Centre for Congenital Heart Diseases, Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, The Netherlands.
Int J Cardiol. 2013 Sep 30;168(2):1370-7. doi: 10.1016/j.ijcard.2012.12.080. Epub 2013 Jan 20.
There is a need for reliable prognostic parameters in pulmonary arterial hypertension (PAH), especially in children. Pulsatile components of the right ventricular afterload, represented by pulmonary arterial compliance (PACi) and pulmonary stroke volume (PSVi), may provide important additional prognostic information to conventional static haemodynamic parameters. The aim of this study was to determine the prognostic value of PACi and PSVi in paediatric PAH.
Right heart catheterization data of 52 consecutive paediatric idiopathic/hereditary PAH and PAH associated with congenital heart disease patients with full haemodynamic evaluation seen at the Dutch national referral centre for paediatric pulmonary hypertension between 1993 and 2010 were reviewed. A control group was composed of patients with normal pulmonary vascular resistance. PSVi and PACi were calculated and tested for predictive value for transplant-free survival.
PAH patients had significantly lower PSVi and PACi compared to control patients. PSVi and PACi were lower in patients with higher WHO-functional class compared to those with lower functional classes. Higher PSVi, PACi and mSAP and lower mPAP/mSAP and heart rate were associated with improved survival, independent from WHO-functional class and PAH-targeted therapy. In multivariate analyses PSVi, heart rate and mSAP emerged as the strongest haemodynamic predictors of survival. The effect of vasodilator challenge on the haemodynamic variables did not provide additional prognostic information.
The parameters of both the pulsatile and static pulmonary circulations are strong independent predictors for transplant-free survival, and therefore can be of complementary value in assessing disease severity, predicting survival and guiding treatment in paediatric PAH.
肺动脉高压（PAH），尤其是儿童患者，需要可靠的预后参数。右心室后负荷的脉动成分，由肺动脉顺应性（PACi）和肺动脉每搏量（PSVi）表示，可能为传统的静态血流动力学参数提供重要的额外预后信息。本研究旨在确定 PACi 和 PSVi 在儿科 PAH 中的预后价值。
回顾了 1993 年至 2010 年间荷兰国家儿科肺动脉高压转诊中心对 52 例连续的特发性/遗传性儿童 PAH 和先天性心脏病相关 PAH 患者进行的右心导管检查数据，这些患者进行了全面的血流动力学评估。对照组由肺血管阻力正常的患者组成。计算了 PSVi 和 PACi，并对其进行了无移植生存预测价值的检验。
与对照组相比，PAH 患者的 PSVi 和 PACi 明显较低。与低功能分级的患者相比，WHO 功能分级较高的患者的 PSVi 和 PACi 较低。较高的 PSVi、PACi 和 mSAP，较低的 mPAP/mSAP 和心率与生存率的提高相关，与 WHO 功能分级和 PAH 靶向治疗无关。多变量分析显示 PSVi、心率和 mSAP 是生存率的最强血流动力学预测因子。血管扩张剂挑战对血流动力学变量的影响并未提供额外的预后信息。
脉动和静态肺循环的参数都是无移植生存的独立强预测因子，因此在评估疾病严重程度、预测生存率和指导儿科 PAH 治疗方面具有互补价值。
