特发性肺动脉高压39例临床分析

目的提高对特发性肺动脉高压（IPAH）的认识和早期诊治水平。方法回顾北京安贞医院1997年10月至2010年6月收治的39例IPAH患者的临床资料,对IPAH的临床表现、实验室检查、诊断、治疗等进行总结分析。结果 39例患者中,男14例（35.9%）,女25例（64.1%）;平均年龄（29.7±16.4）岁。临床表现主要有活动性呼吸困难/气促（90.9%）、胸闷（72.7%）、胸痛（30.7%）、咳嗽（41.0%）、乏力（48.7%）、晕厥（35.9%）、发绀（28.2%）、双下肢水肿（43.6%）等。超声心动图检查示39例肺动脉收缩压（SPAP）平均为（88.8±24.2）mm Hg,37例右心扩大,2例右心正常。肺动脉造影检查36例表现为中心型肺动脉扩张、外周血管纹理稀疏紊乱,3例未见明显异常。15例患者在右心导管下行急性肺血管扩张试验,肺动脉收缩压平均为（77.6±27.83）mm Hg,3例阳性,阳性率为20.0%。入院时24例被误诊,误诊率为61.5%,平均误诊时间为（26.0±24.5）个月。2008年4月前21例患者中20例给予一般内科治疗,1例肺移植。其后18例中13例给予西地那非等肺动脉高压靶向药物治疗。住院期间死亡2例,死亡率5.1%。结论 IPAH临床上少见,以中青年居多,女性多于男性,症状无特异性,容易被误诊。超声心动图、肺动脉造影等有较大诊断价值。右心导管检查及急性肺血管扩张试验有待进一步推广。治疗上应尽早应用肺动脉高压靶向药物,终末期患者可考虑肺移植。

Clinical Analysis of 39 Patients with Idiopathic Pulmonary Arterial Hypertension

Objective To improve the knowledge of idiopathic pulmonary arterial hypertension（IPAH） to elevate the levels of early diagnosis and treatment.Methods The clinical data of 39 IPAH patients admitted in Beijing Anzhen Hospital from October 1997 to June 2010 were reviewed.Results Of the 39 IPAH patients,14 cases were male and 25 cases were female,with an average age of（29.7±16.4） years old.Main clinical manifestations were exertional dyspnea/breathlessness（90.9%）,chest tightness（72.7%）,chest pain（30.7%）,cough（41.0%）,fatigue（48.7%）,syncope（35.9%）,cyanosis（28.2%）,edema of lower extremity（43.6%）,etc.As revealed through echocardiography,39 cases had a mean systolic pulmonary arterial pressure（SPAP） of（88.8±24.2）mm Hg,with right ventricle enlargement in 37 cases and normal in 2 cases.Pulmonary angiography showed central pulmonary arterial dilatation with pruning of the peripheral blood vessels in 36 cases and normal in 3 cases.Right heart catheterization and acute vasodilator testing was performed with iloprost in 15 patients,systolic pulmonary arterial pressure was（77.6±27.8）mm Hg,and positive rate was 20.0%.24 cases were misdiagnosed at admission,and misdiagnosis rate was 61.5%.The average time of misdiagnosis was（26.0±24.5）months.20 cases were treated with general medical therapy and 1 case was managed with lung transplantation before April 2008.Then 13 cases were given pulmonary arterial hypertension-targeted therapies,including sidenafil,iloprost or bosentan.Two patients died in hospital with a mortality rate of 5.1%.Conclusions IPAH is uncommon and often occurs in young and middle-aged women.The symptoms are nonspecific and easily misdiagnosed.Echocardiography and pulmonary angiography are helpful in diagnosis.Right heart catheterization and acute vasodilator testing should be carried out if available.The patients should be early treated with pulmonary arterial hypertension-targeted therapies.Lung transplantation may be an option for end-stage cases.