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荆志成 , M.D; FCCP. 上海市肺科医院 同济大学医学院

肺动脉高压： 2010 － Pulmonary Arterial Hypertension. 荆志成 , M.D; FCCP. 上海市肺科医院 同济大学医学院. Patho-Anatomy of AMI.

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荆志成 , M.D; FCCP. 上海市肺科医院 同济大学医学院

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  1. 肺动脉高压：2010－ Pulmonary Arterial Hypertension 荆志成, M.D; FCCP. 上海市肺科医院 同济大学医学院

  2. Patho-Anatomy of AMI

  3. In 1891, the German pathologist Romberg observed that the heart of an individual, suffering from a stricture of the pulmonary arteries due to severe sclerosis, was double the size of a man´s fist. He again named this disease sclerosis of the pulmonary arteries.

  4. 危险因素 (芬氟拉明…) 遗传易感性 (BMPR2, ALK-1, endoglin, 5-HTT...) 血管损伤 平滑肌细胞功能紊乱 (Kv1.5 …) 内皮细胞功能紊乱 (NO, PgI2, ET-1, vWF…) 炎症因子 (IL-1, IL-6, MIP-1a, RANTES, fractalkine) 肺动脉高压

  5. 肺动脉高压发病机理 McLaughlin,VV,et al. JACC. 2009;53;1573-619

  6. 2. 左心疾病相关肺高血压 • 收缩功能障碍 • 舒张功能障碍 • 瓣膜病 肺高血压临床分类(updated 4rd WHO-Danapoint 2008) 1. 肺动脉高压 • 特发性肺动脉高压 • 遗传性 BMPR2 ALK1, endoglin 未知突变 • 药物或毒物 • 相关性 结缔组织病 心脏或大血管左向右分流 门脉高压 HIV 感染 血吸虫病 慢性溶血性贫血 • 1′肺静脉闭塞病，肺毛细血管瘤样病 3. 慢性肺病或缺氧性疾病相关肺高血压 • COPD • 间质性肺病 • 其他混合性通气功能或限制性通气功能障碍 • 呼吸睡眠暂停 • 慢性高原病 • 发育异常 4. 慢性血栓栓塞性肺高血压 5. 原因不明的或多因素肺高血压 • 血液学异常:骨髓异常增生，脾切除 • 系统性异常: 血管炎, 结节病, 肺朗格罕氏细胞 • 增多症, 多发性神经纤维瘤病 • 代谢异常: 糖原累积病, 高雪氏病, 甲状腺异常 • 其他: 肿瘤阻塞, 纤维纵隔炎, 肾衰或透析,

  7. What we have done? - Concepts of PH • PH - pulmonary hypertension 肺高血压症 • PAH - pulmonary arterial hypertension 肺动脉高压 • IPAH - Idiopathic PAH 特发性肺动脉高压 • PVH - pulmonary venous hypertension 肺静脉高压 • In China：most patients with PH were still wrongly called PAH

  8. Could not diagnosis early No standardized treatments Expensive cost Current Status of PAH in China Poor Prognosis

  9. What we have done?- Prevalence • The lowest estimates of the prevalence of PAH and IPAH are 15 cases and 5.9 cases/million adult population. • The lowest estimate of PAH incidence is 2.4 cases/million adult population/year. • Still no prevalence data in China • The awareness is poor in public, in physicians and in government • No support from Government can be available to the patients

  10. The 1st registry study of PAH in China Z-C JING et al. Chest.2007;132:373-379

  11. What we have done?- registry study

  12. What we have done? - acute vasoreactivity test • Long-term response to CCBs in IPAH should be identified by acute vasoreactivity test • But, in fact…

  13. What we have done?etiologies of PAH France China 2007-2009

  14. Demographic characteristics in our center from 2007-2009 Unpublished data of Shanghai Pulmonary hospital

  15. Adult congenital heart disease and idiopathic pulmonary artery hypertension like physiology • Small defect with PAH • Virus infection during pregnancy • Pulmonary vascular pathology was onset to intervene with the outcome of cardiovascular development Dana point meeting, THE NEW DIAGNOSIS: IPAH LIKE PHYSIOLOGY

  16. What we have done? - Definition for PAH mPAP ≥ 25 mm Hg PCWP ≤15 mm Hg assessed by RHC PVR > 3 Wood U • However, only few centers like to use RHC as diagnostic measure for PAH • Echo was still the most popular way to confirm PAH in China up to now • But more and more centers initiated standard RHC procedure and pulmonary vasoreactivity testing after 2006

  17. 毛细血管后压 毛细血管前压 正常肺动脉压力 毛细血管水平 左心房

  18. ECHO SCREEN

  19. What we have done? - RHC Firstly perform RHC through antebranchial vein in China Data from the Cathé Lab of Shanghai Pulmonary Hospital

  20. Edwards Swan-Ganz Cath

  21. Durg testing must be done in the 1st evaluation Rubin LJ and Badesch DB: Ann Intern Med 2005; 143: 282-92.

  22. 1 Long-term CCB responders .8 .6 Cumulative Survival .4 Long-term CCB failure .2 0 0 2 4 6 8 10 12 14 16 18 (Years) Survival in IPAHLong-term CCB responders Rich et al. N Engl J Med 1992 O. Sitbon et al. Circulation. 2005;111:3105-3111.

  23. Should we classify IPAH into 2 subgroups? • Responders to CCBs versus Prinzmental’s Variant Angina: Spasm of artery, younger, better survival, calcium antagonists are useful • Non-responder to CCBs versus angina secondary to Coronary atherosclerosis: more complex artery remodeling Different vascular disease, attacked the same target organ: pulmonary artery and right ventricular

  24. What we have done? - acute vasoreactivity test Infused Epoprostenol iNO Guidelines Infused Adenosine Inhaled Iloprost ? Available in China

  25. What we have done? - acute vasoreactivity testing Hoeper MM, et al. JACC. 2006; 48: 2546-52.

  26. What we have done? - acute vasoreactivity test Opitz CF, et al. Eur Respir J. 2009; 33: 1247–9.

  27. What we have done? - acute vasoreactivity test The first studyto prospectively address this question in a sizable group of IPAH patients is published by JING et al. in this issue of the ERJ. Z-C JING, et al. Eur Respir J. 2009; 33: 1354–60.

  28. Acute responder to Iloprost PAP 92/34/59 mm Hg PAP 36/11/20 mm Hg CO 2.94 L/min CO 3.53 L/min data of Shanghai Pulmonary hospital

  29. What we have done? - acute vasoreactivity test

  30. What we have done ? – pulmonary angiography • Pulmonary angiography is very important to diagnostic use in PH, especially for CTEPH • But, doctors in China seldom underwent this procedure just because the severely elevated pulmonary arterial pressure

  31. What we have done ? – pulmonary angiography Unpublished data of Shanghai Pulmonary hospital

  32. What we have done ? – pulmonary angiography Unpublished data of Shanghai Pulmonary hospital

  33. What we have done ? – pulmonary angiography

  34. 肺动脉造影-CTEPH

  35. 肺动脉造影-肺血管炎

  36. 肺动脉造影-肺动脉发育不良

  37. PE OR TUMOR : CATHETERIZE BIOPSY

  38. What we should do in 2010?Genetics study BMPRII Mutation rate in IPAH IN China ：21% ID 432号患者417bp 位置A/G杂合 Unpublished data of Shanghai Pulmonary hospital

  39. 1 6-min WT > 250 m(n = 88) .8 .6 Cumulative Survival p = 0.0008 .4 .2 6-min WT  250 m(n = 90) 0 0 12 24 36 48 60 72 84 96 108 Months 运动能力评估: 6分钟步行距离 Epoprostenol cohort Miyamoto et al. AJRCCM 2000; 161:487-492 Sitbon et al. J Am Coll Cardiol 2002; 40: 780-8

  40. + 12 12 * * 10 10 < 6.4 mg/dl (median) 8 8 6 6 Survival (%) Serum uric acid levels (mg/dl) 4 4 2 2 Serum uric acid levels (mg/dl)  6.49 mg/dl 0 0 Control(n = 30) NYHA II(n = 5) NYHA III(n = 72) NYHA IV(n = 13) Time (years) ASSESSMENT OF PAH SEVERITYURIC ACID Nagaya N, et al. Am J Respir Crit Care Med 1999; 160:478-92

  41. Baseline BNP Follow-up BNP 100 100 BNP < 180 pg/ml 80 BNP < 150 pg/ml 80 60 60 Survival rate (%) 40 40 BNP  150 pg/ml 20 20 BNP  180 pg/ml 0 0 0 12 24 36 48 0 12 24 36 48 Time (months) Time (months) ASSESSMENT OF PAH SEVERITYNATRIURETIC PEPTIDES Nagaya N, et al. Circulation 2000; 102:865-70.

  42. ASSESSMENT OF PAH SEVERITYTROPONIN T Torbicki A, et al. Circulation 2003

  43. What we have done? – treatments • Up to now, only two PAH-specific drugs have been marketed in China • Bosentan • Iloprost • Other off-label PAH-specific drugs • PDE-5: Sildenafil, Vardenafil • Prostanoids: Beraprost Both more than 10,000 € annually 1,500-2,000 €annually

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