Adatia I, Barrow S E, Stratton P, Ritter J M, Haworth S G
Department of Developmental Vascular Biology and Pharmacology, Institute of Child Health, London.
Br Heart J. 1993 Feb;69(2):179-82. doi: 10.1136/hrt.69.2.179.
Children with cyanotic congenital heart disease and pulmonary outflow tract obstruction have shortened platelet survival times and are susceptible to thrombosis and organ infarction. Thromboxane A2 and prostacyclin have opposing actions on platelet aggregability and an imbalance in their biosynthesis might contribute to the pathophysiology of these complications.
Biosynthesis of thromboxane A2 and prostacyclin was investigated in 16 children (4-32 months, median 18 months) with cyanotic congenital heart disease and pulmonary outflow tract obstruction and compared with 16 healthy children of a similar age (6-34 months, median 24 months). Urinary excretion of 2,3-dinor-thromboxane B2 (a metabolite of thromboxane A2) and of 2,3-dinor-6-oxo-prostaglandin F1 alpha (a metabolite of prostacyclin) was measured.
The children with cyanotic congenital heart disease and pulmonary outflow tract obstruction excreted more 2,3-dinor-thromboxane B2 than the healthy children: 916(163) compared with 592(122) ng/g creatinine (mean(SEM); 2p = 0.014). The ratio of excretion of 2,3-dinor-thromboxane B2 to 2,3-dinor-prostaglandin F1 alpha was greater in the patients than in the healthy control group (2.38(0.28) v 1.3(0.22)) (2p = 0.002).
The balance between biosynthesis of prostacyclin and of thromboxane A2 is abnormal in children with cyanotic congenital heart disease and pulmonary outflow tract obstruction and favours platelet aggregation and vasoconstriction.
患有青紫型先天性心脏病和肺流出道梗阻的儿童血小板存活时间缩短，易发生血栓形成和器官梗死。血栓素A2和前列环素对血小板聚集性有相反作用，其生物合成失衡可能导致这些并发症的病理生理过程。
对16名患有青紫型先天性心脏病和肺流出道梗阻的儿童（4 - 32个月，中位年龄18个月）的血栓素A2和前列环素生物合成进行研究，并与16名年龄相仿的健康儿童（6 - 34个月，中位年龄24个月）作比较。测量尿中2,3 - 二去甲血栓素B2（血栓素A2的一种代谢产物）和2,3 - 二去甲 - 6 - 氧代前列腺素F1α（前列环素的一种代谢产物）的排泄量。
患有青紫型先天性心脏病和肺流出道梗阻的儿童排泄的2,3 - 二去甲血栓素B2比健康儿童多：分别为916(163) ng/g肌酐和592(122) ng/g肌酐（均值(标准误)；P = 0.014）。患者中2,3 - 二去甲血栓素B2与2,3 - 二去甲前列腺素F1α的排泄比值高于健康对照组（2.38(0.28) 对1.3(0.22)）（P = 0.002）。
患有青紫型先天性心脏病和肺流出道梗阻的儿童，前列环素和血栓素A2的生物合成平衡异常，有利于血小板聚集和血管收缩。
