The prognosis of common arterial trunk from a fetal perspective: A prenatal cohort study and systematic literature review

Nisselrooij, Amber E L van; Herling, Lotta; Clur, Sally‐Ann B.; Linskens, Ingeborg H.; Pajkrt, Eva; Rammeloo, Lukas A. J.; Harkel, Arend D J Ten; Hazekamp, Mark G.; Blom, Nico A.; Haak, Monique C.

doi:10.1002/pd.5907

Cited by 8 publications

(9 citation statements)

References 39 publications

(101 reference statements)

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“…However, with advances in fetal echocardiography, there is an increasing frequency of inutero diagnosis of TA. [1][2][3][4] Prenatal diagnosis offers the bene ts of providing su cient time for parental counseling, making informed family planning decisions, completing multi-disciplinary delivery planning, and choosing an appropriate delivery location. Although TA outcomes after neonatal surgical repair have been well described with improved survival in the current era [1,[5][6][7], the fetal and neonatal outcomes prior to the surgical repair are not well characterized.…”

Section: Introductionmentioning

confidence: 99%

“…[1,2,8] A pre-surgical mortality rate higher than what would be expected in other congenital heart disease (CHD) has been suggested previously in small cohorts. [3,9] Despite this, due to the lack of data, prognostication and prenatal counseling of this rare CHD are often based on outcomes data following neonatal surgical repair. [1,5,[10][11][12][13] Moreover, previously published studies have reported up to 47% elective termination frequency.…”

Section: Introductionmentioning

confidence: 99%

“…[1,5,[10][11][12][13] Moreover, previously published studies have reported up to 47% elective termination frequency. [1][2][3] The scarcity of studies focusing on fetal echocardiographic (fetal-echo) predictors of perinatal pre-surgical mortality makes prenatal counseling somewhat speculative, potentially contributing to this high termination rate.…”

Section: Introductionmentioning

confidence: 99%

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Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Cox

Husain

Geiger³

et al. 2022

Preprint

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Background: Truncus arteriosus (TA) is a rare congenital heart defect that can be prenatally detected by fetal echocardiography. However, prognostication and prenatal counseling focus primarily on surgical outcomes due to limited fetal and neonatal pre-surgical mortality data. We aimed to describe the incidence and identify predictors of perinatal pre-surgical mortality in prenatally detected TA. Methods: This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was perinatal mortality, defined by fetal or neonatal pre-surgical death or transplantation prior to discharge. Univariable regression modeling and Chi-square test assessed for associations between prenatal clinical, demographic, and fetal-echo variables and perinatal mortality. Results: Of 23 pregnancies with prenatal diagnosis of TA, 4(17%) were terminated. Of the remaining 19, perinatal mortality occurred in 4(26%), including 2(11%) fetal deaths and 2(11%) neonatal pre-surgical deaths. No transplantation listings. Of liveborn fetuses(n=17), 15(88%) underwent a neonatal surgery, and 1(6%) required ECMO. As compared to the survivors, the perinatal mortality group had a higher likelihood of having left ventricular dysfunction (0%vs.40%;p=0.01), right ventricular dysfunction (0%vs.60%;p=0.002), cardiovascular profile score(CVP)<7(0%vs.40%;p=0.01), skin edema (0%vs.40%;p=0.01), and abnormal umbilical venous(UV) Doppler (60%vs.0%;p=0.002). Presence of truncal valve regurgitation or stenosis neared significance. Conclusion: In prenatally diagnosed TA, there is significant perinatal mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal echocardiographic variables associated with perinatal mortality include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for perinatal mortality may guide parental counseling and guide postnatal planning in prenatally diagnosed TA.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Cox

Husain

Geiger³

et al. 2022

Preprint

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“…Common arterial trunk (CAT) is a rare congenital heart defect (CHD), accounting for about 1% of CHD foetuses. 1 CAT represents an early stage of the development of primitive arterial septum during embryonic development. Therefore, only one large artery leading from the bottom of the embryonic heart is preserved, and the ventricular blood is pumped into systemic circulation, pulmonary circulation and coronary circulation through a set of semilunar valves' instead.…”

Section: Introductionmentioning

confidence: 99%

Clinical value of ultrasound in diagnosis and classification of common arterial trunk

et al. 2022

Preprint

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Objectives: This study aims to explore the clinical value of prenatal ultrasonography in the diagnosis of the common arterial trunk (CAT) classification and associated deformities. Materials and methods: The two-dimensional ultrasound images spatiotemporal image correlations (STICs) and clinical data of 108 foetuses diagnosed with CAT malformations were retrospectively analysed, their CAT types were classified, and the correlations between these different types and foetal malformations and pregnancy outcomes were analysed. Results: Among the 108 foetuses, there were 47 cases (43.5%) of type A1, 51 cases (47.3%) of type A2, 9 cases (8.3%) of type A3 and 1 case of type A4 (0.9%), of which 22 cases (20.4%) were isolated CAT, 56 cases (51.8%) had complex intra-cardiac structural abnormalities, 30 cases (27.8%) had intra-cardiac or extra-cardiac structural abnormalities, 17 cases had 1 other system abnormality, and 5 cases had 2 other system abnormalities. Four cases were combined with 3 other system abnormalities, while 4 cases were combined with 4 other system abnormalities, among which the facial and physical abnormalities had the highest incidence (40.0%). The STIC images were completely displayed in 88 cases (81.5%). Isolated CAT and CAT combined with other malformations were significantly correlated with foetal pregnancy outcomes ( P < 0.05). Conclusions: Prenatal ultrasonography had a high clinical application value for classifying CAT. Pregnancy outcomes were highly correlated with the classification and combined intra-cardiac and extra-cardiac structural malformations. The early assessment of foetal prognosis before birth has great value for clinical intervention.

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“…Regarding CAT's prognosis, its intrauterine course is relatively stable except for cases with a severe truncal valve insufficiency that can lead to fetal hydrops and intrauterine death [6]. Despite the great advances in neonatal cardiac surgery, CAT still has significant mortality with rates varying from 3 to 30% [7,8] and mostly depending on the perioperative situation as well as on the presence of associated anomalies. Some prenatal ultrasound markers have been proposed to identify fetuses at a higher risk of perinatal mortality such as truncal valve functional anomalies (mostly regurgitation but also stenosis), the presence of an IAA, or coronary anomalies [9].…”

Section: Introductionmentioning

confidence: 99%

Common Arterial Trunk in Fetal Life: Accuracy of Diagnosis and Prediction of Outcome

Villalaín¹,

Gómez-Montes²,

Felipe³

et al. 2022

Fetal Diagn Ther

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Introduction Common arterial trunk (CAT) is a congenital heart disease with significant perinatal mortality in which diagnostic agreement remains low. Methods Retrospective cohort study on fetuses with suspected CAT. Diagnostic accuracy was tested considering gold-standard postnatal ultrasound or necropsy. Prenatal sonographic markers were evaluated by logistic regression for perinatal survival. Results There were 79 fetuses with suspected CAT, and 55 cases had available necropsy/postnatal ultrasound conforming the study population. The diagnostic accuracy was 90.9% for CAT and 83.6% at a subtype level. In those with a confirmed diagnosis and intention-to-treat (n=28) composite early-mortality (intrauterine, pre-surgical and early-surgery) was of 25%, 10.5% in isolated-cases. The OR for early-mortality of isolated-cases was 0.13 [95% confidence interval (CI) 0.03-0.75]. After adjusting for isolated-cases, the only prognostic prenatal markers of perinatal mortality were a dysplastic truncal valve OR 7.78 (95% CI 1.23-49.13) and a stenotic flow OR 8.48 (95%CI 1.40-51.10) Conclusion CAT is a condition that if evaluated by experts, can be diagnosed with a high degree of accuracy. It remains an entity with high perinatal mortality. Its most important prognostic factor is its association with other anomalies. In isolated cases, the presence of a dysplastic truncal valve and a stenotic flow increase the chances of perinatal death.

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The prognosis of common arterial trunk from a fetal perspective: A prenatal cohort study and systematic literature review

Cited by 8 publications

References 39 publications

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Clinical value of ultrasound in diagnosis and classification of common arterial trunk

Common Arterial Trunk in Fetal Life: Accuracy of Diagnosis and Prediction of Outcome

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