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: 4.12). 08/2011; 32 Suppl 2:E151-6. DOI: 10.1055/s-0031-1281651
Source: PubMed

ABSTRACT 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.

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    ABSTRACT: Left-sided congenital diaphragmatic hernia (LCDH) is often associated with a smaller left ventricle, yet in most cases this finding has no apparent functional impact and seems to resolve after birth. In right-sided CDH (RCDH) little is known about fetal cardiac size and function. The aim of this study was to evaluate fetal cardiac anatomy and function in RCDH and to compare values with normal controls. Fetal echocardiography was performed in 17 consecutive cases with isolated RCDH and 17 gestational age matched controls. Two-dimensional measurements included ventricular and outflow tract diameters. Doppler ultrasound was used to measure the flow pattern in the ductus venosus, over the pulmonary and aortic valve, right and left ventricular myocardial performance index and the E/A wave ratio over the atrioventricular valves. Stroke volumes, cardiac output and shortening fractions were calculated. Median GA at evaluation was 27.4 weeks (IQR 24.4-28.9 weeks). RCDH cases were associated with a significantly smaller right ventricle and pulmonary valve diameter. Furthermore, stroke volume and cardiac output from the right ventricle were also lower than in controls. Myocardial contractility however appeared normal. Despite significantly reduced right ventricular dimensions and cardiac output, cardiac contractility was normal in a cohort of fetuses with right-sided CDH.
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