摘要
目的探讨特发性肺动脉高压(idiopathic pulmonary hypertension,IPH)的临床表现及诊治要点,以提高对本病的认识,减少误诊误治。方法对我院收治的2例IPH死亡的临床资料进行回顾性分析并复习相关文献。结果 2例均为男性,年龄6岁和21岁。临床主要表现为颜面部及双下肢水肿、尿量减少、发热、胸闷。超声心动图示右室扩大,肺动脉收缩压分别为80、90 mmHg。例1误诊为肥厚性心肌病,例2误诊为上呼吸道感染。经胸部X线、超声心动图、自身免疫性抗体谱等检查排除继发性肺动脉高压,诊断为IPH。予扩张肺血管、利尿、强心、抗凝、抗感染等治疗,最终均因重度肺动脉高压所致呼吸、循环衰竭于入院3 d和6 d死亡。结论 IPH临床少见,症状缺乏特异性,易误诊,故加强对此病的认识、熟悉肺动脉高压的诊断程序和分类是正确诊断本病的关键。
Objective To explore clinical manifestations and the key points of treatment of idiopathic pulmonary hyperten- sion （IPH） so as to improve the understanding of the disease and reduce misdiagnosis rate. Methods Clinical data of 2 dead cases of IPH in our hospital were retrospectively analyzed with pertinent literature review. Results Two male patients were at the age of 6 years and 21 years respectively. Clinical symptoms were dropsy in facial region and double lower limbs, hypourocrinia, fever and dyspnea. Echocardiography showed that expanded right ventricular and pulmonary arterial systolic pressure were 80 mmHg and 90 mmHg respectively. Two patients were misdiagnosed as having hypertrophic cardiomyopathy and upper respiratory tract infection respectively. 1PH was diagnosed and secondary pulmonary hypertension was obviated by the chest X-ray, echocardiography and au- toimmunity antibody repertoire examinations. The treatment of expansion pulmonary vascular, diuresis, cardiotonic, anticoagulation arid anti-infection were performed, and the two patients died of the respiration and circulation failure due to severe hypertension pul- monary at 3rd and 6th day after hospitalization. Conclusion IPH is extremely rare, and lack of specificity may result in misdiagnosis. Clinicians should strengthen understanding of the disease and be familiar with the diagnosis program and classification of IPH.
出处
《临床误诊误治》
2013年第1期24-26,共3页
Clinical Misdiagnosis & Mistherapy
关键词
高血压
肺性
死亡
呼吸衰竭
循环衰竭
Hypertension, pulmonary
Death
Respiratory failure
Circulatory collapse
