Apostolopoulou S C, Rammos S, Kyriakides Z S, Webb D J, Johnston N R, Cokkinos D V, Kremastinos D Th
Department of Paediatric Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece.
Heart. 2003 Oct;89(10):1221-6. doi: 10.1136/heart.89.10.1221.
To evaluate the acute haemodynamic effect of BQ-123, a selective endothelin A receptor antagonist, in severe chronic pulmonary arterial hypertension (PAH) of primary or autoimmune origin or related to congenital heart disease.
Prospective open clinical study.
Cardiology tertiary referral centre.
26 patients with chronic PAH were studied, with mean (SEM) age 29 (3) years (range 4-71 years), mean pulmonary artery pressure 68 (4) mm Hg, and pulmonary vascular resistance index 1694 (170) dyne x s x cm(-5). Patients were divided in three groups according to PAH aetiology: primary or autoimmune PAH (n = 12), and PAH associated with congenital heart defects with (n = 6) or without (n = 8) complete mixing.
BQ-123 200 nmol/min was infused for 60 minutes in the right atrium with sequential haemodynamic measurements at 30 minute intervals.
BQ-123 improved mean pulmonary artery pressure from 68 (4) to 64 (4) mm Hg (p < 0.05), pulmonary vascular resistance index from 1694 (170) to 1378 (145) dyne x s x cm(-5) (p < 0.001), pulmonary cardiac index from 3.0 (0.2) to 3.4 (0.3) l/min/m2 (p < 0.001), and effective cardiac index from 2.5 (0.2) to 2.7 (0.2) l/min/m2 (p < 0.01). Haemodynamic response was similar in all groups except for systemic cardiac index where a different (p = 0.0001, F = 5.53) response was observed; systemic cardiac index increased from 2.7 (0.2) to 2.9 (0.2) l/min/m2 (p < 0.001) when patients with complete mixing were excluded, in whom systemic cardiac index tended to decrease from 3.4 (1.0) to 3.0 (0.6) l/min/m2 (p = 0.06).
Acute endothelin A receptor antagonism induces substantial haemodynamic improvement in severe chronic PAH of primary or autoimmune origin or related to congenital heart disease.
评估选择性内皮素A受体拮抗剂BQ-123对原发性或自身免疫性起源或与先天性心脏病相关的重度慢性肺动脉高压（PAH）的急性血流动力学效应。
前瞻性开放性临床研究。
心脏病学三级转诊中心。
研究了26例慢性PAH患者，平均（标准误）年龄29（3）岁（范围4 - 71岁），平均肺动脉压68（4）mmHg，肺血管阻力指数1694（170）达因×秒×厘米⁻⁵。根据PAH病因将患者分为三组：原发性或自身免疫性PAH（n = 12），以及与先天性心脏缺陷相关的PAH，有（n = 6）或无（n = 8）完全混合。
以200 nmol/min的速度在右心房输注BQ-123 60分钟，每隔30分钟进行一次血流动力学测量。
BQ-123使平均肺动脉压从68（4）mmHg降至64（4）mmHg（p < 0.05），肺血管阻力指数从1694（170）降至1378（145）达因×秒×厘米⁻⁵（p < 0.001），肺心指数从3.0（0.2）升至3.4（0.3）升/分钟/平方米（p < 0.001），有效心指数从2.5（0.2）升至2.7（0.2）升/分钟/平方米（p < 0.01）。除全身心指数外，所有组的血流动力学反应相似，全身心指数观察到不同的（p = 0.0001，F = 5.53）反应；排除有完全混合的患者后，全身心指数从2.7（0.2）升至2.9（0.2）升/分钟/平方米（p < 0.001），在这些有完全混合的患者中，全身心指数倾向于从3.4（1.0）降至3.0（0.6）升/分钟/平方米（p = 0.06）。
急性内皮素A受体拮抗作用可使原发性或自身免疫性起源或与先天性心脏病相关的重度慢性PAH的血流动力学得到显著改善。
