先天性单侧肺动脉缺如的超声心动图诊断价值

Value of echocardiography in diagnosing unilateral absence of pulmonary artery

目的探讨超声心动图在先天性单侧肺动脉缺如(UAPA)诊断中的临床应用价值。方法回顾性研究2004～2010年经手术和(或)心导管检查证实的21例UAPA(左侧13例,右侧8例)的超声心动图表现。结果 14/21例(66.7%)首诊由超声心动图明确诊断,经手术或心导管检查证实;4/21例(19.0%)例首诊超声心动图发现异常,但未能明确诊断,经手术或心导管检查证实;3/21例(14.3%)首诊超声心动图漏诊,经手术或心导管检查发现。UAPA的超声心动图表现为主肺动脉向远端直接延续为一侧肺动脉,分叉结构消失,缺如一侧的肺动脉通过主-肺动脉侧支循环供血,常伴发复杂的心脏畸形。结论 UAPA较罕见,临床表现无特异性,容易漏诊,但是只要提高认识,仔细探查,超声心动图在UAPA的诊断中具有较高的临床应用价值。

Objective To explore the echocardiography value in unilateral absence of pulmonary artery（UAPA）.Methods A retrospective analysis was made of 21 cases with UAPA between 2004 with 2010.Twenty-one patients suffering UAPA of the left（n=13） or right（n=8） pulmonary artery underwent surgical interventions or cardiac catheter to acquire final diagnosis.Results Fourteen in twenty-one patients（66.7%） were first diagnozed UAPA by echocardiography,four patients were detected of abnormal pulmonary artery,and other three patients failed to be diagnozed by it,eventually all the patients were finally diagnozed by surgical interventions or cardiac catheter.The echocardiography manifestated complete absence of the left or right pulmonary artery branch.Aorticopulmonary collateral artery may support the absent pulmonary artery branch.UAPA is frequently accompanied with congenital cardiac malformations.Conclusion UAPA is a rare anomaly and the disease has non-specific presentation.Echocardiography is an effective method in the diagnosis of UAPA.