Badesch, D. B., Tapson, V. F., McGoon, M. D., Brundage, B. H., Rubin, L. J., Wigley, F. M., . . . Medsger, T. A., Jr. (2000). Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med, 132(6), 425-434.
Barst, R. J., Rubin, L. J., Long, W. A., McGoon, M. D., Rich, S., Badesch, D. B., . . . Crow, J. W. (1996). A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med, 334(5), 296-301. doi:10.1056/nejm199602013340504
Barst, R. J., Rubin, L. J., Long, W. A., McGoon, M. D., Rich, S., Badesch, D. B., . . . Primary Pulmonary Hypertension Study, G. (1996). A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med, 334(5), 296-301. doi:10.1056/nejm199602013340504
Benza, R. L., Gomberg-Maitland, M., Demarco, T., Frost, A. E., Torbicki, A., Langleben, D., . . . Tiwari, H. K. (2015). Endothelin-1 Pathway Polymorphisms and Outcomes in Pulmonary Arterial Hypertension. Am J Respir Crit Care Med, 192(11), 1345-1354. doi:10.1164/rccm.201501-0196OC
Benza, R. L., Miller, D. P., Barst, R. J., Badesch, D. B., Frost, A. E., & McGoon, M. D. (2012). An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest, 142(2), 448-456. doi:10.1378/chest.11-1460
Enright, P. L., & Sherrill, D. L. (1998). Reference equations for the six-minute walk in healthy adults. Am J Respir Crit Care Med, 158(5 Pt 1), 1384-1387. doi:10.1164/ajrccm.158.5.9710086
Galiè, N., Barberà, J. A., Frost, A. E., Ghofrani, H.-A., Hoeper, M. M., McLaughlin, V. V., . . . Rubin, L. J. (2015). Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New England Journal of Medicine, 373(9), 834-844. doi:10.1056/NEJMoa1413687
Galie, N., Channick, R. N., Frantz, R. P., Grunig, E., Jing, Z. C., Moiseeva, O., . . . McLaughlin, V. V. (2019). Risk stratification and medical therapy of pulmonary arterial hypertension. Eur Respir J, 53(1). doi:10.1183/13993003.01889-2018
Ghofrani, H. A., Galiè, N., Grimminger, F., Grünig, E., Humbert, M., Jing, Z. C., . . . Group, P.-S. (2013). Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med, 369(4), 330-340. doi:10.1056/NEJMoa1209655
Halliday, S. J., & Hemnes, A. R. (2017). Identifying "super responders" in pulmonary arterial hypertension. Pulm Circ, 7(2), 300-311. doi:10.1177/2045893217697708
Halliday, S. J., Xu, M., Thayer, T. E., Mosley, J. D., Sheng, Q., Ye, F., . . . Hemnes, A. R. (2018). Clinical and genetic associations with prostacyclin response in pulmonary arterial hypertension. Pulm Circ, 8(4), 2045894018800544. doi:10.1177/2045894018800544
Hemnes, A. R., Trammell, A. W., Archer, S. L., Rich, S., Yu, C., Nian, H., . . . West, J. (2015). Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation, 131(4), 401-409; discussion 409. doi:10.1161/circulationaha.114.013317
Hemnes, A. R., Zhao, M., West, J., Newman, J. H., Rich, S., Archer, S. L., . . . Austin, E. D. (2016). Critical Genomic Networks and Vasoreactive Variants in Idiopathic Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. doi:10.1164/rccm.201508-1678OC
Higenbottam, T., Butt, A. Y., McMahon, A., Westerbeck, R., & Sharples, L. (1998). Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart, 80(2), 151-155. doi:10.1136/hrt.80.2.151
Jacobs, W., Boonstra, A., Brand, M., Rosenberg, D. M., Schaaf, B., Postmus, P. E., & Vonk Noordegraaf, A. (2010). Long-term outcomes in pulmonary arterial hypertension in the first-line epoprostenol or first-line bosentan era. J Heart Lung Transplant, 29(10), 1150-1158. doi:10.1016/j.healun.2010.05.011
Kuhn, K. P., Byrne, D. W., Arbogast, P. G., Doyle, T. P., Loyd, J. E., & Robbins, I. M. (2003). Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med, 167(4), 580-586. doi:10.1164/rccm.200204-333OC
McLaughlin, V. V. (2006). Survival in patients with pulmonary arterial hypertension treated with first-line bosentan. Eur J Clin Invest, 36 Suppl 3, 10-15. doi:10.1111/j.1365-2362.2006.01688.x
McLaughlin, V. V., Shillington, A., & Rich, S. (2002). Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation, 106(12), 1477-1482.
Pulido, T., Adzerikho, I., Channick, R. N., Delcroix, M., Galie, N., Ghofrani, H. A., . . . Investigators, S. (2013). Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med, 369(9), 809-818. doi:10.1056/NEJMoa1213917
Rich, S., Pogoriler, J., Husain, A. N., Toth, P. T., Gomberg-Maitland, M., & Archer, S. L. (2010). Long-term effects of epoprostenol on the pulmonary vasculature in idiopathic pulmonary arterial hypertension. Chest, 138(5), 1234-1239. doi:10.1378/chest.09-2815
Robbins, I. M., Newman, J. H., Johnson, R. F., Hemnes, A. R., Fremont, R. D., Piana, R. N., . . . Byrne, D. W. (2009). Association of the metabolic syndrome with pulmonary venous hypertension. Chest, 136(1), 31-36. doi:10.1378/chest.08-2008
Rubin, L. J., Badesch, D. B., Barst, R. J., Galie, N., Black, C. M., Keogh, A., . . . Simonneau, G. (2002). Bosentan therapy for pulmonary arterial hypertension. N Engl J Med, 346(12), 896-903. doi:10.1056/NEJMoa012212
Rubin, L. J., Mendoza, J., Hood, M., McGoon, M., Barst, R., Williams, W. B., . . . Long, W. (1990). Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med, 112(7), 485-491.
Sastry, B. K., Narasimhan, C., Reddy, N. K., & Raju, B. S. (2004). Clinical efficacy of sildenafil in primary pulmonary hypertension: a randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol, 43(7), 1149-1153. doi:10.1016/j.jacc.2003.10.056
Shapiro, S. M., Oudiz, R. J., Cao, T., Romano, M. A., Beckmann, X. J., Georgiou, D., . . . Brundage, B. H. (1997). Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol, 30(2), 343-349.
Sitbon, O., Humbert, M., Jais, X., Ioos, V., Hamid, A. M., Provencher, S., . . . Simonneau, G. (2005). Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation, 111(23), 3105-3111. doi:10.1161/circulationaha.104.488486
Sitbon, O., Humbert, M., Nunes, H., Parent, F., Garcia, G., Herve, P., . . . Simonneau, G. (2002). Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol, 40(4), 780-788.
Weatherald, J., Boucly, A., Chemla, D., Savale, L., Peng, M., Jevnikar, M., . . . Sitbon, O. (2018). Prognostic Value of Follow-Up Hemodynamic Variables After Initial Management in Pulmonary Arterial Hypertension. Circulation, 137(7), 693-704. doi:10.1161/CIRCULATIONAHA.117.029254
Zhao, L., Mason, N. A., Morrell, N. W., Kojonazarov, B., Sadykov, A., Maripov, A., . . . Wilkins, M. R. (2001). Sildenafil inhibits hypoxia-induced pulmonary hypertension. Circulation, 104(4), 424-428.