ArticleOpen heart
Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review.
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Who cites it
13 citing papers in PubMed, 31 citations in OpenAlex.
- Molecular characterization of FAM222B as a novel disease gene for dominant cardiovascular laterality defects.Scientific reports · 2026Article
- Risk Factors for Adverse Outcome in Fetuses With Heterotaxy Syndrome and Congenital Heart Disease.Prenatal diagnosis · 2026Article
- Outcomes of Cardiac Repair in Heterotaxy Syndrome Patients-Tertiary Center Experience.CJC pediatric and congenital heart disease · 2026Article
- Cardiac Laterality: Surgical Results of Right Atrial Isomerism.Diseases (Basel, Switzerland) · 2023Article
- A multi-disciplinary, comprehensive approach to management of children with heterotaxy.Orphanet journal of rare diseases · 2022Review
- Utility of Three-Dimensional Printed Model in Biventricular Repair of Complex Congenital Cardiac Defects: Case Report and Review of Literature.Children (Basel, Switzerland) · 2022Review
- Discordant Post-natal Patterns in Fetuses With Heterotaxy Syndrome: A Retrospective Single-Centre Series on Outcome After Fetal Diagnosis.Frontiers in pediatrics · 2022Article
- [Isomorfismo cardiaco: Una perspectiva multidisciplinaria].Archivos de cardiologia de Mexico · 2021Article
- Heterotaxy syndrome with complex congenital heart disease, facial palsy, and asplenia: A rare newborn finding.Clinical case reports · 2021Article
- Heterotaxy polysplenia syndrome in an adult female with complete endocardial cushion defect.Radiology case reports · 2021Article
- Situs Inversus Totalis.Academic forensic pathology · 2018Article
- Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism.Ochsner journal · 2018Article
- Noncompaction cardiomyopathy and heterotaxy syndrome.Progress in pediatric cardiology · 2017Article
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Authors and funding
7 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo determine predictors of poor outcome in patients with heterotaxy syndrome.
methodsA retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV.
resultsThere were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally. 22 patients had right atrial isomerism and 13 had left atrial isomerism. The median age of postnatal diagnosis was 2.5 days old (1 day to 19 months). 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. 5 patients had a biventricular repair and the remaining 30 had a univentricular repair. Type of atrial isomerism, univentricular or biventricular anatomy, severity of atrioventricular valve regurgitation or ventricular dysfunction, obstructed pulmonary venous return, occurrence of arrhythmia and presence of pulmonary atresia did not predict poor outcome. Fetal diagnosis also did not confer a survival advantage. The median duration of follow-up in this cohort was 65 months (2 days to 16.8 years).
conclusionsSurvival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. 34% of patients had a poor outcome. None of the variables studied were predictive of death, transplantation or NYHA classification III or IV.
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