先天性心脏病肺动脉高压手术前后右心功能变化和治疗

Preoperative and postoperative right heart function and treatment of children with severe pulmonary hypertension due to left-to-right shunting

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右 心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿,用心导管 的方法进行术前、术后5-7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排 指数显著高于术后;术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高,缺损修补后右室舒张压恢复正 常,收缩压的降低与肺动脉压力下降有关;大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出 量心力衰竭,且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物;降低肺动脉压力、减少左向右 分流、根治心内畸形是合理的选择;术后右室收缩压持续不能恢复至正常水平,提示继发性肺血管梗阻性病变存 在。

Objective To study the right heart function prior to and after operation in children with severe pulmonary hypertension(PH) caused by congenital heart disease with left-to-right shunting. Methods 40 children with severe PH(increased total pulmonary circulation resistance) due to large ventricular septum defect (VSD) were followed consecutively with cardiac catheterization prior to and after cardiac surgery for 5 to 7 years. Results Hemodynamic parameters of the pulmonary circulation showed that preoperative right cardiac output index, right ejection index and right cardiac index were higher than those after operation. Right atrial pressure, right ventricular systolic/diastolic pressure, pulmonary artery pressure and resistance were significantly increased before surgery. After operation, right atrial pressure and right ventricular diastolic pressure were all become normal.The postoperative decrease of right ventricular systolic pressure was related to the decreased pressure in the pulmonary artery. Right heart failure in VSD patients with severe PH was not only the result of increased cardiac output, but also the excessive pre-load which was indicated by an increased right ventricular diastolic pressure. Conclusions In the treatment of heart failure, positive inotropic drug should not always be considered the first choice, reduction of pulmonary artery pressure and correction of left-to-right shunting are also reasonable therapeutic selections. The persistence of post-operative pulmonary hypertension indicates secondary obstruction of pulmonary arterial vessels in VSD patients.