Department of Internal Medicine, The Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
Heart Fail Clin. 2012 Jul;8(3):427-45. doi: 10.1016/j.hfc.2012.04.002.
Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.
许多患有先天性心脏病和体肺分流的患者会出现肺动脉高压（PAH），尤其是如果心脏缺陷未得到修复。肺血流量的持续增加可能导致阻塞性小动脉病和肺血管阻力增加，这种情况可导致分流逆转和发绀（艾森曼格综合征）。心导管检查对于明确诊断和治疗非常重要。波生坦是迄今为止唯一一种在专门针对先天性心脏病相关 PAH 的随机对照试验中与安慰剂进行比较的药物。肺移植联合心脏缺陷修复或心肺联合移植保留用于顽固病例。
