40岁以上成人先天性心脏病相关的肺动脉高压
Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years.
作者信息
Maurer Susanne J, Stöckemann Katharina, Pujol Claudia, Hörer Jürgen, Ewert Peter, Tutarel Oktay
机构信息
Department of Congenital Heart Disease and Paediatric Cardiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany.
Department of Electrophysiology, German Heart Centre Munich, Technical University of Munich, 80636 Munich, Germany.
出版信息
J Clin Med. 2020 Dec 17;9(12):4071. doi: 10.3390/jcm9124071.
BACKGROUND
Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking.
METHODS
PAH-ACHD patients ≥ 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality.
RESULTS
In total, 65 patients (67.7% female, mean age 45.19 ± 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2-7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2-14.4, = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2-118.7, = 0.006) remained as independent predictors of all-cause mortality.
CONCLUSIONS
PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.
背景
与成人先天性心脏病相关的肺动脉高压（PAH-ACHD）在年轻时会导致显著的死亡率。目前缺乏关于老年患者不良结局的危险因素。
方法
纳入2005年1月至2018年12月期间正在接受积极随访的年龄≥40岁的PAH-ACHD患者。从医院记录中获取人口统计学数据以及医疗/手术史。主要终点是全因死亡率。
结果
共纳入65例患者（女性占67.7%，平均年龄45.19±6.75岁）。其中，46例（70.8%）有分流病变，12例（18.5%）的PAH与复杂先天性心脏缺陷相关，7例（10.8%）因主要主-肺动脉侧支导致节段性肺动脉高压。13例患者（20.0%）患有唐氏综合征。在中位随访4.2年（四分位间距1.2 - 7.5年）期间，16例患者（24.6%）死亡。单因素分析显示，NT-proBNP（对数）、肌酐以及既往室性心律失常病史是全因死亡率的预测因素。多因素分析显示，NT-proBNP（对数）（HR：4.1，95%CI：1.2 - 14.4，P = 0.029）和肌酐（HR：16.3，95%CI：2.2 - 118.7，P = 0.006）仍然是全因死亡率的独立预测因素。
结论
40岁以上的PAH-ACHD患者面临着显著的死亡率，其中NT-proBNP和肌酐是独立的预测因素。
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