先天性左肺动脉缺如并肺动脉高压2例

目的提高临床医生对左肺动脉缺如伴肺动脉高压的认识和诊治水平。方法报告2例先天性左肺动脉缺如伴肺动脉高压病例,并复习相关文献。结果 2例男性患者,均因发作性劳累后胸闷气短多次就诊,最终经右心导管及肺动脉造影确诊为先天性左肺动脉缺如伴肺动脉高压,给予吸氧、强心、利尿、降低肺动脉压等治疗后病情缓解。结论肺动脉缺如发病率低,缺乏特异性,极易误诊、漏诊,早期诊断早期治疗可改善患者的生存质量及延长寿命。

Congenital absence of left pulmonary artery accompanying pulmonary hypertension: A report of two cases

Objective To improve the recognition and diagnosis and treatment of congenital absence of left pulmonary artery accompanying pulmonary hypertension.Methods Two cases of congenital absence of left pulmonary artery accompanying pulmonary hypertension were reported and related literature was reviewed.Results The two male patients were admitted to our hospital due to paroxysmal chest distress and short breath because of exertion,and diagnosed as congenital absence of left pulmonary artery accompanying pulmonary hypertension by right heart catheterization and pulmonary arteriography.Their condition was relieved after treatment with oxygen inhalation,cardiac tonics,diuretics,and pulmonary hypertension-reducing drugs.Conclusion Since the incidence of congenital absence of left pulmonary artery is low and lacks of specificity,it is easily misdiagnosed and even its diagnosis is missed.Its early diagnosis and treatment can improve the quality of life of such patients and prolong their life.