenfluramine derivatives.
abstract: Fenfluramine derivatives (Fds) are a well-established risk factor for primary pulmonary hypertension (PPH). We compared 62 Fd-PPH patients (61 women) evaluated in our center between 1986 and 1997 with 125 sex-matched PPH patients nonexposed to Fd referred during the same period (control PPH). In the Fd-PPH group, 33 patients (53%) used dexfenfluramine alone, 7 patients (11%) used fenfluramine alone, and 5 patients (8%) used both drugs. In 17 cases (27%), Fd use was associated with that of amphetamines. Most of the exposed patients used Fd for at least 3 months (81%). The interval between the onset of dyspnea and that of drug intake was 49+/-68 months (27 days to 23 years). At the time of diagnosis, Fd-PPH and control PPH were similar in terms of New York Heart Association functional class and symptoms. The two groups significantly differed only in terms of age (50+/-12 vs 40+/-14 years) and body mass index (28+/-6 vs 23+/-4). The two groups displayed similar severe baseline hemodynamics (total pulmonary vascular resistance: 32+/-12 vs 31+/-12 IU/m2), but the percentage of responders to acute vasodilator testing was higher in control PPH (27% vs 10%, p < 0.01). As a result, more patients were treated with oral vasodilators in the control PPH group (36% vs 16%, p < 0.01) and long-term epoprostenol infusion was more frequently used in the Fd-PPH group (52% vs 31%, p < 0.01). Overall survival was similar in the two groups with a 3-year survival rate of 50%.
authors.affiliation: Center for Pulmonary Vascular Disease, Paris-Sud University, Antoine Béclère Hospital, Clamart, France.
authors.forename: G
authors.initials: G
authors.lastname: Simonneau
authors.forename: M
authors.initials: M
authors.lastname: Fartoukh
authors.forename: O
authors.initials: O
authors.lastname: Sitbon
authors.forename: M
authors.initials: M
authors.lastname: Humbert
authors.forename: J L
authors.initials: JL
authors.lastname: Jagot
authors.forename: P
authors.initials: P
authors.lastname: Hervé
mesh_terms: D000328:Adult; D001067:Appetite Depressants / Q000008:administration & dosage / Q000009:adverse effects*; D004359:Drug Therapy, Combination; D005260:Female; D005277:Fenfluramine / Q000008:administration & dosage / Q000009:adverse effects* / Q000031:analogs & derivatives*; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000139:chemically induced* / Q000175:diagnosis / Q000401:mortality; D008168:Lung / Q000098:blood supply; D008297:Male; D008875:Middle Aged; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D011985:Receptors, Serotonin / Q000187:drug effects; D012307:Risk Factors; D015996:Survival Rate; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D001067:Appetite Depressants; D011985:Receptors, Serotonin; D005277:Fenfluramine