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Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension

Published online by Cambridge University Press:  16 March 2020

Dafni Charisopoulou Open the ORCID record for Dafni Charisopoulou [Opens in a new window] ,
Roberta Margarita Bini ,
Gillian Riley ,
Kalai Janagarajan ,
Shahin Moledina  and
Jan Marek
Dafni Charisopoulou*
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK Department of Pediatrics, Division of Pediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands Academic Centre for Congenital Heart Disease, Netherlands
Roberta Margarita Bini
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK
Gillian Riley
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK
Kalai Janagarajan
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK
Shahin Moledina
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK Institute of Cardiovascular Science, UCL, London, UK
Jan Marek
Affiliation:
Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, UK Institute of Cardiovascular Science, UCL, London, UK
*
Author for correspondence: Dr D. Charisopoulou, Department of Pediatrics, Division of Pediatric Cardiology, Radboud University Medical Center, 6500 HC Nijmegen, Netherlands. Tel: +0044651912675. E-mail: [email protected]
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Abstract

Introduction:

Infants with isolated atrial septal defects are usually minimally symptomatic, and repair is typically performed after infancy. Early repair may be considered if there is high pulmonary blood flow and reduced respiratory reserve or early signs of pulmonary hypertension. Our aim was to review the characteristics and outcomes of a cohort of patients who underwent infant repair at our institute.

Methods:

The study included 56 infants (28 female, 19 trisomy 21) with isolated atrial septal defect (age: 8 months (1.5–12), weight: 6 kg (2.8–7.5), echo Qp/Qs: 1.9 ± 0.1) who underwent surgical closure (20 fenestrated). Three groups were identified: 1) chronic lung disease and pulmonary hypertension (group A: n = 28%); 2) acutely unwell infants with pulmonary hypertension but no chronic lung disease (group B: n = 20, 36%); and 3) infants with refractory congestive heart failure without either pulmonary hypertension or chronic lung disease (group C: n = 9, 16%).

Results:

Post-operatively, pulmonary hypertension infants (47/56) showed improvement in tricuspid annular plane systolic excursion z-score (p < 0.001) and right ventricular systolic/diastolic duration ratio (p < 0.05). All ventilator (14.3%) or oxygen-dependent (31.6%) infants could be weaned within 2 weeks after repair. One year later, weight z-score increased in all patients and by +1 in group A, +1.3 in group B and +2 in group C. Over a median follow-up of 1.4 years, three patients died, four patients continued to have pulmonary hypertension evidence and two remained on targeted pulmonary hypertension therapy.

Conclusion:

Atrial septal defect repair within the first year may improve the clinical status and growth in infants with early signs of pulmonary hypertension or those requiring respiratory support and facilitate respiratory management.

Keywords

Atrial septal defectpulmonary hypertensionchronic lung diseasebronchopulmonary dysplasia of prematurity
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 4 , April 2020 , pp. 511 - 520
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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