先天性左肺动脉缺如并动脉导管未闭、主动脉骑跨1例  

Congenital absence of left pulmonary artery accompanying patent ductus arteriosus and aortic overriding:a case report

作　　者：张瑜 向玉鸾 李利华 ZHANG Yu;XIANG Yu-luan;LI Li-hua(Department of Geriatrics,the First Affiliated Hospital of the Dali University,Yunnan Dali 671000,China)

机构地区：[1]大理大学第一附属医院老年病科,云南大理671000

出　　处：《中西医结合心血管病电子杂志》2018年第24期7-9,12,共4页Cardiovascular Disease Electronic Journal of Integrated Traditional Chinese and Western Medicine

基　　金：国家自然科学基金(编号:81460084;81660072);大理大学博士启动基金(编号:2014-21);大理大学大学生创新项目(编号:X-CXCY2015-11);云南省中青年学术和技术带头人培养(编号:2015HB056);云南省医学学科带头人培养(D-201672);云南省高校重点实验室建设项目(编号:云教科[2016]37号)

摘　　要：1例36岁女性患者,因活动后胸闷、心悸、气促就诊,最终经肺动脉CTA确诊为先天性左肺动脉缺如并动脉导管未闭、主动脉骑跨,予吸氧、利尿、扩血管、抗凝、降低肺动脉压等治疗后病情缓解。先天性肺动脉缺如发病率极低,常合并其他心血管畸形,由于临床表现缺乏特异性,极易误诊及漏诊。目前诊断的金标准仍为肺动脉造影检查和右心导管检查,早期诊断及治疗可改善患者的生存质量及延长寿命。目前先天性肺动脉缺如的治疗多为对症处理,行患侧肺动脉重建术是理想的手术治疗方案,但目前在我国还无法实行。目前手术治疗主要为解决合并的其他心血管畸形。A 36-years old female patient was admitted duo to chest distress,palpitation and exertional dyspnea,and diagnosed as congenital absence of left pulmonary artery accompanying patent ductus arteriosus and aortic overriding by computed tomographic pulmonary angiography.These symptoms were relieved after treatment with oxygen inhalation,diuretics,vasodilator,anticoagulants and pulmonary hypertensionreducing drugs.The incidence of congenital absence of pulmonary artery is low and usually combines with other cardiovascular malformations.It is easy to underdiagnose and even misdiagnose because lacking specific clinical manifestations.At present,pulmonary angiography and right heart catheterization remains the gold standard for diagnosis of congenital absence of pulmonary artery,and early diagnosis and treatment can improve patients’quality of life and prolong the life.The treatment of congenital absence of pulmonary artery is mainly symptomatic management,and ipsilateral pulmonary artery reconstruction is an ideal surgical treatment scheme,but it is not available in China at present.Now surgical treatment of congenital absence of pulmonary artery is to repair the combining cardiovascular malformations.

关 键 词：先天性肺动脉缺如 动脉导管未闭 主动脉骑跨 肺动脉畸形 肺动脉高压 

分 类 号：R541.1[医药卫生—心血管疾病]