Gembruch U, Hansmann M, Redel D A, Bald R, Knöpfle G
Department of Prenatal Diagnosis and Therapy, University of Bonn, F.R.G.
Eur J Obstet Gynecol Reprod Biol. 1989 Apr;31(1):9-22. doi: 10.1016/0028-2243(89)90022-1.
Complete heart block was diagnosed prenatally in 21 fetuses. Associated structural cardiac defects were present in 18 fetuses, in particular complete atrioventricular canal with atrial isomerism (5 cases), and 'corrected' transposition of the great arteries (4 cases). Maternal systemic lupus erythematosus was proved in only one case. In 11 fetuses, intra-uterine congestive heart failure with the signs of non-immune hydrops fetalis occurred. In all 11 fetuses, the hydrops was associated with a cardiac defect, in particular complete atrioventricular canal with atrial isomerism in 5 cases. A review of the literature confirms that only the association of complete heart block and cardiac malformation can cause intra-uterine congestive heart failure, whereas in the case of fetal complete heart block without cardiac malformation or with prenatally hemodynamically insignificant cardiac malformation, congestive heart failure is rare. Only 30% of newborns with complete heart block have associated cardiac malformations. In our series, however, 86% of the fetuses with complete heart block had cardiac malformations. The most important reason for this percentage discrepancy is that almost all fetuses with associated severe cardiac defects, in particular atrioventricular canal defects, develop heart failure which frequently results in prenatal death. Thus, fetal deaths are not included in pediatric statistics. Nevertheless, fetuses with isolated complete heart block generally do not develop heart failure and in almost all of the cases are born alive.
21例胎儿在产前被诊断为完全性心脏传导阻滞。18例胎儿伴有心脏结构缺陷，尤其是完全性房室通道合并心房异构（5例）以及大动脉“矫正型”转位（4例）。仅1例孕妇被证实患有系统性红斑狼疮。11例胎儿出现宫内充血性心力衰竭并伴有非免疫性胎儿水肿的体征。在这11例胎儿中，水肿均与心脏缺陷相关，尤其是5例完全性房室通道合并心房异构。文献回顾证实，只有完全性心脏传导阻滞与心脏畸形同时存在才会导致宫内充血性心力衰竭，而对于无心脏畸形或产前血流动力学意义不显著的心脏畸形的胎儿完全性心脏传导阻滞病例，充血性心力衰竭较为罕见。仅有30%的完全性心脏传导阻滞新生儿伴有心脏畸形。然而，在我们的系列研究中，86%的完全性心脏传导阻滞胎儿存在心脏畸形。造成这一百分比差异的最重要原因是，几乎所有伴有严重心脏缺陷（尤其是房室通道缺陷）的胎儿都会发生心力衰竭，这常常导致产前死亡。因此，胎儿死亡未被纳入儿科统计数据中。不过，孤立性完全性心脏传导阻滞的胎儿一般不会发生心力衰竭，并且几乎所有病例都能存活出生。
