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Abdominal right isomerism with normal intracardiac anatomy in the fetus.

Jowett, V; Bartsota, M; Carvalho, JS (2018) Abdominal right isomerism with normal intracardiac anatomy in the fetus. Cardiol Young, 28 (Supplement 1). S91. ISSN 1047-9511 https://doi.org/10.1017/S1047951118000318
SGUL Authors: Carvalho, Julene

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Abstract

Objectives In the postnatal sequential segmental diagnosis of cardiac abnormalities, it has historically been recognised that atrial situs usually follows that of the abdominal situs. Recognition of abdominal right isomerism (RI) or left isomerism (LI) is often associated with complex congenital heart disease (CHD). However, whilst ‘less complex or minor CHD’ and ‘normal hearts’ have been reported with LI, the same has not been previously reported in the setting of abdominal RI. We had identified a number of such cases in our fetal practice and therefore sought to clarify their features. Methods Patients were identified from the electronic databases in our tertiary fetal cardiac service comprising three fetal cardiology units. Prenatal echo reports and postnatal reports were reviewed. Results Six cases of abdominal RI without complex CHD were identified. Gestational age at diagnosis ranged from 18+0 to 23+1 weeks gestation. Four cases were livebirths; one pregnancy was terminated and one lost to follow up. The stomach was right sided with levocardia and apex to the left in 5 cases and the stomach left sided with dextrocardia and apex to the right in 1 case. The position of the aorta and inferior vena caca (IVC) is summarised in table 1: Table 1: Ultrasound findings of six fetuses with abdominal features of RI Key: R=right, L=left; IVC = inferior vena cava All cases had biventricular atrio-ventricular connections. There was right hand topology in the cases with laevocardia and apex to the left and left hand topology in the case with dextrocardia and apex to the right. Ventriculo-arterial connections were concordant in all cases. In four cases, no cardiac abnormalities were seen. In two cases, abnormalities of systemic venous return were identified: One fetus had a left superior vena cava (SVC) and IVC draining to a dilated coronary sinus; a second fetus had anomalous drainage of the IVC to the left sided atrium, a left SVC draining to the coronary sinus and abnormal drainage of hepatic veins. The presence of a coronary sinus in these 2 cases indicated discordance between abdominal and thoracic situs. One case developed bowel obstruction postnatally secondary to malrotation. Conclusions Abdominal RI can occur with normal intracardiac anatomy. This may be explained in some cases by discordance between abdominal and thoracic situs. Detailed sequential segmental analysis of the fetus is important to identify such cases as these fetuses are at risk of the postnatal complications of isomerism such as gut malrotation, splenic abnormalities and primary ciliary dyskinesia.

Item Type: Article
Additional Information: This article has been published in a revised form in Cardiology in the Young [http://doi.org/10.1017/S1047951118000318]. This version is free to view and download for private research and study only. Not for re-distribution, re-sale or use in derivative works. © Cambridge University Press 2018.
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA)
Journal or Publication Title: Cardiol Young
ISSN: 1047-9511
Dates:
DateEvent
4 May 2018Published
25 January 2018Accepted
Publisher License: Publisher's own licence
URI: https://openaccess.sgul.ac.uk/id/eprint/109621
Publisher's version: https://doi.org/10.1017/S1047951118000318

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