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Impact of gastrointestinal comorbidities in patients with right and left atrial isomerism

Published online by Cambridge University Press:  02 September 2021

Anastasia Fotaki Open the ORCID record for Anastasia Fotaki [Opens in a new window] ,
Victoria L. Doughty ,
Winston Banya ,
Stefano Giuliani ,
Sarah Bradley  and
Julene S. Carvalho
Anastasia Fotaki*
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK St. George’s University Hospitals NHS Foundation Trust, St George’s Hospital, London SW17 0QT, UK
Victoria L. Doughty
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK
Winston Banya
Affiliation:
Department of Medical Statistics, Research & Development, Royal Brompton & Harefield NHS Foundation Trust, London SW3 6NP, UK
Stefano Giuliani
Affiliation:
Department of Neonatal & Paediatric Surgery, St. George’s University Hospitals NHS Foundation Trust, London SW17 0QT, UK
Sarah Bradley
Affiliation:
Department of Neonatal & Paediatric Surgery, St. George’s University Hospitals NHS Foundation Trust, London SW17 0QT, UK
Julene S. Carvalho
Affiliation:
Brompton Centre for Fetal Cardiology, Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, London SW3 6NP, UK St. George’s University Hospitals NHS Foundation Trust, St George’s Hospital, London SW17 0QT, UK Cardiovascular Clinical Academic Group, Molecular and Clinical Sciences Research Institute, St. George’s, University of London, St. George’s University Hospitals NHS Foundation Trust, London SW17 0RE, UK
*
Author for correspondence: Dr A. Fotaki, Clinical Fellow in Paediatric and Fetal Cardiology, Brompton Centre for Fetal Cardiology, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. Tel: +44 (20) 7351 8361. E-mail: [email protected]
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Abstract

Background and aim:

Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome.

Methods:

We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention.

Results:

Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001).

Conclusions:

In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.

Keywords

Heart defectscongenitalprenatal diagnosisfetalechocardiography
Type
Original Article
Information
Cardiology in the Young , Volume 32 , Issue 7 , July 2022 , pp. 1053 - 1060
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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