Association of Intrathoracic Herniation of the Liver with Left Heart Hypoplasia in Fetuses with a Left Diaphragmatic Hernia but not in Fetuses with a Right Diaphragmatic Hernia

Department of Obstetrics and Prenatal Medicine, University Hospital of Bonn, Bonn, Germany.
Ultraschall in der Medizin (Impact Factor: 4.92). 08/2011; 32 Suppl 2(S 02):E151-6. DOI: 10.1055/s-0031-1281651
Source: PubMed


Predominant left heart hypoplasia is commonly observed in human fetuses with left diaphragmatic hernia and intrathoracic liver herniation ("liver-up"). In contrast, marked left/right heart disproportion has not been described in fetuses with right diaphragmatic hernia, despite intrathoracic herniation of large parts of the liver.
We analyzed the data of 15 fetuses with left diaphragmatic hernia and 10 fetuses with right diaphragmatic hernia and all with intrathoracic liver herniation ("liver-up") that were examined with fetal echocardiography between 21 weeks + 1 day and 35 weeks + 0 days of gestation. The inflow and outflow dimensions and Z-scores of the two groups were statistically compared. In addition, ductus venosus streaming patterns were examined.
Despite the presence of intrathoracic liver herniation, predominant left heart hypoplasia, defined as a mitral valve Z-score < - 2 in combination with a tricuspid valve Z-score that was at least 2 Z larger than the mitral valve Z-score in an individual fetus, was observed in 11 of the 15 fetuses with left diaphragmatic hernia but in none of the 10 fetuses (p < 0.001) with right diaphragmatic hernia. Preferential streaming to the right heart was observed in 14 of the 15 fetuses with left hernia but in none of the 7 fetuses with right diaphragmatic hernia in whom this flow information was available (p < 0.0001).
Intrathoracic herniation of the liver ("liver-up") is associated with predominant left heart hypoplasia in left diaphragmatic hernia but not right fetal diaphragmatic hernia. Our observations indicate that this difference may result from different ductus venosus streaming sites in these conditions.

12 Reads
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Purpose: The accuracy of the sonographic weight estimation (WE) of fetuses with congenital diaphragmatic hernia (CDH) is significantly lower than that of fetuses without any malformations. The objective of this study was to develop and evaluate the first specific sonographic weight formula for fetuses with CDH. Materials and methods: In a retrospective, multicenter, cohort study, a statistical estimation technique known as "multivariable fractional polynomial regression" was applied to a group of 146 fetuses with CDH. Each fetus underwent an ultrasound examination with complete biometric parameters within 7 days of delivery. A new formula was derived using the obtained data and was then compared with other commonly used equations. The accuracy of the different formulas was compared using means of signed percentage errors (SPE), medians of absolute percentage errors (MAPE), and fractions of estimates within prespecified error bounds. Results: The new derived formula is: EFW = 10^(4.6729 107 371 + 0.2365 011 768 * HC + 0.2228 897 682 * FL^2 - 0.0129 895 773 * FL^3 - 1.0470 039 072 * (FL * HC)^0.5 + 0.0004 314 661 * (AC * HC) - [in case of liver herniation] 0.0062 112 122), where EFW is the estimated fetal weight, HC is the head circumference, AC is the abdominal circumference, and FL is the femur length. The new formula proved to be superior to other established equations, showing both the lowest median absolute percentage error (MAE: 6.97) and mean signed percentage error (SPE: 0.40), and the best distribution of absolute percentage errors within prespecified error bounds. Conclusion: This new formula significantly improves weight estimation in fetuses with CDH.
    Ultraschall in der Medizin 11/2012; 34(6). DOI:10.1055/s-0032-1325337 · 4.92 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Purpose: Preferential streaming of the ductus venosus (DV) toward the right atrium has been observed in fetuses with left diaphragmatic hernia (LDH). The purpose of this retrospective study was to compare survival rates to discharge between a group with preferential streaming of the DV toward the right heart and a group in which this abnormal flow pattern was not present. Materials and methods: We retrospectively searched our patient records for fetuses with LDH in whom liver position, DV streaming and postnatal outcome information was available. 55 cases were found and divided into two groups: Group I fetuses exhibited abnormal DV streaming toward the right side of the heart; group II fetuses did not. Various prognostic and outcome parameters were compared. Results: 62 % of group I fetuses and 88 % of group II fetuses survived to discharge (p = 0.032). Fetoscopic tracheal balloon occlusion (FETO) was performed in 66 % of group I fetuses and 23 % of group II fetuses (p = 0.003). Postnatal ECMO therapy was performed in 55 % of group I fetuses and 23 % of group II infants (p = 0.025). Moderate to severe chronic lung disease in survivors was observed in 56 % of the survivors of group I and 9 % of the survivors of group II (p = 0.002). Conclusion: Preferential streaming of the DV toward the right heart in human fetuses with left-sided diaphragmatic hernia was associated with a poorer postnatal outcome despite a higher rate of invasive pre- and postnatal procedures compared to fetuses without this flow abnormality. Specifically, abnormal DV streaming was found to be an independent predictor for FETO.
    Ultraschall in der Medizin 05/2013; 34(6). DOI:10.1055/s-0032-1330702 · 4.92 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Congenital diaphragmatic hernia is a malformation presenting with varying degrees of severity. An accurate prediction of outcome is crucial for parental counselling and therapeutic planning. In selected cases, foetal endoscopic tracheal occlusion (FETO) can improve foetal outcome. Timely referral to a highly specialised centre is important when the requirement for extracorporeal membrane oxygenation (ECMO) is expected.
    Zeitschrift für Geburtshilfe und Neonatologie 02/2014; 218(1):6-17. DOI:10.1055/s-0034-1367041 · 0.48 Impact Factor
Show more