Cyanotic congenital heart disease (CCHD): focus on hypoxemia, secondary erythrocytosis, and coagulation alterations
Cyanotic congenital heart disease (CCHD): focus on hypoxemia, secondary erythrocytosis, and coagulation alterations
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DOI：
10.1111/pan.12705
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发表时间：
2015-10-01
影响因子：
1.7
通讯作者：
Guzzetta, Nina A.
中科院分区：
文献类型：
--
作者：
Zabala, Luis M.;Guzzetta, Nina A.
紫绀型先天性心脏病（CCHD）患儿由于长期低氧血症，其全血成分和凝血功能发生复杂的改变。继发性红细胞增多症是一种旨在增加循环红细胞和携氧能力的相关生理反应。然而，这种反应经常被全血粘度的增加所抵消，全血粘度的增加反而减少了血流和组织灌注。此外，伴随的血浆体积减少导致多种凝血蛋白（包括血小板、纤维蛋白原和其他凝血因子）的显著不足。一方面，这些患者可能患有严重的高粘血症和外周血管中的亚临床淤积，血栓形成的风险增加。另一方面，由于其止血特征的复杂紊乱，他们术后出血的风险增加。照顾患有CCHD和继发性红细胞增多症的儿童的麻醉师需要了解这些改变的病理生理学，并了解降低出血和/或血栓形成风险的可用策略。本综述的目的是对原发性先天性心脏病患儿长期低氧血症的全身影响进行最新分析，特别关注继发性红细胞增多和止血。
Children with cyanotic congenital heart disease (CCHD) have complex alterations in their whole blood composition and coagulation profile due to long-standing hypoxemia. Secondary erythrocytosis is an associated physiological response intended to increase circulating red blood cells and oxygen carrying capacity. However, this response is frequently offset by an increase in whole blood viscosity that paradoxically reduces blood flow and tissue perfusion. In addition, the accompanying reduction in plasma volume leads to significant deficiencies in multiple coagulation proteins including platelets, fibrinogen and other clotting factors. On the one hand, these patients may suffer from severe hyperviscosity and subclinical sludging' in the peripheral vasculature with an increased risk of thrombosis. On the other hand, they are at an increased risk for postoperative hemorrhage due to a complex derangement in their hemostatic profile. Anesthesiologists caring for children with CCHD and secondary erythrocytosis need to understand the pathophysiology of these alterations and be aware of available strategies that lessen the risk of bleeding and/or thrombosis. The aim of this review is to provide an updated analysis of the systemic effects of long-standing hypoxemia in children with primary congenital heart disease with a specific focus on secondary erythrocytosis and hemostasis.