Troost Els, Van De Bruaene Alexander, Lampropoulos Konstantinos, Post Martijn, Gewillig Marc, Moons Philip, Delcroix Marion, Budts Werner
University Hospitals Leuven, Belgium.
Acta Cardiol. 2011 Jun;66(3):293-301. doi: 10.1080/ac.66.3.2114128.
Several patients with trisomy 21 developed the Eisenmenger syndrome (ES) because the underlying congenital heart defect was not corrected. However, little is known about their prognosis.This study aimed at (1) identifying risk factors for worse prognosis in ES patients, and (2) evaluating whether outcome of ES patients with trisomy 21 differs from ES patients without trisomy 21.
Data on all Eisenmenger patients in follow-up at the paediatric and adult congenital heart disease clinic of the University Hospitals Leuven were collected for retrospective analysis. Regression analysis was performed where applicable and survival rate was compared between patients with and without trisomy 21.
One hundred thirty-four patients (mean age at latest follow-up 33.2 +/- 13.6 years, 41.8% male, 44.8% trisomy 21) were included in the study. Complex lesions, right heart failure, impaired renal function, lower transcutaneous saturation and lower body mass index were predictive of impaired outcome. Mean survival of the global ES group was 44.9 +/- 2.2 years. However, long-term survival of trisomy 21 patients was not statistically different from patients without trisomy 21 (mean survival 44.5 +/- 2.6 years vs 44.5 +/- 2.9 years, respectively, P = 0.80, log rank test).
Long-term survival is markedly reduced in Eisenmenger patients. Complex lesions, right heart failure, impaired renal function, lower transcutaneous saturation and lower body mass index were related to worse prognosis. However, survival of trisomy 21 patients did not differ from patients without trisomy 21.
由于潜在的先天性心脏缺陷未得到纠正，数例21三体综合征患者发展为艾森曼格综合征（ES）。然而，对其预后了解甚少。本研究旨在：（1）确定ES患者预后较差的危险因素；（2）评估21三体综合征ES患者的预后与非21三体综合征ES患者是否不同。
收集鲁汶大学医院儿科和成人先天性心脏病门诊随访的所有艾森曼格患者的数据进行回顾性分析。在适用的情况下进行回归分析，并比较21三体综合征患者与非21三体综合征患者的生存率。
134例患者（最后一次随访时的平均年龄为33.2±13.6岁，男性占41.8%，21三体综合征患者占44.8%）纳入研究。复杂病变、右心衰竭、肾功能受损、经皮饱和度降低和体重指数较低可预测预后不良。整个ES组的平均生存期为44.9±2.2年。然而，21三体综合征患者的长期生存率与非21三体综合征患者无统计学差异（平均生存期分别为44.5±2.6年和44.5±2.9年，P = 0.80，对数秩检验）。
艾森曼格患者的长期生存率显著降低。复杂病变、右心衰竭、肾功能受损、经皮饱和度降低和体重指数较低与预后较差有关。然而，21三体综合征患者的生存率与非21三体综合征患者并无差异。
