Favoccia Carla, Constantine Andrew H, Wort Stephen J, Dimopoulos Konstantinos
a 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.
Expert Rev Cardiovasc Ther. 2019 Jun;17(6):449-459. doi: 10.1080/14779072.2019.1623024. Epub 2019 Jun 6.
: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. : In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. : PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.
艾森曼格综合征（ES）是先天性心脏病（CHD）患者中最严重的肺动脉高压（PAH）形式。其特征是肺血管阻力严重升高，导致分流逆转和发绀。
本文提供了成人ES及其他与CHD相关的PAH类型（PAH-CHD）的概述。描述了三级中心PAH-CHD的现代管理方法，重点关注合并症和并发症。
PAH-CHD描述了一系列广泛的病症，其中ES是典型代表。分流的大小和位置、右心室对增加的后负荷的适应程度以及其他代偿机制，如继发性红细胞增多症，决定了这些患者的临床表现和自然病程。PAH治疗改善了许多PAH-CHD患者的生活质量和预后，但专家多学科管理对于优化这群罕见且复杂患者的护理仍然至关重要。
