{
  "abstract": "Background Previous research on congenital aortic stenosis (AS) mainly focused on children, while data on long-term outcomes in adults is scarce. Therefore, this study aims to evaluate outcomes in adult patients with congenital AS and identify prognostic markers for aortic valve replacement (AVR).Methods In this multicentre study, patients aged 18–55 years with congenital AS (peak velocity ≥2.5 m/s) registered in the Dutch CONCOR (Congential Cor Vitia) registry from expert centres for congenital heart disease between 2001 and 2019 were included. Exclusion criteria were severe aortic regurgitation (AR) or prior AVR. Associations with the composite endpoint of all-cause mortality and AVR were assessed with multivariable Cox regression.Results Of the 427 included patients (median age 26 years, 62.5% male) median aortic peak velocity was 3.1 (IQR 2.7–3.6) m/s, 35% had moderate AR and 29% showed signs of left ventricular (LV) remodelling. During a median follow-up period of 7.9 (IQR 4.1–12.6) years, 7 patients died (1.6%) and 177 patients underwent AVR (41.5%). LV systolic dysfunction was the primary indication for AVR in three patients (1.7%). Peak velocity at baseline (HR 3.17, 95% CI 2.29 to 4.39), non-sinus rhythm (HR 3.12, 95% CI 1.62 to 6.02) and concentric LV geometry (HR 1.64, 95% CI 1.04 to 2.58) were associated with the primary endpoint beside age. Significant male–female differences were observed in prognostic factors for the primary endpoint.Conclusion AVR was often indicated in adult patients with congenital AS, even if the stenosis at baseline was mild. Moreover, monitoring LV remodelling alongside severity of AS is more important than focusing on systolic LV dysfunction.",
  "authors": [
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Zoë A Keuning"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Frederike Meccanici"
    },
    {
      "affiliations": [
        "Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands"
      ],
      "name": "Bibi A Schreurs"
    },
    {
      "affiliations": [
        "Department of Cardiothoracic Surgery, Thorax Center, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Kevin Veen"
    },
    {
      "affiliations": [
        "Department of Cardiology, Amsterdam UMC Location AMC, Amsterdam, Netherlands"
      ],
      "name": "Berto J Bouma"
    },
    {
      "affiliations": [
        "Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands"
      ],
      "name": "Michiel Voskuil"
    },
    {
      "affiliations": [
        "Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands"
      ],
      "name": "Monique R M Jongbloed"
    },
    {
      "affiliations": [
        "Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands"
      ],
      "name": "Joost P van Melle"
    },
    {
      "affiliations": [
        "Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands"
      ],
      "name": "Roland R J van Kimmenade"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Rowina Kapoor"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Irem Bozkurt"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands",
        "Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Alexander Hirsch"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN-GUARD-HEART), Amsterdam, The Netherlands"
      ],
      "name": "Jolien W Roos-Hesselink"
    },
    {
      "affiliations": [
        "Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN-GUARD-HEART), Amsterdam, The Netherlands"
      ],
      "name": "Annemien E van den Bosch"
    }
  ],
  "title": "Long-term clinical outcomes in adult congenital aortic stenosis: results from a national study",
  "uid": "4b060e8e-9191-5623-8310-08833dc2ef55"
}