Pascall Emma, Tulloh Robert Mr
Department of Congenital Heart Disease, Bristol Heart Institute, Upper Maudlin Street, Bristol, BS2 8BJ, UK.
Future Cardiol. 2018 Jul;14(4):343-353. doi: 10.2217/fca-2017-0065. Epub 2018 May 24.
Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mmHg. We focus on its relevance in congenital heart disease, reviewing pathophysiology, diagnosis and management. Pulmonary hypertension is a relatively common complication of congenital heart disease, with adult prevalence between 5 and 10%. A multifactorial cause is recognized, relating to the size and nature of cardiac defect as well as environmental and genetic factors. More complex disease is increasingly recognized rather than pure Eisenmenger complex. Remodeling of the pulmonary vascular bed causes increased pulmonary vascular resistance diagnosed by a collection of investigations including echocardiography, exercise testing, cardiac catheterization, MRI and CT scanning. Management employs disease-modifying medications which are now used with increasing benefit.
肺动脉高压定义为平均肺动脉压≥25 mmHg。我们关注其在先天性心脏病中的相关性，回顾其病理生理学、诊断和管理。肺动脉高压是先天性心脏病相对常见的并发症，在成人中的患病率为5%至10%。其病因是多因素的，与心脏缺损的大小和性质以及环境和遗传因素有关。越来越多地认识到更为复杂的疾病，而非单纯的艾森曼格综合征。肺血管床的重塑导致肺血管阻力增加，这可通过包括超声心动图、运动试验、心导管检查、磁共振成像和CT扫描在内的一系列检查来诊断。治疗采用改善病情的药物，目前使用这些药物的益处越来越多。
