780-6 Effect of Long Term Prostacyclin (PGI2) on Exercise in Patients with Primary Pulmonary Hypertension (PPH) Who Have Deteriorated on Maximal Conventional Therapy

Continuous IV PGI2 has been shown to improve survival and quality of life in patients with PPH. We used cycle ergometrywith continuous measurement of gas exchange to evaluate 15 pts (6–57 yrs) with PPH who were treated with PGI2 after having deteriorated on maximal conventional therapy (CT: oral vasodilators, cardiac glycosides, diuretics, oxygen and anticoagulation).Column I shows the performance while on maximal CT 2 to 13 mos before starting PG12. Column 2 is the baseline performance immediately prior to starting PGI2. Data are presented as mean±SD.CTBaseline2–8 mos.11–18 mos.18–24 mos.N=15N=15N=15N=10N=6VO2†49±18*39±1145±16*49±1747±20Max. workt†45±12*35±1441±1846±261±33O2 Pulse‡6.2±3*5.1±25.9±2.3*6.6±2.7*4.8±1.8*p≤0.05 vs. baseline.†% predicted.‡ml/beatAt 2 to 8 mos f/u 11/15 pts increased their performance compared to baseline. At 11 to 18 mos f/u 8/10 pts continued to show improvement. Four of 6 were still performing above baseline at 18 to 24 mos f/u.In conclusion. continuous IV PGl2 improved andlor prevented progression in patients who had deteriorated on maximal conventional therapy. Exercise testing is useful for assessing functional capacity in patients with PPH and may help to optimize therapy