Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival

Konstantinos Dimopoulos, Darlington O. Okonko, Gerhard Paul Diller, Craig Broberg, Tushar V. Salukhe, Sonya V. Babu-Narayan, Wei Li, Anselm Uebing, Stephanie Bayne, Roland Wensel, Massimo F. Piepoli, Philip A. Poole-Wilson, Darrel P. Francis, Michael A. Gatzoulis

Research output: Contribution to journalArticle

186 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)2796-2802
Number of pages7
JournalCirculation
Volume113
Issue number24
DOIs
StatePublished - Jun 2006
Externally publishedYes

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Cyanosis
Heart Diseases
Exercise
Survival
Pulmonary Hypertension
Mortality
Carbon Dioxide
Oxygen Consumption
Ventilation
Multivariate Analysis

Keywords

  • Congenital heart defects
  • Exercise test
  • Prognosis
  • Ventilation

ASJC Scopus subject areas

  • Physiology
  • Cardiology and Cardiovascular Medicine

Cite this

Dimopoulos, K., Okonko, D. O., Diller, G. P., Broberg, C., Salukhe, T. V., Babu-Narayan, S. V., ... Gatzoulis, M. A. (2006). Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation, 113(24), 2796-2802. https://doi.org/10.1161/CIRCULATIONAHA.105.594218

Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. / Dimopoulos, Konstantinos; Okonko, Darlington O.; Diller, Gerhard Paul; Broberg, Craig; Salukhe, Tushar V.; Babu-Narayan, Sonya V.; Li, Wei; Uebing, Anselm; Bayne, Stephanie; Wensel, Roland; Piepoli, Massimo F.; Poole-Wilson, Philip A.; Francis, Darrel P.; Gatzoulis, Michael A.

In: Circulation, Vol. 113, No. 24, 06.2006, p. 2796-2802.

Research output: Contribution to journalArticle

Dimopoulos, K, Okonko, DO, Diller, GP, Broberg, C, Salukhe, TV, Babu-Narayan, SV, Li, W, Uebing, A, Bayne, S, Wensel, R, Piepoli, MF, Poole-Wilson, PA, Francis, DP & Gatzoulis, MA 2006, 'Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival', Circulation, vol. 113, no. 24, pp. 2796-2802. https://doi.org/10.1161/CIRCULATIONAHA.105.594218
Dimopoulos, Konstantinos ; Okonko, Darlington O. ; Diller, Gerhard Paul ; Broberg, Craig ; Salukhe, Tushar V. ; Babu-Narayan, Sonya V. ; Li, Wei ; Uebing, Anselm ; Bayne, Stephanie ; Wensel, Roland ; Piepoli, Massimo F. ; Poole-Wilson, Philip A. ; Francis, Darrel P. ; Gatzoulis, Michael A. / Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. In: Circulation. 2006 ; Vol. 113, No. 24. pp. 2796-2802.
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abstract = "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.",
keywords = "Congenital heart defects, Exercise test, Prognosis, Ventilation",
author = "Konstantinos Dimopoulos and Okonko, {Darlington O.} and Diller, {Gerhard Paul} and Craig Broberg and Salukhe, {Tushar V.} and Babu-Narayan, {Sonya V.} and Wei Li and Anselm Uebing and Stephanie Bayne and Roland Wensel and Piepoli, {Massimo F.} and Poole-Wilson, {Philip A.} and Francis, {Darrel P.} and Gatzoulis, {Michael A.}",
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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

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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