Abstract

In 11 of 21 fetuses with prenatally diagnosed complete heart block, congestive heart failure with signs of non-immune hydrops fetalis was seen. In all 11 fetuses, cardiac malformations were present: complete atrioventricular canal with levocardia, situs inversus viscerum, and polysplenia: 5 cases; tricuspid atresia with situs inversus viscerum, endocardial fibroelastosis, common atrium with aortic valve atresia, hypoplastic left heart, atrial septal defect of secundum type: one case each. All these cardiac malformations can also cause intrauterine heart failure without heart block except hypoplastic left heart and atrial septal defect. A review of the literature confirms, that only the association between complete heart block and cardiac malformation--in particular AV-canal and endocardial fibroelastosis--can cause an intrauterine congestive heart failure, whereas in the case of fetal complete heart block without cardiac malformation or with prenatally hemodynamically insignificant cardiac malformations--in particular "corrected" transposition of great arteries--a congestive heart failure is rare.

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