Department of Pediatrics and Neonatology, Maastricht University Medical Center, Maastricht, The Netherlands.
School for Mental Health and Neuroscience, Maastricht University, Maastricht, The Netherlands.
Pediatr Cardiol. 2021 Jan;42(1):1-18. doi: 10.1007/s00246-020-02440-w. Epub 2020 Dec 29.
Congenital heart defects (CHD) is one of the most common types of birth defects. Thanks to advances in surgical techniques and intensive care, the majority of children with severe forms of CHD survive into adulthood. However, this increase in survival comes with a cost. CHD survivors have neurological functioning at the bottom of the normal range. A large spectrum of central nervous system dysmaturation leads to the deficits seen in critical CHD. The heart develops early during gestation, and CHD has a profound effect on fetal brain development for the remainder of gestation. Term infants with critical CHD are born with an immature brain, which is highly susceptible to hypoxic-ischemic injuries. Perioperative blood flow disturbances due to the CHD and the use of cardiopulmonary bypass or circulatory arrest during surgery cause additional neurological injuries. Innate patient factors, such as genetic syndromes and preterm birth, and postoperative complications play a larger role in neurological injury than perioperative factors. Strategies to reduce the disability burden in critical CHD survivors are urgently needed.
先天性心脏病（CHD）是最常见的出生缺陷之一。由于外科技术和重症监护的进步，大多数患有严重 CHD 的儿童能够存活到成年。然而，这种存活率的提高是有代价的。CHD 幸存者的神经功能处于正常范围的下限。广泛的中枢神经系统发育不良导致了严重 CHD 中出现的缺陷。心脏在妊娠早期发育，CHD 对妊娠后期胎儿大脑发育有深远影响。患有严重 CHD 的足月儿出生时大脑未成熟，极易受到缺氧缺血性损伤。由于 CHD 导致的围手术期血流紊乱以及在手术中使用体外循环或循环停止会导致额外的神经损伤。先天的患者因素，如遗传综合征和早产，以及术后并发症在神经损伤中的作用比围手术期因素更大。迫切需要制定策略来降低严重 CHD 幸存者的残疾负担。
