Extracardiac anomalies in prenatally diagnosed heterotaxy syndrome

Gottschalk, Ingo; Stressig, R.; Ritgen, J; Herberg, Ulrike; Breuer, Johannes; Vorndamme, A.; Strizek, Brigitte; Willruth, A.; Geipel, A.; Gembruch, Ulrich; Berg, Christoph

doi:10.1002/uog.14871

Cited by 33 publications

(59 citation statements)

References 25 publications

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“…A total of 2037 articles were identified from the electronic search, of which 134 were assessed with respect to their eligibility for inclusion (Table S2) and a total of 16 studies were eventually included in the systematic review ( Figure 1, Table 1) 12,14,[25][26][27][28][29][30][31][32][33][34][35][36][37][38] . These 16 studies included 647 fetuses with a prenatal diagnosis of isomerism, of which LAI was confirmed at birth in 61.7% (95% CI, 57.8-65.4%) and RAI in 38.3% (95% CI, 34.6-42.2%).…”

Section: Resultsmentioning

confidence: 99%

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta‐analysis

Buca

Khalil

Rizzo

et al. 2018

Ultrasound in Obstet & Gyne

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Fetal heterotaxy is associated with a high prevalence of cardiac and extracardiac anomalies. Approximately one quarter of fetuses with heterotaxy died during or after surgery. Abnormal heart rhythm, especially heart block, is common in fetuses with LAI, while this finding is uncommon in RAI. Biventricular repair was common in LAI while univentricular repair was required in the majority of children affected by RAI. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

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Section: Resultsmentioning

confidence: 99%

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta‐analysis

Buca

Khalil

Rizzo

et al. 2018

Ultrasound in Obstet & Gyne

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“…Assessment of lung lobulation patterns is difficult in the absence of effusion in the pleural space. Liver, stomach, and gall bladder may be in either position (the solitus or inversus) and viserocardiac ambiguity is not a consistent feature . In contrast, cardiovascular abnormalities are more prevalent in antenatally detected cases …”

Section: Discussionmentioning

confidence: 99%

“…Among extracardiac defects, gut malrotation and biliary atresia are the most common anomalies, reported in about 40–50% of cases . Gottschalk et al reported biliary atresia as the most important extracardiac contributing factor to the postnatal prognosis. In our case, the nonvisualization of the gallbladder could suggest biliary atresia.…”

Section: Discussionmentioning

confidence: 99%

“…Liver, stomach, and gall bladder may be in either position (the solitus or inversus) and viserocardiac ambiguity is not a consistent feature. 1,4 In contrast, cardiovascular abnormalities are more prevalent in antenatally detected cases. 1,3,5 Berg et al 1 presented the following criteria for in utero detection of heterotaxy syndromes.…”

Section: Discussionmentioning

confidence: 99%

“…Interruption of the inferior vena cava (IVC) with azygos continuation to the superior vena cava (SVC) is a hallmark finding of left isomerism and was reported in 85%-93% of cases. [2][3][4] About 35% of patients also have bilateral SVC. 3,4 We report a case of a prenatally detected left atrial isomerism at second trimester with interrupted IVC and hemiazygos continuation draining into the right atrium via the persistent left SVC and coronary sinus.…”

Section: Introductionmentioning

confidence: 99%

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A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava

Moradi

Moosavi

Kazemi

et al. 2017

J of Clinical Ultrasound

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Heterotaxy syndromes are defined as the disorders that involve abnormal arrangement of viscera. We present a case of prenatally diagnosed left isomerism in a 30-year-old primigravida woman referred to our hospital for complex cardiac abnormality. Sonographic findings included heart block, unbalanced atrioventricular septal defect, interruption of the inferior vena cava with hemiazygos continuation, double superior vena cava, a right-sided stomach, and biliary atresia. The hemiazygos vein drained into the right atrium by the persistent left superior vena cava. This type of inferior vena cava interruption and continuation is rarely reported in prenatally detected cases of left isomerism. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:430-433, 2017.

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Comorbidities in situs inversus totalis: A hospital‐based study

Chen

Guo

Qian

et al. 2020

Birth Defects Research

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Background and objective Few studies have assessed the comorbid diseases in situs inversus totalis (SIT) comprehensively. The aim of this study was to provide insight into the spectrum and prevalence of comorbidities in SIT. Methods Children ≤18 years of age with SIT were enrolled in this retrospective observational study. Situs status and comorbidities were independently confirmed by two physicians, based on review of radiologic, ultrasonic examination, operative records, and case notes. Results A total of 155 children (median age: 1.24 years; range: 1 day–17.8 years) confirmed to have SIT were recruited between January 2008 and December 2018. Associated conditions were diagnosed in 114 children (73.5%). Among them, 25 children (16.1%) had multiple anomalies affecting two or more organ systems. The most commonly associated conditions were congenital heart defects (n = 72, 46.5%) followed by primary ciliary dyskinesia (n = 19, 12.3%), renal disorders (n = 12, 7.7%), biliary atresia (n = 7, 4.5%), skeletal dysplasia (n = 8, 5.2%), and mental retardation (n = 4, 2.6%). Conclusion A substantial proportion of children with SIT have comorbidities affecting multiple systems, especially cardiovascular and respiratory abnormalities. Children with SIT warrant careful examination for the presence of congenital and acquired abnormalities.

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Extracardiac anomalies in prenatally diagnosed heterotaxy syndrome

Abstract: Objective To assess the incidence and impact of extracardiac anomalies on the prognosis of fetuses with heterotaxy syndrome.Methods All fetuses diagnosed with heterotaxy syndrome by three experienced examiners over a period of 14 years (1999-2013)

Cited by 33 publications

References 25 publications

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta‐analysis

Outcome of prenatally diagnosed fetal heterotaxy: systematic review and meta‐analysis

A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava

Comorbidities in situs inversus totalis: A hospital‐based study

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