From Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Biomedical Research Unit, Royal Brompton Hospital, Imperial College, London, UK (A.K., W.L., G.-P.D., K.D., S.T.W., M.A.G.); Department of Cardiology, Rigshospitalet, Copenhagen, Denmark (C.S.H., A.S.J., L.S.); Beijing Anzhen Hospital, Capital Medical University, China (G.H.); Boston Adult Congenital Heart and Pulmonary Hypertension Service, Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, MA (A.R.O., M.J.L.); Department of Cardiology, University Hospitals Leuven, Belgium (W.B.); Department of Cardiology, Academic Medical Center, Amsterdam, Netherlands (B.J.M., I.B.); Institute of Cardiology, Jagiellonian University Medical College, Cracow, Poland (L.T.-P.); AGH University of Science and Technology, Cracow, Poland (K.S.); Department of Cardiology, Second University of Naples, Italy (M.D'A., G.S.); Adult Congenital Heart Centre, University Medical Center, Ljubljana, Slovenia (K.P.); Department of Cardiology, Rikshospitalet Oslo University Hospital, Norway (M.-E.E.); and Department of Cardiology, Lund University Hospital, Sweden (U.T.).
Circulation. 2017 Apr 11;135(15):1432-1440. doi: 10.1161/CIRCULATIONAHA.116.023033. Epub 2016 Dec 15.
Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients.
In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters.
We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; <0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; =0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; <0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; =0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; <0.001) remained significant predictors of death.
There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.
艾森曼格综合征与大量发病率和死亡率相关。然而，对于死亡率风险分层尚无共识。我们旨在调查一个大型当代艾森曼格综合征患者队列的生存情况及其死亡预测因素。
采用多中心方法，我们确定了 2000 年至 2015 年期间接受随访的艾森曼格综合征成人患者。我们检查了生存情况及其与临床、心电图、超声心动图和实验室参数的关联。
我们研究了 1098 名患者（中位年龄 34.4 岁；范围 16.1-84.4 岁；65.1%为女性；31.9%患有唐氏综合征）。大多数患者存在三尖瓣后缺陷（n=643，58.6%），其次是复杂缺陷患者（n=315，28.7%）和三尖瓣前缺陷患者（n=140，12.7%）。在中位随访 3.1 年（四分位间距 1.4-5.9）期间，观察到 4361.6 患者-年，278 例患者死亡，6 例患者接受移植。单变量分析显示 12 个参数是死亡的显著预测因素。多变量 Cox 回归分析显示，仅年龄（风险比[HR]，每 10 年增加 1.41；95%置信区间[CI]，1.24-1.59；<0.001）、三尖瓣前分流（HR，1.56；95%CI，1.02-2.39；=0.041）、静息时血氧饱和度（HR，0.53/10%；95%CI，0.43-0.65；<0.001）、窦性节律（HR，0.53；95%CI，0.32-0.88；=0.013）和心包积液（HR，2.41；95%CI，1.59-3.66；<0.001）仍然是死亡的显著预测因素。
当代艾森曼格综合征成人患者存在显著的过早死亡。我们在此报告了一个基于该人群 5 个简单、非侵入性死亡预测因素的多变量死亡风险分层模型。
