Fetal Double Inlet Left Ventricular Outcomes: Prenatal and Postnatal Diagnostic Concordance

Fetal double-inlet left ventricle (DILV), is a rare anomaly forming, about 1%–2% of the congenital heart defects [1]. In DILV, both atria (right and left) are seen to be connected to the left ventricle. A considerably underdeveloped small right ventricle is usually seen that can be accompanied by a ventricular septal defect, which is called a bulboventricular foramen (BVF) [2]. However, during fetal echocardiography, differential diagnosis of DILV from cardiac anomalies that have single ventricle morphology, such as double inlet right ventricle, double inlet mono ventricle, and tricuspid atresia with ventricular septal defect, is challenging. The most important thing is understanding the morphology of the ventricles during fetal echocardiography. A few studies have been published about the prenatal diagnosis and outcomes of fetal DILV [3-5]. Ocal et al. presented the prenatal and postnatal echocardiographic findings of fetuses with DILV, and also the postnatal outcomes were recorded [3]. This study was held in a referral tertiary center with numerous patients (n: 34 fetuses) over an 8-year period. The prenatal diagnosis of the fetuses was made in a multidisciplinary manner, including maternal-fetal specialists and pediatric cardiologists. Postnatal evaluations of the fetuses were carried out by the same pediatric cardiologists by transthoracic echocardiography. Furthermore, the surgical notes of the newborns were taken into account. Prenatal and postnatal diagnostic concordance was assessed, and 79.4% of the fetuses were confirmed to have DILV. The study has high diagnostic accuracy in fetal DILV anomaly, which has many differential diagnoses. This study highlighted the use of multidimensional assessment during fetal echocardiographic evaluation, such as para-axial and sagittal views. This is the first study in the literature showing the importance of careful evaluation of additional cardiac images for prenatal DILV diagnosis. Therefore, the diagnosis of DILV can be made more easily, and the families can be provided information about the outcome of this rare fetal cardiac anomaly. The only limitation of this study was the retrospective design, and the authors couldn't present the follow-up of all cases that have undergone surgery. A recently published study by Böckenhoff et al. assessed prenatally diagnosed double inlet ventricle cases [4]. In the study, 24 of the fetuses were diagnosed to have DILV. However, this study was held retrospectively in two centers, and no multidisciplinary council was made in order to make the diagnosis. In another study, there were only eight prenatally diagnosed DILV [5]. Although, in that study, the cases were from the same institution and reviewed by the single examiner, and the pre and postnatal care was uniform, the low number of the cases is not enough to depict a result. The purpose of this commentary is to define the importance and context of the original work ‘Navigating the spectrum of DILV presentations: contemporary retrospective cohort study’ and to go further in speculating where the field might be heading in the future. Prenatal diagnosis of DILV has increased with the advanced ultrasonographic modalities. As mentioned, prenatal fetal cardiac anomaly diagnosis can be made more accurately with multidimensional assessment during fetal echocardiographic evaluation, such as para-axial and sagittal views. In this way, parents may have the opportunity to be counseled and discuss this complex, rare fetal cardiac anomaly. The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Journal
Echocardiography
Published
2025-01-01
DOI
https://doi.org/10.1111/echo.70071
Primary Topic
Congenital Heart Disease Studies
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article

Fetal Double Inlet Left Ventricular Outcomes: Prenatal and Postnatal Diagnostic Concordance

Dilek Şahın, Atakan Tanaçan, Duygu Tuğrul Ersak
Echocardiography
Congenital Heart Disease Studies
article

Fetal Double Inlet Left Ventricular Outcomes: Prenatal and Postnatal Diagnostic Concordance

Dilek Şahın, Atakan Tanaçan, Duygu Tuğrul Ersak
article en

Abstract

Fetal double-inlet left ventricle (DILV), is a rare anomaly forming, about 1%–2% of the congenital heart defects [1]. In DILV, both atria (right and left) are seen to be connected to the left ventricle. A considerably underdeveloped small right ventricle is usually seen that can be accompanied by a ventricular septal defect, which is called a bulboventricular foramen (BVF) [2]. However, during fetal echocardiography, differential diagnosis of DILV from cardiac anomalies that have single ventricle morphology, such as double inlet right ventricle, double inlet mono ventricle, and tricuspid atresia with ventricular septal defect, is challenging. The most important thing is understanding the morphology of the ventricles during fetal echocardiography. A few studies have been published about the prenatal diagnosis and outcomes of fetal DILV [3-5]. Ocal et al. presented the prenatal and postnatal echocardiographic findings of fetuses with DILV, and also the postnatal outcomes were recorded [3]. This study was held in a referral tertiary center with numerous patients (n: 34 fetuses) over an 8-year period. The prenatal diagnosis of the fetuses was made in a multidisciplinary manner, including maternal-fetal specialists and pediatric cardiologists. Postnatal evaluations of the fetuses were carried out by the same pediatric cardiologists by transthoracic echocardiography. Furthermore, the surgical notes of the newborns were taken into account. Prenatal and postnatal diagnostic concordance was assessed, and 79.4% of the fetuses were confirmed to have DILV. The study has high diagnostic accuracy in fetal DILV anomaly, which has many differential diagnoses. This study highlighted the use of multidimensional assessment during fetal echocardiographic evaluation, such as para-axial and sagittal views. This is the first study in the literature showing the importance of careful evaluation of additional cardiac images for prenatal DILV diagnosis. Therefore, the diagnosis of DILV can be made more easily, and the families can be provided information about the outcome of this rare fetal cardiac anomaly. The only limitation of this study was the retrospective design, and the authors couldn't present the follow-up of all cases that have undergone surgery. A recently published study by Böckenhoff et al. assessed prenatally diagnosed double inlet ventricle cases [4]. In the study, 24 of the fetuses were diagnosed to have DILV. However, this study was held retrospectively in two centers, and no multidisciplinary council was made in order to make the diagnosis. In another study, there were only eight prenatally diagnosed DILV [5]. Although, in that study, the cases were from the same institution and reviewed by the single examiner, and the pre and postnatal care was uniform, the low number of the cases is not enough to depict a result. The purpose of this commentary is to define the importance and context of the original work ‘Navigating the spectrum of DILV presentations: contemporary retrospective cohort study’ and to go further in speculating where the field might be heading in the future. Prenatal diagnosis of DILV has increased with the advanced ultrasonographic modalities. As mentioned, prenatal fetal cardiac anomaly diagnosis can be made more accurately with multidimensional assessment during fetal echocardiographic evaluation, such as para-axial and sagittal views. In this way, parents may have the opportunity to be counseled and discuss this complex, rare fetal cardiac anomaly. The data that support the findings of this study are available from the corresponding author upon reasonable request.

EchocardiographyVol. 42(1)
Bilkent University (TR)
Openalex Percentile: Top 100%
Congenital Heart Disease Studies
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