Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome

Berg, Christoph; Lachmann, Robert; Kaiser, Christina; Kozlowski, P.; Stressig, R; Schneider, Markus; Asfour, B; Herberg, Ulrike; Breuer, Johannes; Gembruch, Ulrich; Geipel, A.

doi:10.1002/uog.7499

Cited by 33 publications

(24 citation statements)

References 20 publications

(32 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…While in PSAIVS defect, the degree of RVH is determined by the size and type of the ventricular septal defect and is therefore predictable to some extent even in the intrauterine period [14][15][16][17][18][19][20][21][22][23], the likeliness of a univentricular (UV), or biventricular (BV) postnatal circulation in severe PSAIVS is much more difficult to predict. Postnatal series showed that RV size, TV size, and the coronary morphology are the most important determinants of UV or BV outcome [3,9,[24][25][26][27][28].…”

Section: Introductionmentioning

confidence: 99%

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

et al. 2019

View full text Add to dashboard Cite

Purpose: To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS). Methods: All cases of PSAIVS diagnosed prenatally over a period of 14 years were retrospectively collected in 2 tertiary referral centers. Results: Forty-nine fetuses with PSIVS (n = 11) or PAIVS (n = 38) were identified prenatally. Nineteen (38.8%) fetuses had additional ventriculocoronary connections (VCCs) and 21 (42.9%) fetuses had right ventricular hypoplasia. Four (8.2%) pregnancies were terminated, 2 (4.1%) ended in intrauterine fetal death, 4 (8.2%) in neonatal death, and 5 (10.2%) children died in infancy or childhood, including one case with compassionate care. Thirty-four of 44 (77.3%) fetuses with the intention-to-treat were alive at latest follow-up, 25 (73.5%) with BV, and 9 (26.5%) with UV circulation. Most significant predictive markers of UV circulation were Vmax of tricuspid regurgitation (TR) < 2 m/s, right ventricle/left ventricle length ratio ≤0.6, and presence of VCC. A scoring system including these 3 markers had 100% sensitivity and 100% specificity predicting an UV outcome if more than one of these criteria was fulfilled. All 25 liveborn infants that were suitable for BV repair survived, whereas only 9 out of 14 candidates for UV repair survived. None of the 14 fetuses with predicted UV outcome would have met the inclusion criteria for fetal intervention, as 10 of them had VCC and the remaining 4 had absent TR or Vmax < 2 m/s. Conclusion: The prognosis of prenatally diagnosed PSAIVS is good if BV circulation can be achieved, while postnatal mortality in UV circulation is high within the first 4 months of life. Postnatal outcome can be predicted prenatally with high accuracy using a simple scoring system. This information is mandatory for parental counseling and may be useful in selecting fetuses for intrauterine valvuloplasty.

show abstract

Section: Introductionmentioning

confidence: 99%

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

et al. 2019

View full text Add to dashboard Cite

show abstract

“…L'atrésie tricuspide est une anomalie isolée dans 75 à 80 % des cas [106,109]. Une ou plusieurs autres anomalies cardiovasculaires sont présentes chez 4 à 18 % des foetus [106,1075].…”

Section: Anomalies Associéesunclassified

“…Une ou plusieurs autres anomalies cardiovasculaires sont présentes chez 4 à 18 % des foetus [106,1075]. En dehors des anomalies aortiques ou pulmo naires évoquées ci-dessus, il s'agit essentiellement de la persistance d'une veine cave supérieure gauche, d'une crosse aortique droite, d'une juxtaposition des auricules ou d'un retour veineux pulmonaire anormal total [109]. Enfin, dans 10 à 15 % des cas coexistent des malformations extracardiaques importantes : association VACTERL, agénésie du ductus venosus, anomalies urogénitales, etc.…”

Section: Anomalies Associéesunclassified

“…En dehors des anomalies aortiques ou pulmo naires évoquées ci-dessus, il s'agit essentiellement de la persistance d'une veine cave supérieure gauche, d'une crosse aortique droite, d'une juxtaposition des auricules ou d'un retour veineux pulmonaire anormal total [109]. Une anomalie chromosomique semble rare, moins de 10 % des cas, et non spécifique dans les formes d'atrésie tricuspide non compliquées (trisomie 13 ou 18, tétrasomie p22q11 partielle [109]). Une anomalie chromosomique semble rare, moins de 10 % des cas, et non spécifique dans les formes d'atrésie tricuspide non compliquées (trisomie 13 ou 18, tétrasomie p22q11 partielle [109]).…”

Section: Anomalies Associéesunclassified

“…• un caractère restrictif du foramen ovale et/ou de la CIV ; • l'aggravation de l'obstruction de la voie artérielle droite, phénomène fréquent et pouvant n'apparaître qu'en toute fin de grossesse [109]. …”

Section: Avant La Naissanceunclassified

See 2 more Smart Citations

Malformations cardiaques

2013

Echocardiographie Fœtale

View full text Add to dashboard Cite

Anatomical and physiological diagnostic discrepancies in fetuses with single‐ventricle congenital heart disease in a contemporary cohort

Patel,

Kreeger,

Sachdeva

et al. 2024

Ultrasound in Obstet & Gyne

View full text Add to dashboard Cite

ObjectiveImage quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE specifically in single ventricle congenital heart disease (SV). Our study aims to assess the ability of FE to correctly predict postnatal anatomy and physiology in SV in a contemporary cohort.MethodsThe contemporary, clinical reports of FE with SV performed from 7/2017 to 7/2021 were compared with postnatal echocardiograms in a formal quality assurance program. SV were grouped by anatomic subtype. Diagnostic errors were designated as major if the error would have resulted in significant alteration in fetal counseling or postnatal management. Remaining errors were classified as minor. Physiologic discrepancies, including prostaglandin dependency (PGE‐D), atrioventricular valve regurgitation (AVVR), pulmonary venous obstruction, and atrial septal restriction were assessed by review of postnatal course.ResultsA total of 119 subjects were analyzed. SV subtypes in the cohort included hypoplastic left heart syndrome (HLHS) (n=68), tricuspid atresia (n=16), double inlet left ventricle (n=12), unbalanced atrioventricular canal (UAVC) (n=11), heterotaxy (n=9), and other (n=3). The rate of major anatomic and physiologic errors was low (n=6, 5.0%). A higher proportion of minor errors were noted in HLHS and tricuspid atresia but the differences were not statistically significant. Physiologic discrepancies were uncommon, with three major discrepancies including underestimation of degree of venous obstruction in one non‐HLHS fetus with total anomalous pulmonary venous return, overestimation of atrial septal restriction in one HLHS fetus, and incorrect prediction of PGE‐D with 1 false‐negative for pulmonary blood flow. No discrepancy in degree of AVVR or atrial septal restriction affected postnatal care. Minor physiologic discrepancies included two false‐positive predictions of PGE‐D with 1 false‐positive for ductal‐dependent systemic flow, and 1 false‐positive for pulmonary blood flow.ConclusionsIn this contemporary review of FE in SV at our center, there was high accuracy in describing anatomic and physiologic findings in fetuses with SV. Major physiologic discrepancies were uncommon but included important cases of false‐negative prediction of PGE‐dependence and underestimation of obstruction of total anomalous pulmonary venous return. These data can both inform more accurate counseling of families with SV fetuses and guide diagnostic improvement efforts.This article is protected by copyright. All rights reserved.

show abstract

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome

Abstract: Objective To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus. Methods This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany. Results Fifty

Cited by 33 publications

References 20 publications

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History

Malformations cardiaques

Anatomical and physiological diagnostic discrepancies in fetuses with single‐ventricle congenital heart disease in a contemporary cohort

Contact Info

Product

Resources

About