Pulmonary arterial hypertension, a disease largely neglected until a few decades ago, is presently the object of intense studies by several research teams. Despite considerable progress, pulmonary arterial hypertension remains a major clinical problem, because it is not always easy to diagnose, treat, and prevent. The disease was considered incurable until the late 1990s, when Epoprostenol was introduced as the first tool against this illness. More recently, therapy for pulmonary arterial hypertension gained momentum after publication of the SERAPHIN and AMBITION trials, which also highlighted the importance of upfront therapy. This review also focuses on recent substudies from these trials and progress in drugs targeting the endothelin pathway. Future perspectives with regard to endothelin-receptor antagonists are also discussed.
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Actelion Pharmaceuticals, 2016, OPS MAC TABL OR US P
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Ahn, Li Young
;
Kim, Sung Eun
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Kim, Sung Eun
;
Yi, SoJeong
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Yi, SoJeong
;
Dingemanse, Jasper
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Actel Pharmaceut Ltd, Dept Clin Pharmacol, Allschwil, SwitzerlandSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Dingemanse, Jasper
;
Lim, Kyoung Soo
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Lim, Kyoung Soo
;
Jang, In-Jin
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Jang, In-Jin
;
Yu, Kyung-Sang
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
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Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Atsmon, Jacob
;
Dingemanse, Jasper
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Actel Pharmaceut Ltd, Dept Clin Pharmacol, CH-4123 Allschwil, SwitzerlandTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Dingemanse, Jasper
;
Shaikevich, Dimitri
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Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Shaikevich, Dimitri
;
Volokhov, Inna
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Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Volokhov, Inna
;
Sidharta, Patricia N.
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Actel Pharmaceut Ltd, Dept Clin Pharmacol, CH-4123 Allschwil, SwitzerlandTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
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Univ Colorado, Clin Res Ctr, Aurora, CO 80045 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Badesch, David B.
;
Feldman, Jeremy
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Arizona Pulm Specialists Ltd, Phoenix, AZ USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Feldman, Jeremy
;
Keogh, Anne
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St Vincents Hosp, Sydney, NSW 2010, AustraliaUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Keogh, Anne
;
Mathier, Michael A.
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Univ Pittsburgh, Pittsburgh, PA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Mathier, Michael A.
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Oudiz, Ronald J.
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Harbor UCLA Med Ctr, LA Biomed Res Inst, Torrance, CA 90509 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Oudiz, Ronald J.
;
Shapiro, Shelley
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VA Greater Angeles VA Healthcare Syst, Los Angeles, CA USA
David Geffen UCLA Sch Med, Los Angeles, CA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Shapiro, Shelley
;
Farber, Harrison W.
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Boston Univ, Sch Med, Boston, MA 02118 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Farber, Harrison W.
;
McGoon, Michael
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Mayo Clin, Rochester, MN USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
McGoon, Michael
;
Frost, Adaani
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Baylor Coll Med, Houston, TX 77030 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Frost, Adaani
;
Allard, Martine
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Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Allard, Martine
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Despain, Darrin
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Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Despain, Darrin
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Dufton, Christopher
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Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Dufton, Christopher
;
Rubin, Lewis J.
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UCSD, La Jolla, CA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Ahn, Li Young
;
Kim, Sung Eun
论文数: 0引用数: 0
h-index: 0
机构:
Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Kim, Sung Eun
;
Yi, SoJeong
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Yi, SoJeong
;
Dingemanse, Jasper
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h-index: 0
机构:
Actel Pharmaceut Ltd, Dept Clin Pharmacol, Allschwil, SwitzerlandSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Dingemanse, Jasper
;
Lim, Kyoung Soo
论文数: 0引用数: 0
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机构:
Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Lim, Kyoung Soo
;
Jang, In-Jin
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
Jang, In-Jin
;
Yu, Kyung-Sang
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h-index: 0
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Seoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South KoreaSeoul Natl Univ, Coll Med & Hosp, Dept Clin Pharmacol & Therapeut, Seoul 110744, South Korea
机构:
Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Atsmon, Jacob
;
Dingemanse, Jasper
论文数: 0引用数: 0
h-index: 0
机构:
Actel Pharmaceut Ltd, Dept Clin Pharmacol, CH-4123 Allschwil, SwitzerlandTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Dingemanse, Jasper
;
Shaikevich, Dimitri
论文数: 0引用数: 0
h-index: 0
机构:
Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Shaikevich, Dimitri
;
Volokhov, Inna
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h-index: 0
机构:
Tel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Tel Aviv Univ, Tel Aviv Sackler Fac Med, IL-69978 Tel Aviv, IsraelTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
Volokhov, Inna
;
Sidharta, Patricia N.
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h-index: 0
机构:
Actel Pharmaceut Ltd, Dept Clin Pharmacol, CH-4123 Allschwil, SwitzerlandTel Aviv Univ, Tel Aviv Sourasky Med Ctr, Clin Res Ctr, IL-69978 Tel Aviv, Israel
机构:
Univ Colorado, Clin Res Ctr, Aurora, CO 80045 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Badesch, David B.
;
Feldman, Jeremy
论文数: 0引用数: 0
h-index: 0
机构:
Arizona Pulm Specialists Ltd, Phoenix, AZ USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Feldman, Jeremy
;
Keogh, Anne
论文数: 0引用数: 0
h-index: 0
机构:
St Vincents Hosp, Sydney, NSW 2010, AustraliaUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Keogh, Anne
;
Mathier, Michael A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pittsburgh, Pittsburgh, PA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Mathier, Michael A.
;
Oudiz, Ronald J.
论文数: 0引用数: 0
h-index: 0
机构:
Harbor UCLA Med Ctr, LA Biomed Res Inst, Torrance, CA 90509 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Oudiz, Ronald J.
;
Shapiro, Shelley
论文数: 0引用数: 0
h-index: 0
机构:
VA Greater Angeles VA Healthcare Syst, Los Angeles, CA USA
David Geffen UCLA Sch Med, Los Angeles, CA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Shapiro, Shelley
;
Farber, Harrison W.
论文数: 0引用数: 0
h-index: 0
机构:
Boston Univ, Sch Med, Boston, MA 02118 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Farber, Harrison W.
;
McGoon, Michael
论文数: 0引用数: 0
h-index: 0
机构:
Mayo Clin, Rochester, MN USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
McGoon, Michael
;
Frost, Adaani
论文数: 0引用数: 0
h-index: 0
机构:
Baylor Coll Med, Houston, TX 77030 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Frost, Adaani
;
Allard, Martine
论文数: 0引用数: 0
h-index: 0
机构:
Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Allard, Martine
;
Despain, Darrin
论文数: 0引用数: 0
h-index: 0
机构:
Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Despain, Darrin
;
Dufton, Christopher
论文数: 0引用数: 0
h-index: 0
机构:
Gilead Sci Inc, Foster City, CA 94404 USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA
Dufton, Christopher
;
Rubin, Lewis J.
论文数: 0引用数: 0
h-index: 0
机构:
UCSD, La Jolla, CA USAUniv Colorado, Clin Res Ctr, Aurora, CO 80045 USA