Center for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, PO Box 30 001, 9700 RB Groningen, The Netherlands.
Eur Heart J. 2011 Dec;32(24):3137-46. doi: 10.1093/eurheartj/ehr282. Epub 2011 Sep 4.
To assess the occurrence and prognostic value of acute vasodilator response (AVR) in paediatric vs. adult pulmonary arterial hypertension, and idiopathic/hereditary pulmonary arterial hypertension (iPAH/HPAH) vs. pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) using three different response criteria.
Ninety-nine PAH patients underwent AVR testing (37 children, 62 adults; 70 iPAH/HPAH, 29 PAH-CHD). Three response criteria from clinical practice were used to define AVR. The number of responders was evaluated separately in subgroups based on age, diagnosis, and presence of a non-restrictive post-tricuspid shunt. Numbers of responders varied importantly using the different criteria but were always higher in iPAH/HPAH, compared with PAH-CHD. The number of responders did not differ between paediatric and adult iPAH/HPAH. No responders were identified in patients with a post-tricuspid shunt. Acute vasodilator response was associated with improved survival using all three criteria. Low baseline mean right atrial pressure (mRAP) was associated with improved survival in adults (P< 0.001). High baseline mean pulmonary arterial pressure (mPAP)/mean systemic arterial pressure (mSAP) and pulmonary vascular resistance (PVR)/systemic vascular resistance (SVR) were associated with worse survival, statistically independent from age, diagnosis, and the presence of a post-tricuspid shunt.
The proportion of patients with AVR highly depends on the used criteria, but did not differ between paediatric and adult iPAH/HPAH. Current response criteria are not suitable for patients with a post-tricuspid shunt. In both children and adults without post-tricuspid shunts, AVR was associated with improved survival independent of the used criteria. Nevertheless, prognostic value in the individual patient was limited. Baseline mRAP showed a good correlation with survival for adult PAH patients, but not for children. High baseline mPAP/mSAP and PVR/SVR was associated with worse prognosis independent from age, diagnosis, or the presence of a post-tricuspid shunt.
使用三种不同的反应标准，评估儿科与成人肺动脉高压、特发性/遗传性肺动脉高压（iPAH/HPAH）与先天性心脏病相关肺动脉高压（PAH-CHD）之间急性血管扩张剂反应（AVR）的发生和预后价值。
99 名肺动脉高压患者接受了 AVR 测试（37 名儿童，62 名成人；70 名 iPAH/HPAH，29 名 PAH-CHD）。使用三种来自临床实践的反应标准来定义 AVR。根据年龄、诊断和是否存在非限制性三尖瓣后分流，分别评估了反应者的数量。使用不同标准时，反应者的数量差异很大，但 iPAH/HPAH 中的反应者总是高于 PAH-CHD。儿科和成人 iPAH/HPAH 之间的反应者数量没有差异。在存在三尖瓣后分流的患者中，未发现反应者。所有三种标准均显示急性血管扩张剂反应与生存改善相关。低基线平均右心房压（mRAP）与成人的生存改善相关（P<0.001）。高基线平均肺动脉压（mPAP）/平均体动脉压（mSAP）和肺血管阻力（PVR）/体血管阻力（SVR）与较差的生存相关，统计学上独立于年龄、诊断和三尖瓣后分流的存在。
反应者的比例高度取决于使用的标准，但儿科和成人 iPAH/HPAH 之间没有差异。目前的反应标准不适用于存在三尖瓣后分流的患者。在没有三尖瓣后分流的儿童和成人中，AVR 与生存改善相关，与使用的标准无关。然而，对个体患者的预后价值有限。基线 mRAP 与成人 PAH 患者的生存有很好的相关性，但与儿童无关。高基线 mPAP/mSAP 和 PVR/SVR 与较差的预后相关，与年龄、诊断或三尖瓣后分流的存在无关。
