title: Clinical course and potential complications of small ventricular septal defects in adulthood: Late development of left ventricular dysfunction justifies lifelong care.
abstract: BACKGROUND
  Patients with small ventricular septal defects (VSDs) are thought to have excellent long-term survival, although complications may not be uncommon.
  
  
  METHODS
  We identified all patients aged ≥ 16 years with native isolated VSD between January 2000 and September 2013. Clinical outcomes were retrospectively reviewed. Transthoracic echocardiograms performed within 2 years of last assessment were reviewed for hemodynamic sequelae.
  
  
  RESULTS
  Two-hundred-and-thirty-one patients, 100 (43%) male, mean age at last follow-up 34 ± 13 years were studied. During the recorded period there were no deaths. The majority (224/231; 97%) were asymptomatic. Documented arrhythmias occurred in 7 patients (3%), double-chamber right ventricle (DCRV) in 29 (13%), more than mild aortic regurgitation in 6 (3%) and infective endocarditis in 24 (10%) patients. Surgery due to complications associated with VSD was performed in 26 (11%) patients at a median age of 27.6 years (IQR: 16.1-38.7) due to DCRV (n=17, 65%), infective endocarditis (n=6, 23%), progression of left-right shunt (n=2, 8%) and aortic regurgitation (n=1, 4%). At most recent echocardiography (n=164), 10 (6%), had reduced LVEF, 34 (21%) had increased LVEDD and 17 (10%) had LVESD >4.0 cm. Thirty-two patients (25%) with normal LV dimensions had LA enlargement suggesting LV diastolic dysfunction.
  
  
  CONCLUSIONS
  We report a non-negligible incidence of major complications or clinical events during late follow-up of adult patients with restrictive VSDs. Furthermore, we show co-existing LV dysfunction, systolic or diastolic in a subset of patients. Indication for VSD closure in childhood may be recognized, whereas lifelong follow-up for adult with restrictive VSDs is clearly warranted.
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom.
authors: Theodoros
authors: T
authors: Karonis
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; Division of Cardiology, Cardio-thoracic and Respiratory Sciences Department, Second University of Naples, AO Dei Colli-Monaldi Hospital, Leonardo Bianchi Street, 80100 Naples, Italy.
authors: Giancarlo
authors: G
authors: Scognamiglio
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom.
authors: Sonya V
authors: SV
authors: Babu-Narayan
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom.
authors: Claudia
authors: C
authors: Montanaro
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom.
authors: Anselm
authors: A
authors: Uebing
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; Adult Congenital and Valvular Heart Disease Centre, Department of Cardiology and Angiology, University Hospital Muenster, Albert-Schweitzer-Campus 1, 48149 Muenster, Germany.
authors: Gerhard P
authors: GP
authors: Diller
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom.
authors: Rafael
authors: R
authors: Alonso-Gonzalez
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom.
authors: Lorna
authors: L
authors: Swan
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom.
authors: Konstantinos
authors: K
authors: Dimopoulos
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom.
authors: Michael A
authors: MA
authors: Gatzoulis
authors: Royal Brompton and Harefield NHS Foundation Trust, Sydney Street, London SW3 6NP, United Kingdom; National Heart and Lung Institute, Imperial College London, Dovehouse Street, London SW3 6LY, United Kingdom; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and Imperial College London, United Kingdom. Electronic address: w.li@rbht.nhs.uk.
authors: Wei
authors: W
authors: Li
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008136:Longevity; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D055815:Young Adult
keywords: Adult; Congenital; Double chamber right ventricle; Endocarditis; Heart defects; Ventricular septal defect