Tuberc Respir Dis.  2009 Sep;67(3):177-182.

Pulmonary Arterial Hypertension

• Park YB

Abstract

Pulmonary arterial hypertension is a progressive, symptomatic, and ultimately fatal disorder for which substantial advances in treatment have been made during the past decade. This article reviews the recent advances in the field of pulmonary arterial hypertension (PAH). Epidemiology, genetics, treatment and prognosis will be the main focus of this update.

Keyword

Pulmonary arterial hypertension; Epidemiology; Genetics; Therapeutics; Prognosis

MeSH Terms

Hypertension
Hypertension, Pulmonary
Prognosis
Hypertension, Pulmonary

Reference

1. Rai PR, Cool CD, King JA, Stevens T, Burns N, Winn RA, et al. The cancer paradigm of severe pulmonary arterial hypertension. Am J Respir Crit Care Med. 2008. 178:558–564.

2. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med. 1987. 107:216–223.

3. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension: results from a national prospective registry. Ann Intern Med. 1991. 115:343–349.

4. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006. 173:1023–1030.

5. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007. 30:104–109.

6. Thenappan T, Shah SJ, Rich S, Gomberg-Maitland . A USA-based registry for pulmonary arterial hypertension: 1982-2006. Eur Respir J. 2007. 30:1103–1110.

7. Jing ZC, Xu XQ, Han ZY, Wu Y, Deng KW, Wang H, et al. Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest. 2007. 132:373–379.

8. Tueller C, Stricker H, Soccal P, Tamm M, Aubert JD, Maggiorini M, et al. Epidemiology of pulmonary hypertension: new data from the Swiss registry. Swiss Med Wkly. 2008. 138:379–384.

9. Sztrymf B, Coulet F, Girerd B, Yaici A, Jais X, Sitbon O, et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008. 177:1377–1383.

10. Sztrymf B, Yaïci A, Girerd B, Humbert M. Genes and pulmonary arterial hypertension. Respiration. 2007. 74:123–132.

11. Elliott CG, Glissmeyer EW, Havlena GT, Carlquist J, McKinney JT, Rich S, et al. Relationship of BMPR2 mutations to vasoreactivity in pulmonary arterial hypertension. Circulation. 2006. 113:2509–2515.

12. Galiè N, Olschewski H, Oudiz RJ, Torres F, Frost A, Ghofrani HA, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008. 117:3010–3019.

13. Galiè N, Rubin LJ, Hoeper M, Jansa P, Al-Hiti H, Meyer G, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008. 371:2093–2100.

14. Simonneau G, Rubin LJ, Galiè N, Barst RJ, Fleming TR, Frost AE, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. 2008. 149:521–530.

15. Forfia PR, Mathai SC, Fisher MR, Housten-Harris T, Hemnes AR, Champion HC, et al. Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2008. 177:1364–1369.