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.

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    ABSTRACT: Preferential left heart underdevelopment in human fetuses with diaphragmatic hernia is almost uniformly associated with preferential streaming of the ductus venosus toward the right side of the heart. The purpose of our study was to examine whether this flow anomaly might also be present in other fetuses with hypoplasia of left-sided cardiovascular structures. We studied 32 fetuses with left heart hypoplasia as defined by gestational age-related Z-scores by echocardiography. Apart from defining cardiovascular morphology as detailed as possible, particular focus was put on the presence or absence of preferential streaming of the ductus venosus and inferior caval vein toward the right side of the heart. 8 of 32 fetuses with hypoplasia of one or more left-sided cardiovascular structures exhibited preferential streaming of the ductus venosus toward the right side of the heart. In all eight fetuses, this finding was associated with additional cardiovascular anomalies like aortic valve stenosis, ventricular septal defect, left superior caval vein-to-coronary sinus, pulmonary vein stenosis and/or aortic arch hypoplasia. As in human fetuses with left diaphragmatic hernia, preferential ductus venosus streaming toward the right side of the heart can be found in a subgroup of fetuses with hypoplasia of left-sided cardiovascular structures.
    No preview · Article · Dec 2011 · Ultraschall in der Medizin
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