ArticleThe American journal of cardiology2014
Perinatal and infant outcomes of prenatal diagnosis of heterotaxy syndrome (asplenia and polysplenia).
Article in The American journal of cardiology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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18 citing papers in PubMed, 61 citations in OpenAlex.
- Risk Factors for Adverse Outcome in Fetuses With Heterotaxy Syndrome and Congenital Heart Disease.Prenatal diagnosis · 2026Article
- Article
- Acute splenic torsion in an adolescent with polysplenia syndrome: case report.Italian journal of pediatrics · 2025Article
- Genomic rare variant mechanisms for congenital cardiac laterality defect: A digenic model approach.American journal of human genetics · 2025Article
- Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome.JTCVS open · 2024Article
- Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia.International journal of cardiology. Congenital heart disease · 2023Article
- Congenitally Corrected Transposition of the Great Arteries: Fetal Diagnosis, Associations, and Postnatal Outcome: A Fetal Heart Society Research Collaborative Study.Journal of the American Heart Association · 2023Article
- Anatomy, embryology, and imaging of situs ambiguous with polysplenia and left IVC.Radiology case reports · 2023Article
- LOF variants identifying candidate genes of laterality defects patients with congenital heart disease.PLoS genetics · 2022Article
- A multi-disciplinary, comprehensive approach to management of children with heterotaxy.Orphanet journal of rare diseases · 2022Review
- Discordant Post-natal Patterns in Fetuses With Heterotaxy Syndrome: A Retrospective Single-Centre Series on Outcome After Fetal Diagnosis.Frontiers in pediatrics · 2022Article
- Characterization of phenotypic spectrum of fetal heterotaxy syndrome by combining ultrasound and magnetic resonance imaging.Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology · 2021Article
- Article
- NovelFrontiers in medicine · 2021Article
- Polysplenia syndrome with complex heart disease and jejunal atresia with malrotation in neonate: A case report.Clinical case reports · 2020Article
- Genetic architecture of laterality defects revealed by whole exome sequencing.European journal of human genetics : EJHG · 2019Article
- Fetal Situs, Isomerism, Heterotaxy Syndrome: Diagnostic Evaluation and Implication for Postnatal Management.Current treatment options in cardiovascular medicine · 2016Review
- Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review.Open heartArticle
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Authors and funding
10 authors at 3 institutions in 2 countries.
Funding
Abstract
Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011. Of 154 fetuses with HS, 61 (40%) had asplenia syndrome (ASP) and 93 (60%) had polysplenia syndrome (PSP). In the ASP group, 22 (36%) patients were elected for termination of pregnancy, 4 (10%) had fetal death, and 35 of 39 (90%) continued pregnancies were live born. In the PSP group, 12 (13%) patients were elected for termination of pregnancy, 5 (6%) had fetal death (4 with bradyarrhythmia), and 76 of 81 (94%) continued pregnancies were live born. Bradyarrhythmia was the only predictor of fetal death. In the live-born ASP group, 43% (15 of 35) died, 7 because of pulmonary vein stenosis, 4 postoperatively, and 4 because of noncardiac causes. In the live-born PSP group, 13% (10 of 76) died, 5 postoperatively, 2 from bradyarrhythmia, 1 from a cardiac event, and 2 from noncardiac causes. Pulmonary vein stenosis and noncardiac anomalies were independent risk factors for postnatal death. Only 8% of ASP patients achieved biventricular circulation, compared with 65% of PSP patients. In the live-born cohort, the 5-year survival rate was 53% for ASP and 86% for PSP. In conclusion, most PSP patients are currently alive with biventricular circulation in contrast with few ASP patients. Bradyarrhythmia was the only predictor of fetal death. Pulmonary vein stenosis and noncardiac anomalies were predictors of postnatal death.
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