BACKGROUND: Characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) who benefit from long-term calcium channel blockers (CCB) are unknown. METHODS AND RESULTS: Acute pulmonary vasodilator testing with epoprostenol or nitric oxide was performed in 557 IPAH patients. Acute responders, defined by a fall in both mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >20%, received long-term oral CCB. Patients who benefit from long-term CCB were defined as those being in New York Heart Association (NYHA) functional class I or II after at least 1 year on CCB monotherapy. Among the 70 patients who displayed acute pulmonary vasoreactivity (12.6%; 95% CI, 9.8% to 15.3%) and received CCB therapy, only 38 showed long-term improvement (6.8%; 95% CI, 4.7% to 8.9%). Long-term CCB responders had less severe disease at baseline than patients who failed. During acute vasodilator testing, long-term CCB responders displayed a more pronounced fall in mean PAP (-39+/-11% versus -26+/-7%; P<0.0001), reaching an absolute value of mean PAP lower than that measured in patients who failed (33+/-8 versus 46+/-10 mm Hg; P<0.0001). After 7.0+/-4.1 years, all but 1 long-term CCB responders were alive in NYHA class I or II, with a sustained hemodynamic improvement. In the group of patients who failed on CCB, the 5-year survival rate was 48%. CONCLUSIONS: Long-term CCB responders represent <10% of IPAH patients evaluated in a pulmonary vascular referral center. During acute vasodilator testing, these patients showed significantly lower levels of both mean PAP and PVR, which reached near-normal values.
BACKGROUND: Characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) who benefit from long-term calcium channel blockers (CCB) are unknown. METHODS AND RESULTS: Acute pulmonary vasodilator testing with epoprostenol or nitric oxide was performed in 557 IPAH patients. Acute responders, defined by a fall in both mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >20%, received long-term oral CCB. Patients who benefit from long-term CCB were defined as those being in New York Heart Association (NYHA) functional class I or II after at least 1 year on CCB monotherapy. Among the 70 patients who displayed acute pulmonary vasoreactivity (12.6%; 95% CI, 9.8% to 15.3%) and received CCB therapy, only 38 showed long-term improvement (6.8%; 95% CI, 4.7% to 8.9%). Long-term CCB responders had less severe disease at baseline than patients who failed. During acute vasodilator testing, long-term CCB responders displayed a more pronounced fall in mean PAP (-39+/-11% versus -26+/-7%; P<0.0001), reaching an absolute value of mean PAP lower than that measured in patients who failed (33+/-8 versus 46+/-10 mm Hg; P<0.0001). After 7.0+/-4.1 years, all but 1 long-term CCB responders were alive in NYHA class I or II, with a sustained hemodynamic improvement. In the group of patients who failed on CCB, the 5-year survival rate was 48%. CONCLUSIONS: Long-term CCB responders represent <10% of IPAH patients evaluated in a pulmonary vascular referral center. During acute vasodilator testing, these patients showed significantly lower levels of both mean PAP and PVR, which reached near-normal values.
Authors: Anna R Hemnes; Aaron W Trammell; Stephen L Archer; Stuart Rich; Chang Yu; Hui Nian; Niki Penner; Mitchell Funke; Lisa Wheeler; Ivan M Robbins; Eric D Austin; John H Newman; James West Journal: Circulation Date: 2014-10-31 Impact factor: 29.690
Authors: John H Newman; Stuart Rich; Steven H Abman; John H Alexander; John Barnard; Gerald J Beck; Raymond L Benza; Todd M Bull; Stephen Y Chan; Hyung J Chun; Declan Doogan; Jocelyn Dupuis; Serpil C Erzurum; Robert P Frantz; Mark Geraci; Hunter Gillies; Mark Gladwin; Michael P Gray; Anna R Hemnes; Roy S Herbst; Adrian F Hernandez; Nicholas S Hill; Evelyn M Horn; Kendall Hunter; Zhi-Cheng Jing; Roger Johns; Sanjay Kaul; Steven M Kawut; Tim Lahm; Jane A Leopold; Greg D Lewis; Stephen C Mathai; Vallerie V McLaughlin; Evangelos D Michelakis; Steven D Nathan; William Nichols; Grier Page; Marlene Rabinovitch; Jonathan Rich; Franz Rischard; Sharon Rounds; Sanjiv J Shah; Victor F Tapson; Naomi Lowy; Norman Stockbridge; Gail Weinmann; Lei Xiao Journal: Am J Respir Crit Care Med Date: 2017-06-15 Impact factor: 21.405