Open Access

ARTICLE

Arrhythmic Risk in Paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

Pietro Paolo Tamborrino^1,2, Corrado Di Mambro^1,2,*, Cecilia Marcolin^1,2, Walter Vignaroli³, Giulia Cafiero⁴, Gianluca Brancaccio³, Sonia Albanese³, Massimo Stefano Silvetti^1,2, Adriano Carotti³, Fabrizio Drago^1,2

1 Paediatric Cardiology and Cardiac Arrhythmia Unit, Department of Paediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
2 European Reference Network for Rare and Low Prevalence Complex Disease of the Heart
3 Paediatric Cardiac Surgery Unit, Department of Paediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy
4 Sport Medicine Unit, Department of Paediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, IRCCS, Rome, Italy

* Corresponding Author: Corrado Di Mambro. Email:

Congenital Heart Disease 2021, 16(1), 85-94. https://doi.org/10.32604/CHD.2021.013038

Received 24 July 2020; Accepted 10 November 2020; Issue published 23 December 2020

Abstract

Introduction: While previous studies only focused on the arrhythmic risk associated with specific correction strategies, this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair. Methods: In this single centre observational cohort study, we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment, patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes. Surgical history and clinical outcomes were reviewed. Results: 86 patients were included in the study (54 male [62.8%], mean age 16.4 ± 6.1 years), with median follow-up from definitive repair of 12.8 years (6.4–18.9 years). They underwent three different final repairs: 23 patients (26.7%) univentricular palliation, 43 (50%) biventricular correction, and 20 (23.3%) one and a half ventricle correction. Thirteen patients (15%) developed arrhythmia: 6 patients (all the subgroups) sinus node disfunction (SND); 2 (biventricular repair) premature ventricular complexes; 2 (one and a half ventricle repair) non-sustained ventricular tachycardia; 1 (biventricular repair) intra-atrial re-entrant tachycardia; 1 (one and a half ventricle repair) supraventricular tachyarrhythmia; 1 (biventricular repair) atrial fibrillation. Three patients with SND needed a pacemaker implantation. Only Fontan circulation showed an association with SND, while the other two groups heterogeneous types of arrhythmias. Conclusions: The low arrhythmic risk is related to surgical repair, it does not appear to be associated with native cardiomyopathy, and it appears to increase with length of follow up. Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

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