Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease

Congenit Heart Dis. 2013 May-Jun;8(3):246-54. doi: 10.1111/chd.12010. Epub 2012 Oct 18.


Background/objectives: Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with CHD.

Methods: One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data were obtained by retrospective chart review.

Results: Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18-63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio = 9.01, P =.05) and history of atrial arrhythmias (odd ratio = 4.25, P =.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio = 3.65, P =.03). Patients with spirometry suggesting RLD also had lower exercise duration (P =.004) and a higher New York Heart Association Functional Class (P =.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity.

Conclusion: Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Exercise Test
  • Exercise Tolerance*
  • Female
  • Heart Defects, Congenital / complications
  • Heart Defects, Congenital / diagnosis
  • Heart Defects, Congenital / physiopathology
  • Heart Defects, Congenital / surgery*
  • Humans
  • Logistic Models
  • Lung / physiopathology*
  • Lung Diseases / complications*
  • Lung Diseases / diagnosis
  • Lung Diseases / physiopathology
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Odds Ratio
  • Predictive Value of Tests
  • Retrospective Studies
  • Risk Factors
  • Spirometry
  • Young Adult