Relation of right ventricular dilation, age of repair, and restrictive right ventricular physiology with patient-reported quality of life in adolescents and adults with repaired tetralogy of fallot

Am J Cardiol. 2010 Dec 15;106(12):1798-802. doi: 10.1016/j.amjcard.2010.08.021. Epub 2010 Nov 2.


The present study aimed to determine the predictors of patient-reported quality of life and restrictive right ventricular (RV) physiology in adolescents and adults with repaired tetralogy of Fallot. A total of 62 patients (median age 28.5 years, range 14 to 69) undergoing cardiovascular magnetic resonance imaging completed the Short Form 36-item questionnaire, version 2, a validated quality of life assessment. RV inflow curves were generated from the sum of tricuspid inflow and pulmonary insufficiency. The patient-reported quality of life was comparable to population norms. Patients repaired after 1 year of age showed a strong trend toward a greater likelihood of physical component summary age-adjusted z-score ≤-1 (odds ratio 7.50, 95% confidence interval 0.90 to 62.3, p = 0.06). Patients with a RV ejection fraction of <45% reported decreased physical component summary (p = 0.02) and physical functioning (p = 0.02) scores. The RV end-diastolic volume, pulmonary regurgitation, and diastolic indexes did not predict the quality of life. The indexed RV end-diastolic volume was related to diastolic abnormalities, correlating with a greater peak early filling rate (r = 0.71, p <0.0001), ratio of peak early to atrial filling rates (r = 0.45, p = 0.006), and showing a strong trend with the end-diastolic forward flow in the pulmonary trunk (odds ratio 2.67 for moderate dilation and 3.50 for severe dilation, p = 0.06). Patients who underwent repair before 1 year old were more likely to have end-diastolic forward flow (15 of 17 vs 25 of 42, p = 0.03). In conclusion, the RV ejection fraction and age of repair were the best predictors of quality of life in this population, in whom end-diastolic forward flow and associated diastolic parameters appeared to reflect an overdistended ventricle, which might suggest a role for early pulmonary valve replacement.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Cardiac Surgical Procedures / methods*
  • Cardiomyopathy, Dilated / diagnosis
  • Cardiomyopathy, Dilated / physiopathology*
  • Cardiomyopathy, Dilated / psychology
  • Diastole
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Myocardial Contraction / physiology*
  • Prognosis
  • Quality of Life*
  • Retrospective Studies
  • Surveys and Questionnaires
  • Tetralogy of Fallot / psychology
  • Tetralogy of Fallot / surgery*
  • Ventricular Dysfunction, Right / diagnosis
  • Ventricular Dysfunction, Right / physiopathology*
  • Ventricular Dysfunction, Right / psychology
  • Young Adult