Usefulness of cardiopulmonary exercise to predict long-term prognosis in adults with repaired tetralogy of Fallot

Adults with tetralogy of Fallot (TOF) have increased long-term mortality. The identification of patients at greater risk for death or cardiac-related morbidity is challenging. This study was conducted to assess the prognostic value of cardiopulmonary exercise testing in adults with repaired TOF. One hundred eighteen consecutive adults with repaired TOF (mean age at repair 4.8 ± 4.2 years) underwent cardiopulmonary exercise testing at a mean age of 24 ± 8 years (range 16 to 59). The degree of pulmonary regurgitation, right ventricular function, and right ventricular systolic pressure were determined by transthoracic echocardiography. After the exercise tests, patients were regularly followed up for cardiac-related events. During a mean follow-up of 5.8 ± 2.3 years (range 0.6 to 9.7), 9 patients died and 18 underwent hospitalization. Peak oxygen uptake (hazard ratio 0.974, 95% confidence interval 0.950 to 0.994), the slope of ventilation (VE) per unit of carbon dioxide production (VCO2) (hazard ratio 1.076, 95% confidence interval 1.038 to 1.115), and New York Heart Association functional class (hazard ratio 2.118, 95% confidence interval 1.344 to 3.542) were independent predictors of death or hospitalization. Patients with peak oxygen uptake ≤36% of predicted value and those with VE/VCO2 slopes >39 were at greater risk for cardiac-related death (5-year mortality 48% vs 0%, p <0.0001, and 31% vs 0%, p <0.0001, respectively). In conclusion, the measurement of peak oxygen uptake and VE/VCO2 slope in adults with repaired TOF can be prognostically important and could become a powerful tool to rationalize decisions regarding the prevention of premature sudden death and the need for reintervention. © 2007 Elsevier Inc. All rights reserved

Recovery kinetics of oxygen uptake is abnormally prolonged in patients with Mustard/Senning repair for transposition of the great arteries

The aim of this study was to evaluate the ability to recover from exercise in patients with a Mustard/Senning (M/S) repair for transposition of the great arteries and to identify the major determinants. A total of 40 consecutive patients with a M/S repair at a mean age of 10.0 +/- 9.8 months underwent maximal cardiopulmonary exercise testing at 19.5 +/- 11.3 years of age. Results were compared to those of a cohort of 153 healthy individuals. Decay of oxygen uptake (VO2), CO2 (VCO2), minute ventilation (VE), heart rate (HR) was calculated for the first minute of recovery. M/S patients had reduced peak VO2 (22.9 +/- 7.2 vs 34.2 +/- 9.5 ml O-2/kg/min, p < 0.0001) and VO2 slope (0.27 +/- 0.10 vs 0.47 +/- 0.2 L O-2/min, p < 0.0001), Peak 02 pulse (p < 0.0001) and peak HR (p = 0.001) were reduced. VCO2 and VE slopes were reduced (p < 0.0001 for both), whereas HR slope was similar (p = 0.38). In M/S patients, the only independent determinants Of V02 slope during recovery were pulse 02 slope (p < 0.0001) and VCO2 slope (p < 0.0001). In M/S patients, a limited cardiopulmonary reserve affects not only maximal exercise responses but also the recovery phase. A prolonged recovery Of 02 pulse and a prolonged CO2 retention with subsequent prolonged hyperpnea are the main determinants of the delayed recover

Strong and independent prognostic value of peak circulatory power in adults with congenital heart disease

The identification of patients with adult congenital heart disease (ACHD) who are at higher risk of death is challenging. Peak circulatory power (CircP; expressed as peak exercise oxygen uptake multiplied for peak mean arterial blood pressure) is a strong predictor of death in adults with acquired heart disease. We sought to establish the distribution and the prognostic value of peak CircP across a wide spectrum of patients with ACHD. METHODS: Four hundred thirty-two consecutive patients with ACHD of varying diagnosis underwent cardiopulmonary exercise testing at a single laboratory between 1996 and 2005. Patient age was 32 +/- 10 years. RESULTS: A gradual variation in peak CircP was found across the spectrum of congenital heart defects (P < .0001 at analysis of variance). Reduced peak CircP values were associated with the presence of heart failure symptoms (P < .0001), absence of sinus rhythm (P = .010), and use of antiarrhythmic medications (P = .0013). At a follow-up of 4.4 +/- 2.4 years, 23 patients (5.3%) had died. Peak CircP was a strong predictor of mortality when univariate analysis was used and the strongest independent predictor of mortality among exercise parameters. A peak CircP < or = 1476 mm Hg mlO2 min(-1) kg(-2) was associated with a 15.4-fold increase in the 4-year risk of death. CONCLUSIONS: Peak CircP is abnormal across the spectrum of ACHD. Peak CircP appears as the strongest predictor of adverse outcome in ACHD