Evidence map›Paper›PMID 39712988›Full record

ArticleInternational journal of cardiology. Congenital heart disease2023

Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia.

Bradley MacDonald, Zoe Vetten, James Ramsay, David Andrews, Deane Yim

Abstract read
In one paragraph

Article in International journal of cardiology. Congenital heart disease, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors.

Bradley MacDonaldChildren's Cardiac Centre, Perth Children's Hospital, Perth, Western Australia, Australia.
Zoe VettenDepartment of Paediatric Cardiology, Starship Hospital, Auckland, New Zealand.
James RamsayChildren's Cardiac Centre, Perth Children's Hospital, Perth, Western Australia, Australia.
David AndrewsChildren's Cardiac Centre, Perth Children's Hospital, Perth, Western Australia, Australia.
Deane YimChildren's Cardiac Centre, Perth Children's Hospital, Perth, Western Australia, Australia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Heterotaxy syndromes encompass left and right atrial isomerism (LAI and RAI respectively) and are associated with variable cardiac and non-cardiac anomalies which greatly influence outcomes. RAI is usually associated with complex congenital heart disease (CHD), early surgical intervention and increased mortality. LAI is less commonly associated with complex CHD but can be associated with heart block. The objective of this study was to review the clinical features and outcomes of patients with heterotaxy syndromes in Western Australia (WA). Methods: A retrospective review was performed of live born patients diagnosed with heterotaxy from 2003 to 2022 in a statewide tertiary cardiac service, representing all cases in WA with a view to compare the outcomes between LAI and RAI at our centre. Results: 30 patients (53% male) were diagnosed with heterotaxy; 16 (53%) with LAI and 14 (47%) with RAI. Overall incidence was 0.48 per 10,000 live births over the defined period. RAI patients were significantly more likely to have an antenatal diagnosis (81.8% versus LAI 28.6%, p = 0.03). Overall, 5-year survival was 56% for RAI and 87% for LAI. No deaths occurred after the first 12 months of life with a median follow-up of 65 months (IQR 114.8). RAI was associated with asplenia (91%), atrioventricular septal defect (91%) and a functionally univentricular circulation (71%). LAI was associated with polysplenia (100%) and complete heart block in 3 patients (19%). Surgical pathways included repair of anomalous pulmonary venous return (45%), Blalock Taussig shunt (60%), bidirectional cavopulmonary connection (50%) and Fontan completion (30%). Conclusions: Patients with RAI suffer high mortality and early surgical intervention, with few making it to Fontan completion. By comparison patients with LAI have less morbidity and mortality. The management of heterotaxy continues to be challenging due to widely associated cardiac and extracardiac manifestations.

Indexed as

AspleniaHeterotaxyIsomerismLeft atrial isomerismPolyspleniaRight atrial isomerism

Identifiers

PMID39712988
PMCPMC11658138

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.