(Abstract 37, Poster B15) American Thoracic Society, May 2002

Authors:
Stuart Rich,
Alicia Shillington,
Vallerie V. McLaughlin.
Rush Heart Institute Center for Pulmonary Heart Disease, Chicago IL, USA

Abstract:

RATIONALE:
Fenfluramines (fen) are a proven cause of primary pulmonary hypertension (PPH). Whether the clinical presentation and prognosis differs from sporadic PPH is unknown.

METHODS/RESULTS:
We compared the clinical profile and outcome of pts with fen-PPH (n=10) against pts with sporadic PPH referred to our center over the same time frame (n=70). Data was retrieved from a prospective clinical database developed at our Institute. Pts with fen-PPH were similar to those with sporadic PPH with respect to age (46.6 vs. 43.5 yrs) and hemodynamics (mean PAP 56.7 vs. 59.1 mmHg; PVR 19.0 vs 18.2 units). None of the fen-PPH pts responded to acute vasodilator challenge and all were treated with i.v. prostacyclin. To evaluate their response to treatment, we compared the survival of the fen-PPH pts to 20 PPH pts treated with prostacyclin matched for baseline functional class and PVR. Pts with fen-PPH did much worse: 1 yr survival 50% vs. 88%; 2 yr survival 33% vs. 69%; 3 yr survival 17% vs. 60%; 4 yr survival 17% vs. 31%. (O.R.=2.7; 95% C.I.=0.95-7.56).

CONCLUSION:
Pts with fen-PPH present similar to those with sporadic PPH but have a much poorer prognosis. Whether the fenfluramines induce a disease pathway that is more resistant to therapy needs further exploration.