Blok I M, van Riel A C M J, Schuuring M J, Duffels M G, Vis J C, van Dijk A P J, Hoendermis E S, Mulder B J M, Bouma B J
Department of Cardiology, Academic Medical Centre, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands.
Neth Heart J. 2015 May;23(5):278-84. doi: 10.1007/s12471-015-0666-9.
Decrease in quality of life (QoL) in left-sided heart failure precedes poor survival, which can be reversed with exercise training. We investigated whether QoL is associated with mortality in pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) patients.
In this observational study, PAH-CHD adults referred for PAH-specific therapy were included. QoL surveys (SF36) were recorded during 2 years of therapy. Based on shift in SF36 scores during this period, patients had either decreased or non-decreased QoL. Subsequently, the patients were followed for mortality.
Thirty-nine PAH-CHD patients (mean age 42, 44 % male, 49 % Down's syndrome) were analysed. Following PAH-specific therapy, SF36 physical component summary (PCS) decreased in 13 (35-31 points, p = 0.001) and showed no decrease in 26 patients (34-43 points, mean values, p < 0.001). Post-initiation phase, median follow-up was 4.5 years, during which 12 deaths occurred (31 %), 10 (56 %) in the decreased and 2 (10 %) in the non-decreased group (p = 0.002). Cox regression showed a decrease in SF36 PCS predicted mortality (HR 3.4, 95 % CI 1.03-11, p = 0.045).
In PAH-CHD patients, decrease in SF36 PCS following initiation of PAH-specific therapy is a determinant of mortality.
左心衰竭患者生活质量（QoL）下降先于生存率降低，而运动训练可逆转这种情况。我们调查了先天性心脏病所致肺动脉高压（PAH-CHD）患者的生活质量是否与死亡率相关。
在这项观察性研究中，纳入了接受PAH特异性治疗的PAH-CHD成年患者。在治疗的2年期间记录生活质量调查（SF36）。根据这段时间内SF36评分的变化，患者的生活质量要么下降，要么未下降。随后，对患者进行死亡率随访。
分析了39例PAH-CHD患者（平均年龄42岁，44%为男性，49%患有唐氏综合征）。接受PAH特异性治疗后，13例患者（35 - 31分，p = 0.001）的SF36身体成分总结（PCS）下降，26例患者（34 - 43分，平均值，p < 0.001）未下降。治疗开始后的中位随访时间为4.5年，在此期间发生了12例死亡（31%），下降组中有10例（56%），未下降组中有2例（10%）（p = 0.002）。Cox回归显示，SF36 PCS下降可预测死亡率（HR 3.4，95% CI 1.03 - 11，p = 0.045）。
在PAH-CHD患者中，开始PAH特异性治疗后SF36 PCS下降是死亡率的一个决定因素。
