Correlation Between Total Repair Timing and Late Aortic Root Dilatation in Repaired Tetralogy of Fallot

Sim, Hyung Tae; Kim, Jeong-Won; Kim, Seong Ho; Park, Su‐Jin; Jang, So-Ick; Lee, Chang Ha

doi:10.1007/s00246-020-02394-z

Cited by 8 publications

(10 citation statements)

References 21 publications

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“…Niwa et al did not reach similar conclusions, probably due to the older age in their study sample at the time of TOF repair compared to that in our study [17]. The findings of this study support the hypothesis that volume overload across the aorta for a longer period of time may contribute to late dilatation and that early correction might have some protective effects against aortic dilatation [25,26].…”

Section: Factors Associated With Proximal Aortic Dilatationcontrasting

confidence: 60%

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study

Mohammed

Frick

Kerst

et al. 2021

JCM

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Aortopathy is a known complication whose incidence is growing within the population of tetralogy of Fallot (TOF) patients. Its pathology and relationship with other comorbidities remain unclear. This study was designed to determine the prevalence and predictors of proximal aortic dilatation after TOF repair. We retrospectively investigated all patients who underwent follow-up cardiac magnetic resonance imaging (CMR; at least 4 years after TOF repair) between March 2004 and December 2019. The dimensions at the ascending aorta (AAo) and sinus of Valsalva (SoV) levels were measured. Aortic dilatation was defined as an internal aortic diameter that was >2 standard deviation of the previously published normal values. We included 77 patients (mean age 28.9 ± 10.5 years, 41.5% female, mean follow-up of 24.5 ± 8.1 years). AAo and SoV were dilated in 19 (24.6%) and 43 (55.8%) patients, respectively. Patients with dilated AAo and SoV were older during the corrective surgery (p < 0.001 and p = 0.004, respectively) and during CMR (p = 0.002 and 0.024, respectively) than patients without AAo and SoV dilatation. Patients of the dilated AAo group were more likely to have prior palliative shunt (p = 0.008), longer shunt duration (p = 0.005), and a higher degree of aortic valve regurgitation (AR) fraction (p < 0.001) and to undergo pulmonary (PVR) and/or aortic valve replacement (p < 0.001 and p = 0.013, respectively). PVR (p = 0.048, odds ratio = 6.413, and 95% CI = 1.013–40.619) and higher AR fraction (p = 0.031, odds ratio = 1.194, and 95% CI = 1.017–1.403) were independent predictors for AAo dilatation. Aortopathy is a common progressive complication that may require reintervention and lifelong follow-up. Our study shows that proximal aortic dilatation may be attributed to factors that increase the volume overload across the proximal aorta, including late corrective surgery and palliative shunt. We also found that PVR and higher AR fraction are independent predictors of AAo dilatation.

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Section: Factors Associated With Proximal Aortic Dilatationcontrasting

confidence: 60%

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study

Mohammed

Frick

Kerst

et al. 2021

JCM

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“…The degree of dilation and risk of aortic dissection in adults with TOF is not well understood, with mixed data on progression after initial repair. 1 , 41 , 42 , 43 , 44 Overall, in large population‐based studies, the risk of aortic dissection in conotruncal defects including TOF is exceedingly rare. 6 , 45 This low incidence makes it difficult to develop evidence‐based guidelines for surveillance or indications for prophylactic repair, which are clear and well‐defined for patients with classic aortopathies.…”

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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“…Although aortic dilation is well-described in TOF, its degree of progression and risk of aortic dissection are less well understood, with mixed data on progressive aortic changes after initial repair 1,[33][34][35][36] . Overall, in large population-based studies, the risk of aortic dissection in conotruncal defects including TOF is exceedingly rare 37,38 .…”

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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Correlation Between Total Repair Timing and Late Aortic Root Dilatation in Repaired Tetralogy of Fallot

Cited by 8 publications

References 21 publications

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study

Proximal Aortic Dilatation and Pulmonary Valve Replacement in Patients with Repaired Tetralogy of Fallot: Is There a Relationship? A Cardiac Magnetic Resonance Imaging Study

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

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