Objective
Congenital diaphragmatic hernia (CDH) is diagnosed by obstetricians who often described HLHS in such cases. The aim of this study was to evaluate heart abnormalities coexisting with CDH.
Material and methods
This study was prepared by the Student`s Scientific Club of Perinatal Cardiology. We evaluated echocardiograms of 35 fetuses examined between 2002-2008 with left sided CDH. Lung development, heart size, disproportion between LV, RV, pulmonary artery and aorta, co-existing heart defects were checked. The mean age of fetal echo was 29 weeks. Karyotype was done in 29 fetuses: 25 had normal and 4 abnormal.
Results
26 fetuses had isolated CDH and 8(23%) with associated heart defects. In all heart to chest area ratio was smaller than 0,25. Secondary left ventricular hypoplasia (LV less that 50% of RV), described by obstetricians as HLHS, was in 4 cases, none of them survived. Additional heart defects were: VSD 6, TOF – 1, critical pulmonary valve stenosis – 1, they didn’t survive either.
Conclusion
Heart defects complicated CDH is serious risk factor for survival, even if lungs were not severely hypoplastic. There were not fetuses with real HLHS, but secondary left ventricular hypoplasia caused low cardiac output what complicated respiratory failure in neonates with pulmonary hypoplasia due to CDH. There is still unknown reason for which just some fetuses with CDH developed secondary left ventricular hypoplasia so further studies will include the role of growth factors (TGF-β), transcription factors (Fog2, COUP-TFII, Gata4), genes (STRA6).