Pulmonary arterial hypertension associated with tetralogy of Fallot.
Pulmonary arterial hypertension associated with tetralogy of Fallot.
复制标题
与法洛四联症相关的肺动脉高压。
DOI：
10.1536/ihj.14-351
复制
发表时间：
2015
影响因子：
1.5
通讯作者：
H. Yamagishi
中科院分区：
文献类型：
--
作者：
J. Yasuhara;H. Yamagishi
肺动脉高压（PAH）是先天性心脏病（CHD）术后常见的并发症。尽管最近的肺动脉高压临床分类将PAH伴CHD（PAH-CHD）分为几个亚类，但术后PAH-CHD的解剖学和血流动力学特征差异很大。因此，目前仍难以获得支持肺血管扩张剂治疗PAH-CHD适应症的临床证据。我们经常遇到法洛四联症（TOF）手术治疗后发生PAH的患者，特别是患有主要肺动脉侧支动脉（MAPCA）的患者。PAH可能由肺发育不全、发育不全和/或血栓形成、室间隔缺损闭合不充分、肺动脉狭窄缓解或既往体肺分流过大引起。此外，因存在PAH而被诊断为不可手术的TOF和MAPCA患者显示出与艾森曼格综合征患者相似的血流动力学和临床特征。这些患者的MAPCA通常表现出发育不良和异常树枝状化。根据我们的经验，我们认为PAH靶向治疗对TOF和MAPCA手术治疗后发生的部分PAH患者有效，尤其是作为经皮肺血管成形术的辅助治疗。为了帮助对TOF相关PAH患者进行分类，特别是MAPCA患者，我们提出了几种新的亚分类：“肺动脉床发育不良所致PAH”、“异常肺动脉分支所致PAH”或“CHD相关节段性PAH”。“使用统一方案的多中心患者登记对于探索肺血管扩张剂治疗术后PAH-CHD的适应症和疗效至关重要。
Pulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurring after surgical treatment of TOF and MAPCAs, especially as an adjunct to percutaneous pulmonary angioplasty. To help classify patients with PAH associated with TOF, especially with MAPCAs, we propose several new subclassifications: "PAH due to hypoplastic pulmonary arterial beds", "PAH due to abnormal pulmonary arborization", or "segmental PAH associated with CHD." A multicenter registry of patients using a unified protocol is essential to explore the indications and efficacy of pulmonary vasodilators for postoperative PAH-CHD.