成人先天性心脏病合并重度肺动脉高压的围术期及中长期药物治疗观察

目的观察成人先天性心脏病合并重度肺动脉高压围手术期及中长期联合药物治疗效果．探讨其治疗规范。方法68例成人先天性心脏病合并重度肺动脉高压患者通过围手术期应用联合药物控制肺动脉压力并确定根治手术指征后，均在体外循环下进行根治手术，手术前、后对患者肺动脉压力进行监测并根据监测结果给予西地那非、前列地尔、NO吸入以及血管紧张素转化酶抑制剂（ACEI）、血管紧张素受体阻断剂（ARB）、钙离子拈抗剂等多种药物联合应用治疗方案，并对患者进行长期随访观察治疗效果。结果手术死亡1例，围手术期肺动脉高压危象6例均经治疗好转出院，除少数患者肺动脉压力反弹性升高外，多数患者经围手术期和长期不间断药物治疗后肺动脉压力控制满意，手术后中长期治疗效果良好。结论围术期和术后中长期多种药物联合应用治疗方案，对于降低成人先天性心脏病合并重度肺动脉高压患者围术期死亡率和维持远期良好治疗效果具有重要意义。

The peri-operative observation and mid-long term follow-up of pharmaceutic therapy in the adults of congenital heart disease combined with severe pulmonary hypertension

Objective To study the effects of peri-operative and mid-long term pharmaceutic therapy to the adults of congenital heart disease(CHD) combined with severe pulmonary hypertension(PH) and explore the standard protocol of drug treatment to these patients.Methods 68 adult CHD patients with severe PH were treated with combined pharmaceutic therapy in peri-opretation,including with sildenafil,alprostadil,NO inhalation,angiotensin-converting enzyme inhibitor(ACEI),angiotensin receptor blocking agent,calcium ion antagon and so on.All the patients were accomplished corrected operation with extracorporeal circulation.Then the pulmonary pressure and the effects of combined pharmaceutic therapy were continuously monitored.Results There was only one patients died after operation.Six patients have the complication of pulmonary hypertensive crisis after operation and went smoothly by given drugs and corresponding therapy.Most of the patients had good results through peri-operation and mid-long term treatments by follow-up and PH monitoring.Conclusion The peri-operative and mid-long term pharmaceutic therapy to the adults of CHD combined with severe PH has significant results in reducing the peri-operative mortality and maintenance the long term operative therapeutic efficacy.