先天性心脏病合并肺高压术后肺高压危象

Pulmonary Hypertensive Crisis Following Surgery for Congenital Heart Defects with Pulmonary Hypertension

目的：了解先天性心脏病（先心病） 并肺动脉高压症（ 肺高压） 术后肺高压危象的发生发展。方法：总结５０ 例１４岁以下先心病合并中、重度肺高压病人心内畸型矫治术后的早期经过，术后出现肺高压危象情况，其中３７ 例行肺活检。结果：术后出现肺高压危象９ 例，１８ 次，半数危象出现在术后２４ ｈ 以内。中度肺高压的病人术后出现危象的比例高于重度者，危象倾向于多次发生。危象病人的肺活检病理ＨｅａｔｈＥｄｗａｒｄｓ 分级均为轻、中度改变。危象的发生近半数（４４ ％） 有呼吸道刺激的诱因，另外近半数（４４％ ）危象未找到明确诱因。结论：先心病肺高压术后肺高压危象多出现于中度肺高压患者，治疗处理以镇静、高氧及血管扩张剂为主要手段，诱因明确的病人治疗效果较好。

Objective: To observe the happening and development of pulmonary hypertensive crisis in post operation of congenital heart defects with pulmonary hypertension. Methods: 50 patients less than 14 years of age with congenital heart defects associated with moderated or severe pulmonary hypertension were reviewed. All of the patients were monitored to identify and characterize pulmonary hypertensive crisis in the post operative period. Open lung biopsies were carried out in 37 patients. Results: 9 patients developed pulmonary hypertensive crisis (PHC) and totally 18 events were happened. Most of crises happened within 24 h after operation. The ratio of PHC in patients with moderated pulmonary hypertension was much higher than that with severe pulmonary hypertension. Heath Edwards classification in lung biopsy showed all PHC cases have mild or moderated pathologic change in pulmonary arteriole. Almost half of the crises were due to the stimulus of the trachea (44%); another half of the events found no definite cause (44%). Conclusion: Most cases of post operative pulmonary hypertensive crisis happen in congenital heart defects with moderate pulmonary hypertension. Analgesia, oxygen hyperventilation and pulmonary vasodilator are the main therapies; crises with definite causes will have a better result.