Jan Müller1, Alfred Hager2, Gerhard-Paul Diller3, Graham Derrick4, Roselien Buys5, Karl-Otto Dubowy6, Tim Takken7, Stefan Orwat8, Ryo Inuzuka9, Luc Vanhees5, Michael Gatzoulis9, Alessandro Giardini4. 1. Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Germany. Electronic address: j.mueller@tum.de. 2. Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Germany. 3. Adult Congenital Heart Center and Center for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, United Kingdom; Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital of Muenster, Muenster, Germany. 4. Cardiorespiratory Unit, Great Ormond Street Hospital for Children, London, United Kingdom. 5. Research Centre for Cardiovascular and Respiratory Rehabilitation, Department of Rehabilitation Sciences, Katholieke Universiteit Leuven, Belgium. 6. Department of Paediatric Cardiology and Congenital Heart Disease, Heart and Diabetes Center of North Rhine-Westphalia, University of Bochum, Bad Oeynhausen, Germany. 7. Child Development & Exercise Centre, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands. 8. Adult Congenital and Valvular Heart Disease Center, Department of Cardiology and Angiology, University Hospital of Muenster, Muenster, Germany. 9. Adult Congenital Heart Center and Center for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, United Kingdom.
Abstract
OBJECTIVE: Patients with repaired tetralogy of Fallot (ToF) have an increased long-term risk of cardiovascular morbidity and mortality. Risk stratification in this population is difficult. Initial evidence suggests that cardiopulmonary exercise testing (CPET) may be helpful to risk-stratify patients with repaired ToF. METHODS AND RESULTS: We studied 875 patients after surgical repair for ToF (358 females, age 25.5 ± 11.7 year, range 7-75 years) who underwent CPET between 1999 and 2009. During a mean follow-up of 4.1 ± 2.6 years after CPET, 30 patients (3.4%) died or had sustained ventricular tachycardia (VT). 225 patients (25.7%) had other cardiac related events (emergency admission, surgery, or catheter interventions). On multivariable Cox regression-analysis, %predicted peak oxygen uptake (V˙O2 %) (p=0.001), resting QRS duration (p=0.030) and age (p<0.001) emerged as independent predictors of mortality or sustained VT. Patients with a peak V˙O2 ≤ 65% of predicted and a resting QRS duration ≥ 170 ms had a 11.4-fold risk of death or sustained VT. Ventilatory efficiency expressed as V˙E/V˙CO2 slope (p<0.001), peak V˙O2 % (p=.001), QRS duration (p=.001) and age (p=0.046) independently predicted event free survival. V˙E/V˙CO2 slope ≥ 31.0, peak V˙O2 % ≤ 65% and QRS duration ≥ 170 ms were the cut-off points with best sensitivity and specificity to detect an unfavorable outcome. CONCLUSIONS: CPET is an important predictive tool that may assist in the risk stratification of patients with ToF. Subjects with a poor exercise capacity in addition to a prolonged QRS duration have a substantially increased risk for death or sustained ventricular tachycardia, as well as for cardiac-related hospitalizations.
OBJECTIVE:Patients with repaired tetralogy of Fallot (ToF) have an increased long-term risk of cardiovascular morbidity and mortality. Risk stratification in this population is difficult. Initial evidence suggests that cardiopulmonary exercise testing (CPET) may be helpful to risk-stratify patients with repaired ToF. METHODS AND RESULTS: We studied 875 patients after surgical repair for ToF (358 females, age 25.5 ± 11.7 year, range 7-75 years) who underwent CPET between 1999 and 2009. During a mean follow-up of 4.1 ± 2.6 years after CPET, 30 patients (3.4%) died or had sustained ventricular tachycardia (VT). 225 patients (25.7%) had other cardiac related events (emergency admission, surgery, or catheter interventions). On multivariable Cox regression-analysis, %predicted peak oxygen uptake (V˙O2 %) (p=0.001), resting QRS duration (p=0.030) and age (p<0.001) emerged as independent predictors of mortality or sustained VT. Patients with a peak V˙O2 ≤ 65% of predicted and a resting QRS duration ≥ 170 ms had a 11.4-fold risk of death or sustained VT. Ventilatory efficiency expressed as V˙E/V˙CO2 slope (p<0.001), peak V˙O2 % (p=.001), QRS duration (p=.001) and age (p=0.046) independently predicted event free survival. V˙E/V˙CO2 slope ≥ 31.0, peak V˙O2 % ≤ 65% and QRS duration ≥ 170 ms were the cut-off points with best sensitivity and specificity to detect an unfavorable outcome. CONCLUSIONS: CPET is an important predictive tool that may assist in the risk stratification of patients with ToF. Subjects with a poor exercise capacity in addition to a prolonged QRS duration have a substantially increased risk for death or sustained ventricular tachycardia, as well as for cardiac-related hospitalizations.
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