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      Long-term follow-up and outcomes of discrete subaortic stenosis resection in children

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          Abstract

          Background:

          Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution.

          Materials and Methods:

          We retrospectively reviewed the records of patients ( n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency.

          Results:

          The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively ( P < 0.001). The peak instantaneous LVOT Doppler gradient decreased from 75.41 ± 15.22 mmHg preoperatively to 18.11 ± 11.44 mmHg postoperatively ( P < 0.001). At the latest follow-up, the peak gradient was 17.63 ± 8.93 mmHg. The mean follow-up was 7.47 ± 3.53 years (median 6.33 years; range 2.67–16 years). There was no operative mortality or late mortality. Recurrence of subaortic membrane occurred in 7 (25.92%, 7/27) patients who underwent primary DSS operation. Four (14.81%, 4/27) patients required reoperation for DSS recurrence at a median time of 4.8 years (3.1–9.1 years) after the initial repair. Risk factors for reoperation were age <6 years at initial repair. Eighteen (66.66%, 18/27) patients had AI preoperatively and progression of AI occurred in 70.37% (19/27). This included 4 (22.22%, 4/18) patients who had worsening of their preoperative AI. Short valve-to-membrane distance was found to be prognostically unfavorable. One (3.7%, 1/27) patient had an iatrogenic ventricular septal defect, and 2 (7.4%, 2/27) patients had complete AV block following membrane resection.

          Conclusions:

          Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

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          Most cited references 29

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          ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

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            Left ventricular outflow obstruction: subaortic stenosis, bicuspid aortic valve, supravalvar aortic stenosis, and coarctation of the aorta.

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              Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation.

              We sought to determine the prevalence and rate of progression of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) in adults with discrete subaortic stenosis (DSS).
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                Author and article information

                Journal
                Ann Pediatr Cardiol
                Ann Pediatr Cardiol
                APC
                Annals of Pediatric Cardiology
                Wolters Kluwer - Medknow (India )
                0974-2069
                0974-5149
                Sep-Dec 2019
                : 12
                : 3
                : 212-219
                Affiliations
                [1 ]Department of Pediatrics, Chest Diseases Hospital, Kuwait City, Kuwait
                [2 ]Faculty of Medicine, Kuwait University, Kuwait City, Kuwait
                [3 ]Department of Pediatric Cardiology, Ministry of Health, Chest Diseases Hospital, Kuwait City, Kuwait
                [4 ]Health Sciences Center, Faculty of Medicine, Kuwait University, Kuwait City, Kuwait
                Author notes
                Address for correspondence: Prof. Lulu Abushaban, Chest Diseases Hospital, Kuwait University, Kuwait City, Kuwait. E-mail: luluabushaban@ 123456hotmail.com
                Article
                APC-12-212
                10.4103/apc.APC_120_18
                6716322
                Copyright: © 2018 Annals of Pediatric Cardiology

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

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