TY - JOUR
T1 - Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival
AU - Dimopoulos, Konstantinos
AU - Okonko, Darlington O.
AU - Diller, Gerhard Paul
AU - Broberg, Craig S.
AU - Salukhe, Tushar V.
AU - Babu-Narayan, Sonya V.
AU - Li, Wei
AU - Uebing, Anselm
AU - Bayne, Stephanie
AU - Wensel, Roland
AU - Piepoli, Massimo F.
AU - Poole-Wilson, Philip A.
AU - Francis, Darrel P.
AU - Gatzoulis, Michael A.
PY - 2006/6
Y1 - 2006/6
N2 - BACKGROUND - Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V̇e/V̇co2 slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V̇e/V̇co2 slope across a wide spectrum of ACHD patients. METHODS AND RESULTS - Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2±12.9 years (mean±SD). Peak oxygen consumption was 23.5±9.0 mL·kg·min. V̇e/V̇co2 slope for all patients was 36.3±15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of abnormal V̇e/V̇co2 slope. The V̇e/V̇co2 slope was the most powerful univariate predictor of mortality in the noncyanotic group and the only independent predictor of mortality among exercise parameters on multivariate analysis. In cyanotic patients, no parameter was predictive of death. CONCLUSIONS - Ventilatory response to exercise is abnormal across the spectrum of ACHD. Cyanosis is a powerful stimulus for such exaggerated ventilatory patterns irrespective of the presence of pulmonary arterial hypertension. Increased V̇e/V̇co2 slope is the strongest exercise predictor of death in noncyanotic ACHD patients.
AB - BACKGROUND - Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V̇e/V̇co2 slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V̇e/V̇co2 slope across a wide spectrum of ACHD patients. METHODS AND RESULTS - Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2±12.9 years (mean±SD). Peak oxygen consumption was 23.5±9.0 mL·kg·min. V̇e/V̇co2 slope for all patients was 36.3±15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of abnormal V̇e/V̇co2 slope. The V̇e/V̇co2 slope was the most powerful univariate predictor of mortality in the noncyanotic group and the only independent predictor of mortality among exercise parameters on multivariate analysis. In cyanotic patients, no parameter was predictive of death. CONCLUSIONS - Ventilatory response to exercise is abnormal across the spectrum of ACHD. Cyanosis is a powerful stimulus for such exaggerated ventilatory patterns irrespective of the presence of pulmonary arterial hypertension. Increased V̇e/V̇co2 slope is the strongest exercise predictor of death in noncyanotic ACHD patients.
KW - Congenital heart defects
KW - Exercise test
KW - Prognosis
KW - Ventilation
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U2 - 10.1161/CIRCULATIONAHA.105.594218
DO - 10.1161/CIRCULATIONAHA.105.594218
M3 - Article
C2 - 16769913
AN - SCOPUS:33745949142
SN - 0009-7322
VL - 113
SP - 2796
EP - 2802
JO - Circulation
JF - Circulation
IS - 24
ER -