CLINICAL RESEARCH: CONGENITAL HEART DISEASE IN THE ADULT
Ventilatory Efficiency and Aerobic Capacity Predict Event-Free Survival in Adults With Atrial Repair for Complete Transposition of the Great Arteries
Alessandro Giardini, MD, PhD*, ,*,
Alfred Hager, MD ,
Astrid E. Lammers, MD ,
Graham Derrick, MD ,
Jan Müller, MSc ,
Gerhard-Paul Diller, MD ,
Konstantinos Dimopoulos, MSc, MD ,
Dolf Odendaal, MSc ,
Gaetano Gargiulo, MD*,
Fernando M. Picchio, MD* and
Michael A. Gatzoulis, MD, PhD
* Pediatric Cardiology/Cardiac Surgery and Adult Congenital Unit, University of Bologna, Bologna, Italy
Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum, Munich, Germany
Cardiac Unit, Great Ormond Street Hospital for Children, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, United Kingdom
Adult Congenital Heart Center and Center for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, United Kingdom
Manuscript received October 29, 2008;
revised manuscript received January 27, 2009,
accepted February 3, 2009.
* Reprint requests and correspondence: Dr. Alessandro Giardini, Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy (Email: alessandro5574{at}iol.it).
Objectives: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation.
Background: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population.
Methods: Between 1996 and 2007, 274 adults (age 26.3 ± 8.9 years, range 16 to 50 years) who had received a Mustard (n = 144) or Senning (n = 130) operation in infancy were studied with CPET. During a follow-up of 3.9 ± 2.3 years (range 0.2 to 10.8 years), 12 patients died at an age of 36 ± 14 years, and 46 patients required a cardiac-related emergency (<24 h from the onset of symptom/condition) hospital admission at an age of 30 ± 11 years.
Results: At multivariate Cox analysis, the slope of ventilation per unit of carbon dioxide output (VE/VCO2 slope) (hazard ratio: 1.088, p < 0.0001) and percentage of predicted peak oxygen uptake (VO
2%) (hazard ratio: 0.979, p = 0.0136) were the strongest predictors of death/cardiac-related emergency hospital admission among demographic, clinical, and exercise variables. A VE/VCO2 slope 35.4 (hazard ratio: 10.7, 95% confidence interval [CI]: 7.8 to 24.6), and a peak VO
2% 52.3% (hazard ratio: 3.4, 95% CI: 2.5 to 8.2) were associated with an increased 4-year risk of death/cardiac-related emergency hospital admission. Patients who had both a VE/VCO2 slope 35.4 and a peak VO
2% 52.3% of predicted value were at highest risk (4-year event rate: 78.8%).
Conclusions: CPET provides important prognostic information in adults with M/S operation. Subjects with enhanced ventilatory response to exercise or those with poor exercise capacity have a substantially higher 4-year risk of death/cardiac-related emergency hospital admission.
Key Words: transposition of great vessels exercise prognosis
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Abbreviations and Acronyms
| | CPET = cardiopulmonary exercise test | | HF = heart failure | | HR = heart rate | | M/S = Mustard and Senning | | PH = pulmonary hypertension | | ROC = receiver-operator characteristic | | RV = systemic right ventricle | | TGA = transposition of the great arteries | | VE/VCO2
= minute ventilation/carbon dioxide production | | VO
2
= oxygen uptake |
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Ventilatory efficiency and aerobic capacity predict event-free survival in adults with atrial repair for complete transposition of the great arteries.
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