Abnormal Ventilatory Response to Exercise in Adults With Congenital Heart Disease Relates to Cyanosis and Predicts Survival (original) (raw)
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Circulation, 2012
Background-Parameters of cardiopulmonary exercise testing were recently identified as strong predictors of mortality in adults with congenital heart disease. We hypothesized that combinations of cardiopulmonary exercise testing parameters may provide optimal prognostic information on midterm survival in this population. Methods and Results-A total of 1375 consecutive adult patients with congenital heart disease (age, 33Ϯ13 years) underwent cardiopulmonary exercise testing at a single center over a period of 10 years. Peak oxygen consumption (peak V O 2 ), ventilation per unit of carbon dioxide production (V E/V CO 2 slope), and heart rate reserve were measured. During a median follow-up of 5.8 years, 117 patients died. Peak V O 2 , heart rate reserve, and V E/V CO 2 slope were related to midterm survival in adult patients with congenital heart disease. Risk of death increased with lower peak V O 2 and heart rate reserve. A higher V E/V CO 2 slope was also related to increased risk of death in noncyanotic patients, whereas the V E/V CO 2 slope was not predictive of mortality in cyanotic patients. The combination of peak V O 2 and heart rate reserve provided the greatest predictive information after adjustment for clinical parameters such as negative chronotropic agents, age, and presence of cyanosis. However, the incremental value of these exercise parameters was reduced in patients with peak respiratory exchange ratio Ͻ1.0. Conclusions-Cardiopulmonary exercise testing provides strong prognostic information in adult patients with congenital heart disease. Prognostication should be approached differently, depending on the presence of cyanosis, use of rate-lowering medications, and achieved level of exercise. We provide 5-year survival prospects based on cardiopulmonary exercise testing parameters in this growing population. (Circulation. 2012;125:250-259.)
Dynamics of oxygen uptake during exercise in adults with cyanotic congenital heart disease
Circulation, 1986
The dynamic increase in oxygen uptake (VO2) at the start of exercise reflects the circulatory adjustments to metabolic changes induced by the exercise. Because VO2 measured at the lungs is the product of pulmonary blood flow and arteriovenous oxygen difference, pathologic conditions affecting the capacity of these factors to change would be expected to alter VO2 kinetics. To determine whether measurement of VO2 kinetics can detect conditions in which the pulmonary blood flow response to exercise is abnormal, VO2 was measured, breath-by-breath, during the transition from rest to exercise in 13 adults with cyanotic congenital heart disease (central venoarterial shunting) and in nine normal subjects. The increase in VO2 above baseline during the first 20 sec of exercise (phase I), reflecting the immediate increase in pulmonary blood flow, was diminished in the patients compared with that in normal subjects (14.8 +/- 10.9 vs. 49.8 +/- 19.2 ml of oxygen) (p less than .001). The patients&...
Pulmonary Function and Ventilatory Limitation to Exercise in Congenital Heart Disease
Congenital Heart Disease, 2009
Pulmonary function in older children and adolescents following surgical repair of congenital heart disease is often abnormal for various reasons. Many of these patients report symptoms of exercise intolerance although the reason(s) for this symptom can be complicated and sometimes interrelated. Is it simply deconditioning due to inactive lifestyle, chronotropic or inotropic insufficiency? or could there indeed be ventilatory limitation to exercise? These are the questions facing the clinician with the increasing frequency of patients undergoing repair early in life and growing into adulthood. Understanding pulmonary functional outcomes and means of determining ventilatory limitation to exercise is essential to thoroughly address the problem. This article reviews pulmonary function in patients with congenital heart disease and then describes a newer technique that should be applied to determine ventilatory limitation to exercise. This was not a systematic review of the literature. Articles were gleaned from the scant literature available, and their results were summarized in table form. References in each manuscript were checked to garner additional data. The aim was to review reports of PF in school-aged children and adults, i.e., patients capable of performing conventional spirometry. Thus, data pertaining to PF tests in infants or to gas exchange (e.g., lung diffusing capacity for carbon monoxide) were not 2
Pediatric Pulmonology, 2020
AimsCardiac surgery has improved life expectancy of patients with congenital heart diseases (CHDs). Exercise capacity is an important determinant of survival in patients with CHDs. There is a lack of studies focusing on the role of resting respiratory performance in reducing exercise tolerance in these patients.ObjectivesTo determine the prevalence and severity of respiratory functional impairment in different types of corrected/palliated CHDs, and its impact on an exercise test.Materials and MethodsRetrospective single‐center study involving 168 corrected/palliated patients with CHD and 52 controls. Patients CHD were divided into subgroups according to the presence of native pulmonary blood flow or total cavopulmonary connection (TCPC). All subjects performed complete pulmonary function tests and gas diffusion; patients with CHD also performed cardiopulmonary exercise test (CPX).ResultsMean values of lung volumes were within the normal range in all CHD groups. Comparing to controls...
American Heart Journal, 2007
Background 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.
Heart, 2000
Objective-To assess the relation between exercise intensity and oxygen uptake during graded exercise in paediatric patients who underwent surgical repair of congenital heart disease, and to compare it with conventional measures of aerobic exercise function. Design-Cross sectional study. Exercise testing was performed on a treadmill and gas exchange was measured on a breath by breath basis. Patients-29 patients who underwent an atrial switch operation for transposition of the great arteries (TGA) (mean (SD) age at testing 10.3 (2.5) years) and 30 patients who underwent total repair of tetralogy of Fallot (TF) (age 12.1 (3.3) years) performed graded exercise testing. Exercise responses were compared with data obtained in 24 normal controls (age 11.4 (2.6) years). Results-The slope of oxygen uptake versus exercise intensity averaged 1.50 (0.64) ml O 2 /min 2 /kg in the patients with TGA and 1.68 (0.75) ml O 2 /min 2 /kg after TF repair, both lower (p < 0.005) than in normal controls (2.42 (0.68) ml O 2 /min 2 /kg). The lower slope of oxygen uptake was correlated with a subnormal value for ventilatory anaerobic threshold, which averaged 78.0 (13.3)% of normal in TGA and 85.1 (10.6)% in TF. This was associated with a steeper slope (p = 0.001) of carbon dioxide output versus oxygen uptake above the ventilatory anaerobic threshold in TGA (1.26 (0.20)) and TF (1.20 (0.18)) compared with the normal controls (1.05 (0.13)), and also a steeper slope of ventilation versus carbon dioxide in TGA (47.0 (15.4)) and TF (41.5 (13.7)) than in the controls (30.3 (8.5)). Conclusions-Calculation of the steepness of the slope of oxygen uptake versus exercise intensity is a valid measurement of oxygen flow to the exercising tissues, which may be limited in congenital heart disease.
Journal of Cardiac Failure, 2004
Aims In chronic heart failure (CHF) patients, the ventilation (VE) needed to eliminate metabolically produced CO 2 during exercise (i.e. the VE/VCO 2 slope) is a strong prognosticator. VE/VCO 2 slope determinants are the dead space-tidal volume (VD/VT) ratio and the arterial CO 2 partial pressure (PaCO 2 ). We aimed at defining the respective prognostic role of these two variables. Methods and results One hundred and twenty-eight stable CHF patients (average left ventricular ejection fraction 34 + 10%) underwent cardiopulmonary exercise testing and blood gas analysis. The prognostic relevance of the VE/VCO 2 slope, VD/VT, and PaCO 2 at peak exercise was evaluated by the Kaplan-Meier approach with log-rank testing and by multivariate Cox regression analysis. During a mean period of 31.3 + 20 months, 24 patients died from cardiac causes. In univariate analysis, predictors of death included the use of anti-aldosterone drugs, low peak VO 2 , peak VE/VO 2 , peak PaCO 2 and high VE/VCO 2 slope, and peak VD/VT. Multivariate analysis identified a low peak PaCO 2 (,35 mmHg) as the strongest independent prognostic indicator [hazard ratio 4.65, 95% confidence interval (CI) (1.695 2 12.751), P ¼ 0.003] that primarily accounts for the VE/VCO 2 slope prognostic power. Conclusion These findings imply that regulatory mechanisms involved in the tight control of ventilatory command and blood gas tension, rather than lung function abnormalities, play a critical pathophysiological role in the exercise ventilation inefficiency of CHF patients.
Current Respiratory Medicine Reviews, 2011
Cardiopulmonary exercise testing (CPET) in pediatric patients differs in many aspects from the tests as performed in adults. Children's cardiopulmonary responses during exercise testing present different characteristics, particularly indices of respiratory gas exchange (e.g. oxygen uptake, ventilation and ventilatory efficiency), which are essential in interpreting hemodynamic data. Diseases that are associated with myocardial ischemia are very rare in children. Important indications for CPET in children are the evaluation of exercise capacity and the non-invasive identification of pathologic features. In this article we will review the methodology, and clinical concepts exercise testing and interpretation of respiratory gas-exchange during exercise in children with congenital heart disease.