Schwerzmann Markus, Pfammatter Jean-Pierre
Center for Congenital Heart Disease, University Hospital Inselspital, Bern, Switzerland.
Expert Rev Cardiovasc Ther. 2015 Jun;13(6):693-701. doi: 10.1586/14779072.2015.1047763. Epub 2015 May 17.
Atrial septal defects (ASDs) are one of the most frequent congenital cardiac malformations, accounting for about 8-10% of all congenital heart defects. The prevalence of pulmonary arterial hypertension (PAH) in adults with an ASD is 8-10%. Different clinical PAH scenarios can be encountered. At one end of the spectrum are adults with no or only mild pulmonary vascular disease and a large shunt. These are patients who can safely undergo shunt closure. In the elderly, mild residual pulmonary hypertension after shunt closure is the rule. At the other end of the spectrum are adults with severe, irreversible pulmonary vascular disease, shunt reversal and chronic cyanosis, that is, Eisenmenger syndrome. These are patients who need to be managed medically. The challenge is to properly classify ASD patients with PAH falling in between the two ends of the spectrum as the ones with advanced, but reversible pulmonary vascular disease amenable to repair, versus the ones with progressive pulmonary vascular disease not responding to shunt closure. There are concerns that adults with progressive pulmonary vascular disease have worse outcomes after shunt closure than patients not undergoing shunt closure. Due to the correlation of pulmonary vascular changes and pulmonary hemodynamics, cardiac catheterization is used in the decision-making process. It is important to consider the hemodynamic data in the context of the clinical picture, the defect anatomy and further noninvasive tests when evaluating the option of shunt closure in these patients.
房间隔缺损（ASD）是最常见的先天性心脏畸形之一，约占所有先天性心脏病的8%-10%。患有ASD的成年人中肺动脉高压（PAH）的患病率为8%-10%。临床上会遇到不同的PAH情况。在这个范围的一端是没有或仅有轻度肺血管疾病且存在大量分流的成年人。这些患者可以安全地进行分流关闭手术。在老年人中，分流关闭后出现轻度残余肺动脉高压是常见的情况。在这个范围的另一端是患有严重、不可逆肺血管疾病、分流逆转和慢性紫绀的成年人，即艾森曼格综合征。这些患者需要进行药物治疗。挑战在于如何正确地将处于这两个极端情况之间的患有PAH的ASD患者分类，哪些是患有晚期但可逆的肺血管疾病适合修复的，哪些是患有进行性肺血管疾病对分流关闭无反应的。有人担心，患有进行性肺血管疾病的成年人在分流关闭后的预后比未进行分流关闭的患者更差。由于肺血管变化与肺血流动力学相关，心脏导管检查被用于决策过程。在评估这些患者的分流关闭选项时，结合临床症状、缺损解剖结构和进一步的非侵入性检查来考虑血流动力学数据非常重要。
