Agarwal Raksheeth, Coats Louise E, Zaidi Ali N
Department of Medicine, Jacobi Medical Center/ Albert Einstein College of Medicine, Bronx, NY, USA.
Adult Congenital Heart Unit, Freeman Hospital, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK.
Int J Cardiol Congenit Heart Dis. 2025 Apr 28;20:100589. doi: 10.1016/j.ijcchd.2025.100589. eCollection 2025 Jun.
Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.
内皮功能障碍的特征是血管收缩、促凝血和促炎表型，已知其在几种慢性非传染性疾病中起作用。已经开发了几种生物物理和生化标志物来临床评估内皮功能。其在单心室先天性心脏病（SV-CHD）患者中的相关性越来越受到认可。在SV-CHD患者的所有姑息治疗阶段均观察到内皮功能障碍。可能有多种机制起作用，包括遗传因素、缺氧、血流搏动性丧失、神经激素和交感神经过度激活以及氧化应激。临床上，它可能导致肺血流受损、运动受限、血栓栓塞、肝功能障碍和不良妊娠结局。基于这些信息，已经提出了几个治疗靶点，如早期手术和运动干预、肺血管扩张剂和其他常用药物。然而，仍有许多未知之处，未来的研究必须阐明内皮功能障碍与这一复杂患者群体的关系，最终改善他们的临床护理。
