Schoetzau A, van Santen F, Sauer U, Irl C
Institut für Strahlenhygiene, Bundesamt für Strahlenschutz, Oberschleissheim/Neuherberg.
Z Kardiol. 1997 Jul;86(7):496-504. doi: 10.1007/s003920050085.
The study presents data on cardiovascular malformations in Bavarian livebirths, born between 1984 and 1991. Cases have been ascertained retrospectively by reviewing hospital records of all children being referred to a children's hospital up to 2 years of age. The classification scheme was based on abnormalities in developmental mechanisms. Among 984,570 livebirths, 7020 cases with structural congenital heart disease were identified. The birth prevalence was 7.1 per 1000 livebirths. Between 1984 and 1991, total prevalence increased from 5.9/10(3) to 8.0/10(3). Prevalence in males was 7.3/10(3) and in females 6.9/10(3). 78.1% of all heart defects were isolated, the remaining 21.9% were associated either with chromosomal abnormalities (9.6%), non-chromosomal syndromes (1.0%), or noncardiac malformations of other organ systems (11.3%). Total fatality rate was 12.0%, with two thirds of deaths occurring within a month of birth or the following month of life. Data were compared with those of the Baltimore-Washington Infant Study. This study presents for the first time regional data on birth prevalences of congenital heart defects in Germany. The classification scheme reduces the wide spectrum of phenotype cardiovascular defects to several pathogenetic groups. The defects in each group may be related to similar causal factors.
该研究展示了1984年至1991年间巴伐利亚州活产儿心血管畸形的数据。通过回顾所有转诊至儿童医院的2岁以下儿童的医院记录，对病例进行了回顾性确定。分类方案基于发育机制异常。在984,570例活产儿中，确定了7020例结构性先天性心脏病病例。出生患病率为每1000例活产儿中有7.1例。1984年至1991年间，总患病率从5.9/10³升至8.0/10³。男性患病率为7.3/10³，女性为6.9/10³。所有心脏缺陷中有78.1%为孤立性，其余21.9%与染色体异常（9.6%）、非染色体综合征（1.0%）或其他器官系统的非心脏畸形（11.3%）相关。总死亡率为12.0%，三分之二的死亡发生在出生后一个月内或出生后的第二个月。将数据与巴尔的摩-华盛顿婴儿研究的数据进行了比较。本研究首次展示了德国先天性心脏病出生患病率的区域数据。分类方案将广泛的心血管表型缺陷归为几个致病组。每组中的缺陷可能与相似的病因相关。
