{
  "abstract": "Objectives Data on long-term outcomes after surgical repair of pulmonary valve stenosis are limited. This study evaluated survival, clinical outcomes and quality of life (QoL) after surgery during childhood.Methods Single centre, longitudinal cohort study evaluating consecutive patients with pulmonary valve stenosis who underwent surgical repair between 1968–1980 and were evaluated every decade since 1990.Results Of the original cohort of 89 operated patients, 11 died (12%), including 2 who died within 30 days postsurgery (2%), and 7 (8%) were lost to follow-up. Survival at 50 years follow-up was 87%, which was not significantly different from the GDP. Of the remaining 71 survivors, 32 refrained earlier from participating in this cohort study, leaving 39 eligible, of whom 34 (87%) participated again (50% male, median age 48 years) with a median follow-up of 45 (range 40–52) years. Event-free survival was 50%, with supraventricular tachycardia (14%) and reintervention (13%) being the most frequent events, although less frequently in the last 10 years. At last follow-up, biventricular function was preserved in most patients. Reduced right and left ventricular ejection fraction (EF) was found in 33% and 13%, respectively. Exercise capacity and maximum rate of oxygen consumption were mildly impaired in 14% and 32% of patients. Patients who underwent an infundibulectomy during initial surgery were significantly more likely to undergo reintervention (HR=8.32, p=0.003). Patient-reported QoL scores remained stable over time and consistently exceeded those of the age-matched GDP.Conclusion Fifty-year survival after surgery for pulmonary valve stenosis was excellent and comparable to the GDP. Most patients maintained preserved ventricular function, functional capacity and excellent QoL. Routine lifelong follow-up may not be necessary for all patients, but should be considered for those who underwent an infundibulectomy or have residual lesions.",
  "authors": [
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Sahra Ünlütürk"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERNGUARD-Heart), Amsterdam, The Netherlands"
      ],
      "name": "Robert M Kauling"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERNGUARD-Heart), Amsterdam, The Netherlands"
      ],
      "name": "Judith A A E Cuypers"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERNGUARD-Heart), Amsterdam, The Netherlands"
      ],
      "name": "Annemien E van den Bosch"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands",
        "Department of Radiology and Nuclear Medicine, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Alexander Hirsch"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Chiara Pelosi"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Daniel J Bowen"
    },
    {
      "affiliations": [
        "Department of Cardiothoracic Surgery, Thorax Center, Erasmus MC, Rotterdam, The Netherlands"
      ],
      "name": "Ad J J C Bogers"
    },
    {
      "affiliations": [
        "Department of Pediatrics, Division of Cardiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"
      ],
      "name": "Willem A Helbing"
    },
    {
      "affiliations": [
        "Clinical Epidemiology and Innovation Unit, Department of Cardiology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands"
      ],
      "name": "Isabella Kardys"
    },
    {
      "affiliations": [
        "Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus MC, Rotterdam, The Netherlands",
        "European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERNGUARD-Heart), Amsterdam, The Netherlands"
      ],
      "name": "Jolien W Roos-Hesselink"
    }
  ],
  "title": "Outcome 50 years after surgical repair of pulmonary valve stenosis: a longitudinal cohort study",
  "uid": "05026e70-caf0-524c-9dbd-a5d1e72222f3"
}