Hofstadler G, Tulzer G, Schmitt K, Mair R
Department für pädiatrische Kardiologie, Landeskinderklinik Linz.
Klin Padiatr. 1998 Jan-Feb;210(1):30-3. doi: 10.1055/s-2008-1043844.
Congenital complete atrioventricular block is a rare entity. The association between this disease, maternal connective tissue disease and maternal antibodies [anti-Ro (SS-A) resp. anti-La (SS B)] is well known. Diagnosis can be made by means of fetal Doppler-echocardiography by the 16th week of gestation. In our case diagnosis was established in the 21st week of gestation. Ventricular rate was 55/min, atrial rate 70/min. There were no signs of fetal hydrops. There were no signs of maternal connective tissue disease, but anti-Ro and anti-La antibodies could be detected. The mother was treated with steroids from the time of diagnosis until the end of pregnancy. Altogether 9 Doppler-echocardiographic studies were performed. A recurrence of normal rhythm did not occur. A slow but continuous decrease of atrial and ventricular rate was observed. Interestingly, there was no development of fetal hydrops until the very end of pregnancy when the fetal heart rate reached a low of 28 beats per minute. We speculate, that the therapy with steroids might have played an important role in the prevention of early hydrops. At the onset of fetal hydrops delivery should be considered. In symptomatic complete atrioventricular block we prefer the implantation of a permanent pacemaker system immediately after birth. Efficient care for the fetus resp. the newborn can only be achieved through well planned cooperation.
先天性完全性房室传导阻滞是一种罕见病症。这种疾病、母体结缔组织病与母体抗体[抗Ro（SS - A）及抗La（SS - B）]之间的关联已为人熟知。在妊娠第16周时可通过胎儿多普勒超声心动图进行诊断。在我们的病例中，诊断是在妊娠第21周时确定的。心室率为55次/分钟，心房率为70次/分钟。没有胎儿水肿的迹象。没有母体结缔组织病的体征，但可检测到抗Ro和抗La抗体。从诊断之时起直至妊娠结束，母亲接受了类固醇治疗。总共进行了9次多普勒超声心动图检查。未出现正常心律的复发。观察到心房率和心室率缓慢但持续下降。有趣的是，直到妊娠末期胎儿心率降至每分钟28次的最低点时，才出现胎儿水肿。我们推测，类固醇治疗可能在预防早期水肿方面发挥了重要作用。当出现胎儿水肿时，应考虑分娩。对于有症状的完全性房室传导阻滞，我们更倾向于在出生后立即植入永久性起搏器系统。只有通过精心规划的合作，才能为胎儿及新生儿提供有效的护理。
