靶向药物在单心室患者Fontan围术期的应用进展

Targeted Therapies in Fontan Patients Due to Single Ventricle:Recent Advances

功能性单心室患者Fontan手术成功的关键主要取决于肺血管阻力。Fontan循环中高肺血管阻力将带来各种并发症,如运动耐量下降、低心排血量、心室功能衰竭和蛋白丢失性肠病,最终导致Fontan循环衰竭。因此,降低肺血管阻力的肺血管靶向药物治疗已成为学者近年的研究热点。本综述主要分析了靶向药物波生坦和西地那非在肺动脉压和肺血管阻力增高的Fontan患者围术期中的有效性和安全性。

The success of staged Fontan palliation for patients with single ventricle is related to low pulmonary vascular resistance （PVR）. The complications of high PVR in Fontan physiology are numerous, such as low exercise tolerance, low cardiac output, ventricular function failure and protein-losing enteropathy; eventually it leads to failing Fontan. Therefore, a low PVR is crucial in Fontan patients. Now, targeted therapies decreasing PVR has been an advanced research hotspot in Fontan patients. In this review we present an overview of the safety and efficacy of the therapy with bosentan or sildenaffl on elevated pulmonary artery pressure and pulmonary vascular resistance in Fontan patients.