76例先天性心脏病合并重度肺动脉高压的外科治疗

Surgical treatment for 76 patients of congenital heart disease with severe pulmonary arterial hypertension

目的 探讨先天性心脏病（先心病）合并重度肺动脉高压患者的手术适应证、围手术期处理和术后降肺动脉压治疗方法.方法 回顾2005年6月至2010年10月收治的76例先心病合并重度肺动脉高压的患者,男24例,女52例;年龄（19.4±12.6）岁.超声心动图估测肺动脉收缩压64～126mmHg,平均（92.8 ±15.3） mmHg（1 mmHg =0.133 kPa）.53例行右心导管检查：吸氧前肺动脉平均压56～102 mmHg,平均（79.0±11.1） mmHg;Qp/Qs 0.60～ 3.33,平均1.48 ±0.78,肺血管阻力40.5～262.7 kPa·s·L-1,平均（128.9±51.2） kPa·s·L-1;吸氧试验后肺动脉平均压51 ～ 96 mmHg,平均（72.8±11.2） mmHg;Qp/Qs 0.96～ 5.33,平均1.98 ±0.89;肺血管阻力22.2～214.1 kPa·s· L-1,平均（90.2 ±38.4）kPa·s·L-1.经降肺动脉压治疗后,行心内畸形矫治术,术后加强心肺护理,继续降肺动脉压治疗.生存患者定期随访.结果 围手术期院内死亡6例,生存70例.术后1～2周复查超声心动图,估测肺动脉收缩压31～ 106 mmHg,平均（63.8 ±15.8） mmHg,较术前下降31.3％.随访2～5年,失访6例;随访64例中6例肺动脉压渐进性升高,其中3例术后2年内死亡.结论 经过降肺动脉药物治疗调整后,右心导管检查吸氧试验后Qp/Qs＜1为手术绝对禁忌证;≥1.0～1.3,术后肺动脉压下降不明显,甚至继续进展,不建议手术;≥1.3～1.5,术后早期并发症多、风险高,手术需谨慎;≥1.5～2.0,多可手术,长期预后尚需随访观察;＞2.0,术后肺动脉压多可得到较好控制.围手术期的处理直接影响到手术的近期疗效,而术后的综合、长期、个体化的降肺动脉压治疗,可以改善生活质量和预后.

Objective To investigate surgical indications,perioperative management and postoperative treatment of congenital heart disease（CHD） with severe pulmonary arterial hypertension（PAH）.Methods 76 patients of CHD with severe PAH underwent surgical operation from Jun 2005 to Oct 2010 in our hospital.Ultrasound cardiograph estimation of their pulmonary artery systolic pressure were between 64 mmHg to 126 mmHg,with an average of（92.8 ± 15.3） mmHg.Right cardiac catheterization illustrated that mean pulmonary artery pressure ranged from 56 mmHg to 102 mmHg[mean（79.0 ± 11.1） mmHg] without oxygen inhalation administration,Qp/Qs 0.60-3.33 （mean 1.48 ± 0.78）,pulmonary vascular resistance （PVR） 40.5-267.6 kPa · s · L-1 [mean （128.9 ± 51.2） kPa · s · L-1].After oxygen inhalation administration,pulmonary artery pressure ranged from 51 mmHg to 96 mmHg [mean （72.8 ± 11.2） mmHg] ; Qp/Qs 0.96-5.33 （mean 1.98 ± 0.89）,PVR 22.2-214.1 kPa · s · L-1 [mean（90.2 ±38.4） kPa · s · L-1].Patients had operations to correct cardiac anomaly after the preparation of antihypertensive treatment.Cardiorespiratory care and antihypertensive measures of pulmonary pressure were administered after surgery.Results Follow-ups were made for the survival cases.Results 6 patients died in-hospital and 70 cases survived.Ultrasound cardiograph examination of pulmonary artery systolic pressure ranged from 31 mmHg to 106 mmHg[mean（63.8 ± 15.8） mmHg] with decline of 31.3% compared to preoperative stage.6 cases were lost in followup.In 2 to 5 years reexamination,6 patients＇pulmonary artery pressure increased progressively and 3 cases died in 2 years after surgery.Conclusion After medical therapy of pulmonary hypertension,right cardiac catheterization with oxygen inhalation test demonstrates that when value of Qp/Qs 〈 1.0,it is absolute contraindication.When Qp/Qs ranging from 1.0 to 1.3,pulmonary artery pressure will decrease a little and will continue to progressed,it should not be recommend to surgery.When values of 1.3 to 1.5,it implies that there will be more early complications and high risk for surgery.When values of 1.5 to 2.0,it has indications of surgery and follow-up needed to appraise long-term prognosis; When values more than 2.0,it means good results of surgery.Perioperative management directly affects the surgical outcome,however,postoperative comprehensive,longterm and individualized treatment of pulmonary hypertension could improve life quality and prognosis.