慢性血栓栓塞性肺动脉高压的诊断和治疗体会

Clinical observation of diagnosis and treatment in patients with chronic thromboembolic pulmonary hypertension

目的提高对慢性血栓栓塞性肺动脉高压(CTEPH)的认识和早期诊治水平。方法对我院1994年10月至2009年9月收治的104例CTEPH患者的临床资料进行回顾性分析。结果 CTEPH患者中男性多见,男:女为1.97:1,平均年龄46.4±16.7岁,病史37.0±19.6月。临床主要表现有活动性呼吸困难/气促、胸闷、胸痛及咯血等。所有患者超声心动图均示肺动脉高压,其他表现有右心室扩大、三尖瓣返流、主肺动脉内占位性病变等。而血管超声示下肢静脉血栓形成占68.3%。患者确诊均通过肺动脉造影(PPA,CTPA或MRPA)。入院时54例被误诊,误诊率为52%。41例给予内科治疗,63例行肺动脉血栓内膜剥脱术,死亡6例,总手术死亡率9.5%。57例成功手术者,肺动脉收缩压由术前93.2±35.8mmHg降至术后的51.2±14.1mmHg,动脉血氧分压由52.8±7.9mmHg升至86.4±9.2mmHg,二组前后相比有显著性差异(P<0.05)。结论 CTEPH临床上比较少见,容易被误诊。UCG和CTPA有较大诊断价值。内科治疗效果欠佳,肺动脉血栓内膜剥脱术应作为首选治疗手段。

Objective To increase the understanding of chronic thromboembolic pulmonary hypertension（CTEPH）and its level of the early diagnosis and treatment.Methods The clinical data of 104 CTEPH patients in Beijing Anzhen Hospital from October 1994 to September 2009 were retrospectively analyzed.Results （1）There were 69 males and 35 females with an average age of 46.4±16.7 years and a disease history of 37.0±19.6 months.（2）Main clinical manifestations were exertional dyspnea,oppressive chest,chest pain,cough,hemoptysis,syncope,edema of lower limbs,etc.（3）Enlarged right vehicle,tricuspid regurgitation,pulmonary arterial hypertension,massive lumps in main pulmonary truck were observed in echocardiography（UCG）,deep venous thrombosis of lower limbs were found in color doppler ultrasonography.（4）CT pulmonary angiography（CTPA）showed expansion of pulmonary artery,large filling defect in main pulmonary truck and right or left pulmonary artery.（5）54 cases were misdiagnosed,and misdiagnosis rate was 52%.（6）41 cases were treated with medical therapy,63 cases were managed with pulmonary thromboendarterectomy（PTE）and overall operative mortality rate was 9.5%（no death in 38 cases after September 2002）.（7）as compared with preoperative values,all survival patients presented with significant improvement of systolic pulmonary artery pressure[（51.2±14.1）mmHg versus（93.2±35.8）mmHg,P〈0.05] and arterial oxygen tension[（86.4±9.2）mmHg versus（52.8±7.9 ）mmHg,P〈0.05.Conclusion CTEPH is uncommon clinically,and is early misdiagnosed.UCG and CTPA are more helpful in the diagnosis.The effect of medical therapy is poor and PTE is the first option in the treatment of CTEPH.