先天性房间隔缺损病人出现迟发性紫绀——艾森门格综合征?(附2例分析)

Tardive Cyanosis in Patients with Congenital Atrial Septal Defects -- Eisenmenger Syndrome ? (Two cases attached)

【目的】探讨先天性房间隔缺损(ASD)出现迟发性紫绀与艾森门格综合征的鉴别,提高对此类疾病的诊疗水平,以免判断失误,丧失治疗机会。【方法】复习2例曾在外院诊断为艾森门格综合征的先天性房间隔缺损患者的临床资料,分析其误诊的原因及危害。其临床表现均有口唇、指甲轻～中度紫绀,并伴有胸闷气促、乏力及明显右心功能不全的相关症状和体征。超声心动图(UCG)提示:右心房、右心室增大,房间隔缺损,彩色多普勒血流显像(CDFI)示双期双向分流,肺动脉瓣轻度,三尖瓣中～重度关闭不全。心导管检查证实肺动脉高压仅为轻～中度【。结果】2例患者均经术中探查进一步排除艾森门格综合征,1例为下腔型巨大房间隔缺损,另1例为心房间隔缺损合并原发性重度三尖瓣环扩大,两例均顺利施行手术矫治,并康复良好出院。【结论】某些迟发性紫绀的先天性心脏病与艾森门格综合征较难鉴别,诊断务必谨慎。

[Objective] To investigate the indentification of tardive cyanosis from eyanosis associated with Eisenmenger syndrome in patients with congenital atrial septal defects （ASD）, to promote the diagnosis and treatment of these diseases in order to avoiding misdiagnosis. [Methods] Clinical data of two patients with congenital ASD, who had ever been diagnosed as Eisenmenger syndrome in other hospital were reviewed and the causes and dangers for misdiagnosis were analyzed. Their common clinical manifestations were cyanosis in labia otis and nail, and associated with chest distress, tachypnea and hypodynamia after slight physical activity, as well as some apparently related signs and symptoms of right heart disfunetion. Ultrasonic cardiography （UCG） showed that right atrium and ventricle dilated, ASD. And color doppler flow imaging （CDFI） indicated that left-to-right and（or） right-to-left shunt at the atrial level both in systole and diastole, regurgitation in pulmonary valve was mild, and in tricuspid valve was mederate-severe. Cardiac catheterization confirmed that pulmonary hypertension was only mild to moderate. [Results] Two patients were excluded Eisenmenger syndrome further in operation, one was confirmed huge ASD of inferior vena cava patter, and the other was ASD coexist primary tricuspid annulus dilation severely. Both of them received surgical correction successfully and discharged after well-recovered. [Conclusion] Tardive cyanosis caused by some congenital heart diseases must be carefully diagnosed for the similarity to that of Eisenmenger syndrome.