Key result
Surgical repair for pulmonary atresia with intact ventricular septum was associated with a 15% incidence of arrhythmias over a median follow-up of 12.8 years, with risk varying by the type of surgical repair.
Why the study?
Previous studies focused only on arrhythmic risk associated with specific correction strategies, leaving the risk across all PA-IVS phenotypes after surgical repair not well evaluated.
Does the type of surgical repair affect the risk of arrhythmia in pediatric patients with pulmonary atresia with intact ventricular septum?
Cohort (n=86)
No
Does the type of surgical repair affect the risk of arrhythmia in pediatric patients with pulmonary atresia with intact ventricular septum?
In pediatric patients with PA-IVS, the overall risk of arrhythmia after surgical repair is relatively low (15% at 12.8 years), but varies by surgical strategy and appears highest following one and a half ventricle repair.
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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Tamborrino et al. (2020) conducted a cohort in Pulmonary atresia with intact ventricular septum (n=86). Surgical repair for pulmonary atresia with intact ventricular septum was evaluated on Development of arrhythmia. Surgical repair for pulmonary atresia with intact ventricular septum was associated with a 15% incidence of arrhythmias over a median follow-up of 12.8 years, with risk varying by the type of surgical repair.
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