Pharmacologic therapy for pulmonary artery hypertension.

作者: Ajith Nair

DOI: 10.1097/HCO.0000000000000796

关键词:

摘要: Purpose of review Pulmonary arterial hypertension (PAH) is a disease that carries significant mortality left untreated. This article aims to pharmacotherapeutics for PAH. Recent findings PAH-specific therapies have evolved over the last three decades and expanded from one therapy in 1990s 14 FDA-approved medications. Current are directed at restoring imbalance vasoactive mediators include nitric oxide, endothelin prostacyclin. Although these agents effective as monotherapy, recent trials promulgated strategy upfront combination therapy. The availability oral prostacyclin agonists has also allowed treatment options. Risk assessment vital guiding PAH patients. There ongoing focus on targeting pathological mechanisms via novel repurposing existing drugs. Summary an array medications available Prudent maximize effect can improve morbidity mortality. reviews data supporting attempts outline approach patient management.

参考文章(93)
Paul W Armstrong, Burkert Pieske, Kevin J Anstrom, Justin Ezekowitz, Adrian F Hernandez, Javed Butler, Carolyn SP Lam, Piotr Ponikowski, Adriaan A Voors, Gang Jia, Steven E McNulty, Mahesh J Patel, Lothar Roessig, Joerg Koglin, Christopher M O’connor, None, Vericiguat in Patients With Heart Failure and Reduced Ejection Fraction The New England Journal of Medicine. ,vol. 382, pp. 1883- 1893 ,(2020) , 10.1056/NEJMOA1915928
Vallerie V. McLaughlin, Raymond L. Benza, Lewis J. Rubin, Richard N. Channick, Robert Voswinckel, Victor F. Tapson, Ivan M. Robbins, Horst Olschewski, Melvyn Rubenfire, Werner Seeger, Addition of Inhaled Treprostinil to Oral Therapy for Pulmonary Arterial Hypertension Journal of the American College of Cardiology. ,vol. 55, pp. 1915- 1922 ,(2010) , 10.1016/J.JACC.2010.01.027
Christopher J. Rhodes, Luke S. Howard, Mark Busbridge, Damien Ashby, Eumorfia Kondili, J. Simon R. Gibbs, John Wharton, Martin R. Wilkins, Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension: clinical prevalence, outcomes, and mechanistic insights. Journal of the American College of Cardiology. ,vol. 58, pp. 300- 309 ,(2011) , 10.1016/J.JACC.2011.02.057
Saira Khaderi, Rashid Khan, Zeenath Safdar, Rise Stribling, John M Vierling, John A Goss, Norman L Sussman, None, Long‐term follow‐up of portopulmonary hypertension patients after liver transplantation Liver Transplantation. ,vol. 20, pp. 724- 727 ,(2014) , 10.1002/LT.23870
Kirk B Lane, Rajiv D Machado, Michael W Pauciulo, Jennifer R Thomson, John A Phillips, James E Loyd, William C Nichols, Richard C Trembath, None, Heterozygous germline mutations in BMPR2 , encoding a TGF-β receptor, cause familial primary pulmonary hypertension Nature Genetics. ,vol. 26, pp. 81- 84 ,(2000) , 10.1038/79226
Edda Spiekerkoetter, Yon K. Sung, Deepti Sudheendra, Matthew Bill, Micheala A. Aldred, Mariëlle C. van de Veerdonk, Anton Vonk Noordegraaf, Janel Long-Boyle, Rajesh Dash, Phillip C. Yang, Allan Lawrie, Andrew J. Swift, Marlene Rabinovitch, Roham T. Zamanian, Low-Dose FK506 (Tacrolimus) in End-Stage Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. ,vol. 192, pp. 254- 257 ,(2015) , 10.1164/RCCM.201411-2061LE
GERALD SIMONNEAU, ROBYN J. BARST, NAZZARENO GALIE, ROBERT NAEIJE, STUART RICH, ROBERT C. BOURGE, ANNE KEOGH, RONALD OUDIZ, ADAANI FROST, SHELMER D. BLACKBURN, JAMES W. CROW, LEWIS J. RUBIN, Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. American Journal of Respiratory and Critical Care Medicine. ,vol. 165, pp. 800- 804 ,(2002) , 10.1164/AJRCCM.165.6.2106079
Paul M. Hassoun, Roham T. Zamanian, Rachel Damico, Noah Lechtzin, Rubina Khair, Todd M. Kolb, Ryan J. Tedford, Olivia L. Hulme, Traci Housten, Chiara Pisanello, Takahiro Sato, Erica H. Pullins, Celia P. Corona-Villalobos, Stefan L. Zimmerman, Mohamed A. Gashouta, Omar A. Minai, Fernando Torres, Reda E. Girgis, Kelly Chin, Stephen C. Mathai, Ambrisentan and Tadalafil Up-front Combination Therapy in Scleroderma-associated Pulmonary Arterial Hypertension American Journal of Respiratory and Critical Care Medicine. ,vol. 192, pp. 1102- 1110 ,(2015) , 10.1164/RCCM.201507-1398OC
Gianluigi Savarese, Stefania Paolillo, Pierluigi Costanzo, Carmen D'Amore, Milena Cecere, Teresa Losco, Francesca Musella, Paola Gargiulo, Caterina Marciano, Pasquale Perrone-Filardi, Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension?: A meta-analysis of 22 randomized trials Journal of the American College of Cardiology. ,vol. 60, pp. 1192- 1201 ,(2012) , 10.1016/J.JACC.2012.01.083
Lewis J. Rubin, Nazzareno Galiè, Friedrich Grimminger, Ekkehard Grünig, Marc Humbert, Zhi-Cheng Jing, Anne Keogh, David Langleben, Arno Fritsch, Flavia Menezes, Neil Davie, Hossein-Ardeschir Ghofrani, Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2) European Respiratory Journal. ,vol. 45, pp. 1303- 1313 ,(2015) , 10.1183/09031936.00090614