Schuuring Mark J, van Riel Annelieke C M J, Vis Jeroen C, Duffels Marielle G, van Dijk Arie P J, de Bruin-Bon Rianne H A C M, Zwinderman Aeilko H, Mulder Barbara J M, Bouma Berto J
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, The Netherlands.
Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.
Int J Cardiol. 2015 Feb 15;181:270-6. doi: 10.1016/j.ijcard.2014.11.222. Epub 2014 Dec 13.
Patients with CHD-PAH have a limited prognosis. In daily practice, combination therapy is often initiated after a clinical event. Although clinical events have been associated with a poor prognosis in idiopathic PAH, data on this association are limited in CHD-PAH. The aim of this study was to determine whether baseline characteristics and clinical events associate with mortality in patients with pulmonary hypertension (PAH) due to congenital heart disease (CHD).
In total 91 consecutive adults (42 ± 14 year) with CHD-PAH were referred for therapy between January 2005 and June 2013. Cox proportional hazard analysis was performed to identify determinants of mortality, including clinical events as time dependent covariates.
Twenty-four patients (nine with Down) died during the median follow-up of 4.7 (range 0.1-7.9) years. The one and eight year mortality rates were 7.3% and 37.3%, respectively. Clinical events included admission for heart failure (n=9), arrhythmias (n=9), haemoptysis (n=5), change to a worse NYHA class (n=16), vascular events (n=1), syncope (n=1) and need for red blood cell depletion (n=4). In univariate analysis, both baseline characteristics and clinical events were associated with mortality. In multivariate analysis, only baseline NT-pro-BNP serum level ≥ 500 ng/L and TAPSE<15mm at echocardiography were significant determinants of mortality. None of the clinical events remained significant. Patients with both a NT-pro-BNP serum level ≥ 500 ng/L and TAPSE<15mm at echocardiography have a nine fold higher mortality rate than patients without both risk factors.
Prognosis is still poor in contemporary patients with CHD-PAH. Both baseline NT-pro-BNP serum level and right ventricular function are superior to clinical events in prognostication. These two baseline characteristics should have a major impact on therapeutic management in patients with CHD-PAH, such as initiation of combination therapy.
冠心病相关肺动脉高压（CHD-PAH）患者预后有限。在日常临床实践中，联合治疗通常在临床事件发生后启动。虽然临床事件与特发性肺动脉高压（PAH）的不良预后相关，但在CHD-PAH中，关于这种关联的数据有限。本研究的目的是确定基线特征和临床事件是否与先天性心脏病（CHD）所致肺动脉高压（PAH）患者的死亡率相关。
2005年1月至2013年6月期间，共91例连续的成年CHD-PAH患者（42±14岁）被转诊接受治疗。进行Cox比例风险分析以确定死亡率的决定因素，包括将临床事件作为时间依赖性协变量。
在中位随访4.7年（范围0.1 - 7.9年）期间，24例患者（9例患有唐氏综合征）死亡。1年和8年死亡率分别为7.3%和37.3%。临床事件包括因心力衰竭入院（n = 9）、心律失常（n = 9）、咯血（n = 5）、纽约心脏协会（NYHA）分级恶化（n = 16）、血管事件（n = 1）、晕厥（n = 1）以及需要进行红细胞去除（n = 4）。在单因素分析中，基线特征和临床事件均与死亡率相关。在多因素分析中，只有基线NT-pro-BNP血清水平≥500 ng/L以及超声心动图检查时三尖瓣环平面收缩期位移（TAPSE）<15mm是死亡率的显著决定因素。没有临床事件仍然具有显著性。超声心动图检查时NT-pro-BNP血清水平≥500 ng/L且TAPSE<15mm的患者的死亡率比没有这两个危险因素的患者高9倍。
当代CHD-PAH患者的预后仍然很差。在预后评估方面，基线NT-pro-BNP血清水平和右心室功能均优于临床事件。这两个基线特征应对CHD-PAH患者的治疗管理产生重大影响，例如联合治疗的启动。
