Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.
Circulation. 2012 Sep 18;126(12):1461-8. doi: 10.1161/CIRCULATIONAHA.112.091421. Epub 2012 Aug 16.
Eisenmenger syndrome differs significantly from other types of pulmonary arterial hypertension in its physiology and prognosis. We sought to assess the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality.
Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome, excluding those with complex congenital heart disease. Patients' mean age was 39.1 ± 12.8 years, 59 (32.6%) were male, 122 (67.4%) were in functional class III or higher, and 74 (40.9%) were on advanced therapies. Mean oxygen saturation at rest was 85.1 ± 7.8%, and median B-type natriuretic peptide was 55.4 ng/L. Over a median follow-up of 16.4 months, 19 patients died; the strongest predictors of mortality were tricuspid annular plane systolic excursion and peak systolic velocity, myocardial performance (expressed as total isovolumic time and ratio of systolic to diastolic duration), and elevated central venous pressure (expressed as right atrial [RA] area, RA pressure, and ratio of RA to left atrial area), even after we accounted for advanced therapies. A composite score based on the strongest echocardiographic predictors of outcome, including 1 point for each of the following: tricuspid annular plane systolic excursion <15 mm, ratio of right ventricular effective systolic to diastolic duration ≥ 1.5, RA area ≥ 25 cm², ratio of RA to left atrial area ≥ 1.5, was highly predictive of clinical outcome (area under the curve 0.90 ± 0.01), with no improvement when B-type natriuretic peptide and resting saturations were added into the model.
Echocardiographic parameters of right ventricular function and RA area predict mortality in Eisenmenger patients. A new composite echocardiographic score, described herewith, may be incorporated into the noninvasive, periodic assessment of these patients.
艾森曼格综合征在生理学和预后方面与其他类型的肺动脉高压有显著不同。我们试图评估艾森曼格综合征患者的超声心动图特征与死亡率之间的关系。
纳入了 181 例连续的艾森曼格综合征患者（排除复杂先天性心脏病患者），评估了其临床和超声心动图变量。患者的平均年龄为 39.1±12.8 岁，59 例（32.6%）为男性，122 例（67.4%）处于功能 III 级或更高，74 例（40.9%）接受了高级治疗。静息时平均血氧饱和度为 85.1±7.8%，中位 B 型利钠肽水平为 55.4ng/L。中位随访 16.4 个月期间，19 例患者死亡；死亡率最强的预测因素是三尖瓣环平面收缩期位移和收缩期峰值速度、心肌性能（表现为总等容收缩时间和收缩期与舒张期持续时间之比）以及中心静脉压升高（表现为右心房[RA]面积、RA 压力和 RA 与左心房面积之比），即使我们考虑了高级治疗也是如此。基于对预后最强的超声心动图预测因素的复合评分，包括以下各有 1 分：三尖瓣环平面收缩期位移＜15mm、右心室有效收缩期与舒张期持续时间之比≥1.5、RA 面积≥25cm²、RA 与左心房面积之比≥1.5，对临床结局具有高度预测性（曲线下面积 0.90±0.01），当将 B 型利钠肽和静息饱和度纳入模型时，并无改善。
右心室功能和 RA 面积的超声心动图参数可预测艾森曼格患者的死亡率。本文描述的新的复合超声心动图评分可用于这些患者的非侵入性、定期评估。
