DOI QR코드

DOI QR Code

폐동맥 고혈압

Pulmonary Arterial Hypertension

  • 박용범 (한림대학교 의과대학 강동성심병원 호흡기 알레르기 내과 및 폐연구소)
  • Park, Yong Bum (Department of Pulmonary, Allergy and Critical Care Medicine and Lung Research Institute, College of Medicine, Hallym University, Kangdong Sacred Heart Hospital)
  • 투고 : 2009.03.29
  • 심사 : 2009.06.02
  • 발행 : 2009.09.30

초록

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.

키워드

참고문헌

  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;178:558-64 https://doi.org/10.1164/rccm.200709-1369PP
  2. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension:a national prospective study. Ann Intern Med1987;107:216-23 https://doi.org/10.7326/0003-4819-107-2-216
  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;115:343-9 https://doi.org/10.7326/0003-4819-115-5-343
  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;173:1023-30 https://doi.org/10.1164/rccm.200510-1668OC
  5. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007;30:104-9 https://doi.org/10.1183/09031936.00092306
  6. Thenappan T, Shah SJ, Rich S, Gomberg-Maitland. A USA-based registry for pulmonary arterial hypertension: 1982-2006. Eur Respir J 2007;30:1103-10 https://doi.org/10.1183/09031936.00042107
  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;132:373-9 https://doi.org/10.1378/chest.06-2913
  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;138:379-84
  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;177:1377-83 https://doi.org/10.1164/rccm.200712-1807OC
  10. Sztrymf B, Ya$\ddot{i}$ci A, Girerd B, Humbert M. Genes andpulmonary arterial hypertension. Respiration 2007;74:123-32 https://doi.org/10.1159/000098818
  11. Elliott CG, Glissmeyer EW, Havlena GT, Carlquist J, McKinney JT, Rich S, et al. Relationship of BMPR2 mutations to vasoreactivity in pulmonary arterial hyper tension. Circulation 2006;113:2509-15 https://doi.org/10.1161/CIRCULATIONAHA.105.601930
  12. Gali$\acute{e}$ 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;117:3010-9 https://doi.org/10.1161/CIRCULATIONAHA.107.742510
  13. Gali$\acute{e}$ 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;371:2093-100 https://doi.org/10.1016/S0140-6736(08)60919-8
  14. Simonneau G, Rubin LJ, Gali$\acute{e}$ 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;149:521-30 https://doi.org/10.7326/0003-4819-149-8-200810210-00004
  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 pulmonaryarterial hypertension. Am J Respir Crit Care Med 2008;177:1364-9 https://doi.org/10.1164/rccm.200712-1876OC