Columbia University College of Physicians and Surgeons, New York, NY 10032, USA.
Prog Cardiovasc Dis. 2012 Sep-Oct;55(2):128-33. doi: 10.1016/j.pcad.2012.07.004.
Pulmonary arterial hypertension (PAH) with increased pulmonary vascular resistance (PVR), previously termed pulmonary vascular obstructive disease or pulmonary vascular disease is a frequent complication of congenital heart disease (CHD).While there have been advances in the medical treatments available for classic Eisenmenger syndrome patients who are not suitable for repair, the sub-group of patients with moderate sized congenital systemic to pulmonary shunts and mild to moderately elevated PVR remains challenging. With the development of targeted medical treatments for pulmonary arterial hypertension (PAH), the concept of a combined medical and interventional/surgical approach for patients with PAH associated with CHD (APAH-CHD) has emerged. Careful evaluation and an understanding of the predominant physiologic features will help guide the management of these complex patients and whether late surgical repair is feasible.
肺动脉高压（PAH）伴肺血管阻力增加（PVR），既往称为肺血管阻塞性疾病或肺血管疾病，是先天性心脏病（CHD）的常见并发症。虽然对于不适合修复的经典艾森曼格综合征患者的可用医疗治疗方法已经取得了进展，但对于中等大小的先天性体肺分流和轻度至中度升高的 PVR 患者亚组仍然具有挑战性。随着肺动脉高压（PAH）靶向药物治疗的发展，对于伴有 CHD 的 PAH 患者（APAH-CHD）的联合药物和介入/手术治疗的概念已经出现。仔细评估和了解主要生理特征将有助于指导这些复杂患者的管理，以及是否可行晚期手术修复。
