Cantor W J, Harrison D A, Moussadji J S, Connelly M S, Webb G D, Liu P, McLaughlin P R, Siu S C
The University of Toronto Congenital Cardiac Centre for Adults, The Toronto Hospital, University of Toronto, Ontario, Canada.
Am J Cardiol. 1999 Sep 15;84(6):677-81. doi: 10.1016/s0002-9149(99)00415-4.
A retrospective study of adults with Eisenmenger syndrome assessed at a tertiary referral center was performed to identify clinical characteristics and establish prognostic determinants. Mortality and cause of death are reported with baseline clinical data correlated with mortality to identify predictors of death. Clinical events during follow-up, including heart failure, atrial arrhythmia, hemoptysis, and syncope were also reviewed. A total of 109 adults with Eisenmenger syndrome (mean +/- SD age 29 +/- 11 years, 43% men) were followed for a median of 6.3 years. Sixty-six patients (61%) had simple cardiac anatomy (13 atrial septal defect, 43 ventricular septal defect, 10 patent ductus arteriosus). The remainder (43 patients) had complex cardiac anatomy (including atrioventricular septal defect, truncus arteriosus, univentricular heart, and transposition of the great arteries). There were 33 deaths and 9 transplantations during follow-up. Median survival was 53 years. Multivariate Cox regression analysis identified age at presentation (hazard ratio [HR] 0.90), supraventricular arrhythmia (HR 3.44), precordial electrocardiogram voltage (HR 1.61/mV increase), and poor New York Heart Association functional class (HR 2.60) as independent predictors of mortality. There is a large variation in the life expectancy for adults with Eisenmenger syndrome. Baseline characteristics associated with increased mortality include younger age at presentation (associated with complex anatomy), functional class, supraventricular arrhythmia, and an electrocardiogram index for right ventricular hypertrophy.
在一家三级转诊中心对患有艾森曼格综合征的成年人进行了一项回顾性研究，以确定临床特征并确立预后决定因素。报告了死亡率和死亡原因，并将其与死亡率相关的基线临床数据进行关联，以确定死亡的预测因素。还回顾了随访期间的临床事件，包括心力衰竭、房性心律失常、咯血和晕厥。共有109例患有艾森曼格综合征的成年人（平均±标准差年龄29±11岁，43%为男性）接受了中位时间为6.3年的随访。66例患者（61%）心脏解剖结构简单（13例房间隔缺损、43例室间隔缺损、10例动脉导管未闭）。其余43例患者心脏解剖结构复杂（包括房室间隔缺损、动脉干、单心室和大动脉转位）。随访期间有33例死亡和9例移植。中位生存期为53岁。多因素Cox回归分析确定就诊时年龄（风险比[HR]0.90）、室上性心律失常（HR 3.44）、心前区心电图电压（每增加1mV，HR 1.61）和纽约心脏协会功能分级差（HR 2.60）为死亡的独立预测因素。患有艾森曼格综合征的成年人的预期寿命差异很大。与死亡率增加相关的基线特征包括就诊时年龄较小（与复杂解剖结构相关）、功能分级、室上性心律失常和右心室肥厚的心电图指数。
