Cardiopulmonary fitness assessment on maximal and submaximal exercise testing in patients with Fabry disease (original) (raw)
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Cardiac performance during exercise in patients with Fabry's disease
European Journal of Clinical Investigation, 2008
Background Fatigability and dyspnoea on effort are present in many patients with Fabry's disease. We assessed the determinants of cardiac performance during exercise in patients with Fabry's disease and preserved left ventricular ejection fraction at rest. Materials and methods Sixteen patients with Fabry's disease and 16 control subjects underwent radionuclide angiography at rest and during exercise, tissue Doppler echocardiography and magnetic resonance imaging at rest. Results The exercise-induced change in stroke volume was +25 ± 14% in controls and +5•8 ± 19% in patients with Fabry's disease (P < 0•001). In 10 patients (group 1), the stroke volume increased (+19 ± 10%), and in 6 patients (group 2) it decreased (-16 ± 9%) with exercise. Patients of group 2 were older, had worse renal function, higher left ventricular mass and impaired diastolic function compared to group 1. The abnormal stroke volume response to exercise in group 2 was associated with a decrease in end-diastolic volume (P < 0•001) and a lack of reduction of end-systolic volume (P < 0•01) compared with both controls and group 1. The ratio of peak earlydiastolic velocity from mitral filling to peak early-diastolic mitral annulus velocity was the only independent predictor of exercise-induced change in stroke volume (B-0•44; SE 0•119; β-0•70; P < 0•005). Conclusions The majority of patients with Fabry's disease were able to augment stroke volume during exercise by increasing end-diastolic volume, whereas patients with more advanced cardiac involvement may experience the inability to increase cardiac output by the Frank Starling mechanism.
Cardiopulmonary Exercise Testing in Patients with Fabry Disease
Heart, Lung and Circulation, 2009
Objectives: To investigate the exercise performance and cardiorespiratory efficiency in patients with liver cirrhosis. Background: Liver cirrhosis is a very common disease in Egypt especially post hepatitis type, it affects the quality of life and daily performance. It is associated with hypoxemia and impaired oxygen consumption. Methods: This study was conducted on 30 patients of liver cirrhosis and 10 healthy subjects (control group), they were selected from the Menoufia University Hospital. All subjects were subjected to clinical history, clinical examination, plain chest-X-ray, blood sample for: complete blood picture, liver function tests, prothrombin time, abdominal ultrasonography and pulmonary function tests (spirometry) and cardiopulmonary exercise tests. Results: The results of this study revealed a significant reduction of most parameters of exercise test and pulmonary function tests in cirrhotic group versus the control group. Prevalence of exercise impairment increased with the increase of the severity of liver cirrhosis. Conclusion: (1) Cirrhotic patients have impaired exercise performance. (2) Prevalence of exercise impairment increased with the increase of the severity of liver cirrhosis.
Cardiopulmonary exercise tests in rare cardiovascular heart diseases
Journal of Rare Cardiovascular Diseases, 2015
Cardiopulmonary exercise testing (CPET) is commonly used in clinical practice for both functional and diagnostic assessments of patients with cardiovascular and pulmonary disease. It provides assessment of the integrative exercise responses involving the pulmonary, cardiovascular and skeletal muscle systems, which are not adequately reflected through the measurement of individual organ system function. In a group of patients with a congenital heart disease or pulmonary hypertension assessment of exercise capacity and exercise tolerance can be a long term evaluation of treatment efficacy. It is also an objective diagnostic and prognostic tool of exercise capacity that allows to evaluate full actual physical condition of this population. JRCD 2015; 2 (5): 139-143
Cardiovascular Events in Patients With Fabry Disease
Journal of the American College of Cardiology, 2011
These analyses were designed to determine the incidence of major cardiovascular (CV) events and the natural history of CV complications in patients with Fabry disease.
European Journal of Cardiovascular Prevention & Rehabilitation, 2009
Cardiopulmonary exercise testing (CPET) is a methodology that has profoundly affected the approach to patients' functional evaluation, linking performance and physiological parameters to the underlying metabolic substratum and providing highly reproducible exercise capacity descriptors. This study provides professionals with an up-to-date review of the rationale sustaining the use of CPET for functional evaluation of cardiac patients in both the clinical and research settings, describing parameters obtainable either from ramp incremental or step constant-power CPET and illustrating the wealth of information obtainable through an experienced use of this powerful tool. The choice of parameters to be measured will depend on the specific goals of functional evaluation in the individual patient, namely, exercise tolerance assessment, training prescription, treatment efficacy evaluation, and/or investigation of exercise-induced adaptations of the oxygen transport/utilization system. The full potentialities of CPET in the clinical and research setting still remain largely underused and strong efforts are recommended to promote a more widespread use of CPET in the functional evaluation of cardiac patients. Eur J Cardiovasc Prev