The Australian Experience With PEARS in the Paediatric Population

Abstract

Background: Personalised external aortic root support (PEARS) procedure is an innovative surgical alternative to reinforce the aortic root and prevent further dilatation, dissection, and rupture. There have been >750 implants in adult patients worldwide. However, PEARS can also be used in paediatric patients with congenital aortic root aneurysms and native or neo-aortic dilatation after repaired congenital heart disease. The aim of this study was to evaluate the outcomes of PEARS in the paediatric population from 3 paediatric centres in Australia. Method: The medical records of patients <18 years who underwent the PEARS procedure at Queensland Children’s Hospital, Brisbane, Royal Children’s Hospital, Melbourne, and Perth Children’s Hospital, Perth, were retrospectively reviewed. The PEARS prosthesis was implanted when the dimensions of the aortic root approached z-score cutoffs or adult sizes. Results: Since 2019, 14 paediatric patients underwent a PEARS procedure. The median age, weight and height was 13.5 (IQR 12.7–16.1) years, 58.1 (33.7–81.2) kg and 162.2 (148.9–184.3) cm, respectively. Indications were Marfan syndrome (n=10), Ehlers-Danlos syndrome (n=2), neo-aortic aneurysmal dilatation of after an arterial switch (n=1) and after a Ross procedure at age 11 years (n=1). There was no mortality or coronary injury. No patient required on table conversion to aortic root replacement. Cardiopulmonary bypass was used in 3 patients (all redo sternotomies). There was 1 perioperative stroke. Median duration of follow-up was 21.4 (15.9–23.5) months. 1 patient with preoperative moderate eccentric aortic regurgitation (AR) required an aortic valve replacement 1.8 years later. The 5-year freedom from re-operation was 92.8%. There has been no progression of the size of the aortic root in any patient on follow up echocardiograms and/or magnetic resonance imaging. Conclusion: PEARS can be used to prevent progressive dilation of the aortic root in the paediatric population with satisfactory mid-term outcomes.