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Pulmonary hypertension associated with vein of Galen malformation. Fetal cardiac hemodynamic findings and physiological considerations

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Abstract

The management of newborns with vein of Galen aneurysmal malformation (VGAM) is clinically challenging for neonatologists and cardiologists. Hemodynamic profiles in four fetuses diagnosed with VGAM who subsequently developed neonatal cardiac failure and pulmonary hypertension were studied using two-dimensional and Doppler echocardiography. All four had an increased cardiothoracic ratio due to right ventricular dilatation on antenatal ultrasound. Doppler studies of the aortic isthmus were abnormal with retrograde flow starting in mid systole and throughout diastole. Left and right ventricular outputs were significantly increased. Net pulmonary flow was highly abnormal with a flow rate almost four times higher than normal. After a short period of clinical stability, all neonates developed cardiac failure and pulmonary hypertension. This article reviews VGAM pathophysiology and the potential relationship between pulmonary hypertension and VGAM, supporting early post-natal treatment of pulmonary hypertension and right ventricular failure.

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Fig. 1: Echocardiographic anomalies in fetuses with VGAM.
Fig. 2: Schematic representation of the fetal circulation in normal fetus (A) and fetuses with VGAM (B).

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Authors and Affiliations

  1. Division of Fetal and Pediatric Cardiology, University of Montreal, Sainte-Justine University Hospital Center, Montreal, QC, Canada

    Ana Mendez, Francisco Gonzalez Barlatay & Marie-Josée Raboisson

  2. Pediatric Cardiology Unit, Hospital Virgen del Rocío, Institute of Biomedicine of Seville, Seville, Spain

    Ana Mendez

  3. Division of Obstetrics, University of Montreal, Sainte-Justine University Hospital Center, Montreal, QC, Canada

    Elisabeth Codsi

  4. Division of Neonatology, Department of Paediatrics, Sainte-Justine University Hospital Center, Montréal, QC, Canada

    Anie Lapointe

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  1. Ana Mendez
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  3. Francisco Gonzalez Barlatay
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  5. Marie-Josée Raboisson
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Contributions

AM, FG, AL, and MJR contribute to conception and design. AM was responsible to acquisition of data. EC revised the final paper. AM, EC, FG, AL, and MJR contribute to analysis and interpretation of data. All authors contribute to drafting the paper of revising it critically for important intellectual content. All authors contributed to write up of the paper and approved the final version for submission.

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Correspondence to Marie-Josée Raboisson.

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The authors declare no competing interests.

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All procedures performed in the study involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Mendez, A., Codsi, E., Gonzalez Barlatay, F. et al. Pulmonary hypertension associated with vein of Galen malformation. Fetal cardiac hemodynamic findings and physiological considerations. J Perinatol 42, 143–148 (2022). https://doi.org/10.1038/s41372-021-01297-y

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