title: Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival.
abstract: BACKGROUND
  Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease.
  
  
  RESULTS
  A total of 1188 patients with adult congenital heart disease (age, 33.1±13.1 years) undergoing lung function testing between 2000 and 2009 were included. Patients were classified according to the severity of lung dysfunction based on predicted values of forced vital capacity. Lung function was normal in 53% of patients with adult congenital heart disease, mildly impaired in 17%, and moderately to severely impaired in the remainder (30%). Moderate to severe impairment of lung function related to complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy/ies, body mass index, scoliosis, and diaphragm palsy. Over a median follow-up period of 6.7 years, 106 patients died. Moderate to severe impairment of lung function was an independent predictor of survival in this cohort. Patients with reduced force vital capacity of at least moderate severity had a 1.6-fold increased risk of death compared with patients with normal lung function (P=0.04).
  
  
  CONCLUSIONS
  A reduced forced vital capacity is prevalent in patients with adult congenital heart disease; its severity relates to the complexity of the underlying heart defect, surgical history, and scoliosis. Moderate to severe impairment of lung function is an independent predictor of mortality in contemporary patients with adult congenital heart disease.
authors: Adult Congenital Heart Disease Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, NIHR Cardiovascular Biomedical Research Unit, London, UK.
authors: Rafael
authors: R
authors: Alonso-Gonzalez
authors: Francesco
authors: F
authors: Borgia
authors: Gerhard-Paul
authors: GP
authors: Diller
authors: Ryo
authors: R
authors: Inuzuka
authors: Aleksander
authors: A
authors: Kempny
authors: Ana
authors: A
authors: Martinez-Naharro
authors: Oktay
authors: O
authors: Tutarel
authors: Philip
authors: P
authors: Marino
authors: Kerstin
authors: K
authors: Wustmann
authors: Menelaos
authors: M
authors: Charalambides
authors: Margarida
authors: M
authors: Silva
authors: Lorna
authors: L
authors: Swan
authors: Konstantinos
authors: K
authors: Dimopoulos
authors: Michael A
authors: MA
authors: Gatzoulis
mesh_terms: D000328:Adult; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006321:Heart / Q000503:physiopathology*; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology*; D006801:Humans; D008171:Lung Diseases / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012129:Respiratory Function Tests / Q000379:methods; D015996:Survival Rate / Q000639:trends; D014797:Vital Capacity / Q000502:physiology*; D055815:Young Adult