Tetralogy of Fallot in the fetus — from diagnosis to delivery. 18-year experience of a tertiary Fetal Cardiology Center

Grzyb, Agnieszka; Koleśnik, Adam; Bokiniec, Renata; Szymkiewicz–Dangel, Joanna

doi:10.33963/kp.a2022.0129

Cited by 5 publications

(3 citation statements)

References 36 publications

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“…This study represents one of the largest single‐center cohorts of fetal TOF to date. 36 Our study sought to investigate a potential developmental cause of aortic dilation in TOF and has 3 notable findings. There were no significant differences in the sums of the great artery dimensions (diameters, circumferences, and cross‐sectional areas) between TOF‐PS and gestational age‐matched controls at the valvar or great artery level.…”

Section: Discussionmentioning

confidence: 99%

Patterns of Aortic Dilation in Tetralogy of Fallot: An Analysis of 100 Fetal Echocardiograms Compared With Matched Controls

Dasgupta,

Kaplinski,

Reddy

et al. 2023

JAHA

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Background Although aortic dilation is common in tetralogy of Fallot (TOF), its progression and risk of dissection are not well understood. The mechanism of dilation is primarily attributed to increased flow in utero; an alternative is unequal septation of the truncus arteriosus resulting in a larger aorta and inherently hypoplastic pulmonary artery (PA). If the latter is true, we hypothesize the aorta to PA ratio in TOF is stable throughout gestation, and sums of great artery dimensions are similar to controls. Methods and Results We performed a single‐center retrospective study of fetuses with TOF (2014–2020) and matched controls. We compared sums of diameters, circumferences, and cross‐sectional areas of the aorta and PA and evaluated the aorta to PA ratio across gestation in 2 TOF subtypes: pulmonary stenosis and atresia (TOF‐PA). There were 100 echocardiograms with TOF (36% TOF‐PA) with median gestational age of 31 weeks (interquartile range 26.5–34.4) and median maternal age of 34 years (interquartile range 30–37). There were no differences in sums of great artery dimensions between TOF‐pulmonary stenosis and controls. In TOF‐PA, sums were significantly lower than controls ( P values <0.01). The aorta to PA ratio was stable throughout gestation (Pearson's r =0.08 [95% CI, −0.12 to 0.27], −0.06 [95% CI, −0.25 to 0.14]). Conclusions The aorta in fetal TOF is large but grows proportionally throughout gestation, with sums of great artery dimensions similar to controls. TOF‐PA appears distinct from TOF‐pulmonary stenosis (with smaller sums), warranting further investigation. In conclusion, our findings suggest an intrinsic developmental mechanism contributes to aortic dilation in TOF.

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Section: Discussionmentioning

confidence: 99%

Patterns of Aortic Dilation in Tetralogy of Fallot: An Analysis of 100 Fetal Echocardiograms Compared With Matched Controls

Dasgupta,

Kaplinski,

Reddy

et al. 2023

JAHA

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“…This study represents one of the largest single-center cohorts of fetal TOF to date 32 . Our study sought to investigate a potential developmental etiology of aortic dilation in TOF and has three notable findings.…”

Section: Discussionmentioning

confidence: 99%

Thinking Outside the “Flow-Mediated” Box: An Analysis of Aortic Dilation in 100 Fetuses with Tetralogy of Fallot Compared to Matched Controls

Dasgupta¹,

Kaplinski²,

Reddy³

et al. 2023

Preprint

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Background: Aortic dilation in tetralogy of Fallot (TOF) is primarily attributed to increased aortic flow in utero. An alternative hypothesis is abnormal neural crest cell migration, with unequal septation of the truncus arteriosus resulting in a larger aorta and inherently hypoplastic pulmonary artery (PA). If so, we hypothesize the aorta to PA ratio (Ao:PA) in TOF is stable throughout gestation, and the total sum of dimensions of the great arteries is similar to controls. Methods: We performed a single-center retrospective study of all fetuses with TOF (2014-2020) and gestational age-matched controls. We compared sums of diameters, circumferences, and cross-sectional areas of the aorta and PA and evaluated the Ao:PA across gestation in TOF with pulmonary stenosis (TOF-PS) and atresia (TOF-PA). We analyzed data with two-tailed t-tests and Pearson's correlation. Results: There were 100 fetuses with TOF (36% TOF-PA) with median gestational age of 31 weeks [IQR 26.5, 34.4] and median maternal age of 34 years [IQR 30, 37]. There were no differences in sums of great artery dimensions between TOF-PS and controls. In TOF-PA, sums were significantly lower than controls. The Ao:PA was stable throughout gestation. Conclusions: The aorta in fetal TOF is large but grows proportionally throughout gestation, with a sum of great artery dimensions similar to controls. TOF-PA appears to be distinct from TOF-PS (with overall smaller dimensions), and is a group that warrants further investigation. In conclusion, our findings do not support the flow-mediated model of aortic dilation in TOF, and instead suggest an intrinsic developmental mechanism.

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“…The prevalence in general population is around 0.03% [3]. Recently Grzyb et al [4] presented a very insightful analysis of the largest single-center cohort of 326 ToF fetuses.…”

Section: Introductionmentioning

confidence: 99%

Tetralogy of Fallot and bicuspid aortic valve: Rare coexistence

et al. 2022

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Tetralogy of Fallot in the fetus — from diagnosis to delivery. 18-year experience of a tertiary Fetal Cardiology Center

Cited by 5 publications

References 36 publications

Patterns of Aortic Dilation in Tetralogy of Fallot: An Analysis of 100 Fetal Echocardiograms Compared With Matched Controls

Patterns of Aortic Dilation in Tetralogy of Fallot: An Analysis of 100 Fetal Echocardiograms Compared With Matched Controls

Thinking Outside the “Flow-Mediated” Box: An Analysis of Aortic Dilation in 100 Fetuses with Tetralogy of Fallot Compared to Matched Controls

Tetralogy of Fallot and bicuspid aortic valve: Rare coexistence

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