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

Méndez, A.; Codsi, Elisabeth; Barlatay, Francisco Gonzalez; Lapointe, Anie; Raboisson, Marie‐Josée

doi:10.1038/s41372-021-01297-y

Cited by 2 publications

(6 citation statements)

References 27 publications

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“…In previous studies, the presence of fetal cardiomegaly and ventriculomegaly were associated with poor outcome [3,39,41,42]. Our prenatal data are overlapping with previous studies.…”

Section: Prenatal Managementsupporting

confidence: 86%

“…Cardiomegaly is present in almost 60% of cases. The increment of the cardio thorax index is mainly due to the dilatation of the superior vena cava and the right ventricle probably due to the high venous return from the brain [39]. Cardiac involvement is less present in fetal life compared to the post-natal period due to the low vascular resistances of VGM that balanced by the low resistance of the uteroplacental unit [3,40].…”

Section: Prenatal Managementmentioning

confidence: 99%

“…We advise also a genetic consult. A fetal ultrasound with fetal echocardiography should be offered at least every two weeks [3,39,40]. The type of birth should be personalized for each case; in the absence of cardiological dysfunction and no other contraindication, vaginal delivery can be performed safely [1].…”

Section: Prenatal Managementmentioning

confidence: 99%

“…The genesis is due to the volume and pressure overload in the right chambers. For the low vascular resistance in the head, the majority of the ejection fraction is directed toward the brain (STEAL effect of VGM) [39]. This phenomenon can cause lactic acidosis and may cause also cardiac and systemic ischemia.…”

Section: Postnatal Managementmentioning

confidence: 99%

“…weeks (28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41). Postnatal outcome are depicted in Table 2.…”

mentioning

confidence: 99%

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A systematic review of prenatal diagnosis Vein of Galen aneurysm: prenatal predictive markers and management from the fetal life to childhood

Meglio,

Sica,

Toscano

et al. 2023

Preprint

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Background Vein of Galen malformations (VGMs)account for less than 1% of all intracranial vascular malformations. However in the fetal and pediatric population represent the most common vascular malformation of the brain. An optimal knowledge of the prenatal and postnatal clinical features is mandatory for an effective management. Methods Articles published between 1st January 2003 and 1st January 2023, reported in PUBMED and EMBASE, were evaluated for a systematic review analyzing prenatal features, postnatal features and management of fetal Galean VGMs. Results Twenty-nine papers reporting information on 50 prenatally-diagnosed VGM were included. The most common prenatal features were: fetal hydrocephalus (40%) and cardiomegaly (58%). Post-natal data of 42 VGMs cases are described .The overall mortality was 59,52%. 76,50% of the survivors had a normal development. Conclusions A close follow-up and a multidisciplinary approach is mandatory to manage this condition. The aim of our study was to provide a guide to gynecologist, neonatologist, cardiologist, and neuroradiologist.

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“…In previous studies, the presence of fetal cardiomegaly and ventriculomegaly were associated with poor outcome [3,39,41,42]. Our prenatal data are overlapping with previous studies.…”

Section: Prenatal Managementsupporting

confidence: 86%

Section: Prenatal Managementmentioning

confidence: 99%

Section: Prenatal Managementmentioning

confidence: 99%

Section: Postnatal Managementmentioning

confidence: 99%

“…weeks (28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41). Postnatal outcome are depicted in Table 2.…”

mentioning

confidence: 99%

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A systematic review of prenatal diagnosis Vein of Galen aneurysm: prenatal predictive markers and management from the fetal life to childhood

Meglio,

Sica,

Toscano

et al. 2023

Preprint

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MRI Reference Ranges for Fetal Cardiothoracic Ratio of Diameter, Area, and Circumference from 21 to 38 Weeks Gestational Age

Gao,

Feng,

Cao

et al. 2024

Magnetic Resonance Imaging

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BackgroundWhether fetal cardiothoracic ratio (CTR) is constant or increasing with gestational age (GA) is controversial. The majority of the fetal CTR data has been obtained through ultrasound.PurposeTo retrospectively analyze CTR of diameter, area, and circumference on prenatal MR images in a low‐risk population of singleton pregnancies, and to clarify its diagnostic value.Study TypeRetrospective.Subjects1024 low‐risk singleton pregnancies undergoing MRI.Field StrengthBalanced steady state free precession sequence and single shot‐fast spin echo sequence at 1.5 Tesla.AssessmentPregnancy clinical data were recorded and diameter, area, and circumference of the fetal heart and thorax were measured by two researchers with 6 and 7 years of radiology experience, respectively, and their variation with GA was investigated. The relationship between CTRs with GA was also investigated. Finally, the value of CTR in the diagnosis of fetuses with abnormal development was explored by using receiver operating characteristic (ROC) curves.Statistical TestsLinear regression and ROC curves. A P value <0.05 was considered significant.ResultsThere were significant positive linear correlations (R2 > 0.7, P < 0.0001) between the diameter, area, and circumference of the heart and thorax with GA. The CTRs remain constant values and do not change with GA. The 5th, 50th, and 95th percentiles of the CTR in 21–38 weeks GA were 0.32, 0.39, and 0.48 respectively. The corresponding percentiles for the area ratio were 0.15, 0.21 and 0.27, respectively, and for the circumference ratio were 0.40, 0.46, and 0.52, respectively. Based on ROC curves of CTR with three methods, the area under curves (AUCs) were up to 0.95, the sensitivity and the specificity were more than 88%.Data ConclusionReference ranges of fetal CTR were established using MRI, which remain constant. These may be helpful in making a definitive diagnosis in fetuses with abnormal development.Level of Evidence4.Technical EfficacyStage 2.

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

Cited by 2 publications

References 27 publications

A systematic review of prenatal diagnosis Vein of Galen aneurysm: prenatal predictive markers and management from the fetal life to childhood

A systematic review of prenatal diagnosis Vein of Galen aneurysm: prenatal predictive markers and management from the fetal life to childhood

MRI Reference Ranges for Fetal Cardiothoracic Ratio of Diameter, Area, and Circumference from 21 to 38 Weeks Gestational Age

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