Exercise performance and quality of life is more impaired in Eisenmenger syndrome than in complex cyanotic congenital heart disease with pulmonary stenosis
Introduction
Nowadays, most cyanotic congenital heart defects can be surgically corrected or palliated with a Fontan operation in infancy or early childhood. Nevertheless, there are several patients with cyanotic congenital heart disease who had no surgical management under the surveillance of adult congenital heart units who seem to have a remarkably stable situation. [1] Their survival depends mainly on the balance of pulmonary to systemic blood flow. If pulmonary blood flow is unrestricted, patients suffer from heart failure beginning in infancy after the natural decline of pulmonary vascular resistance, till they develop pulmonary vascular disease resulting in Eisenmenger syndrome (ES). On the other hand, if any kind of pulmonary stenosis (PS) is too extensive, they become severely cyanotic. [1] Survival is best with a balanced pulmonary blood flow. This situation is achieved by a moderate pulmonary stenosis (PS), either genuine or after pulmonary arterial banding (PAB), or by increased pulmonary vascular resistance in ES. All of these patients show diminished oxygen saturation at rest. At exercise, when systemic vascular resistance drops, pulmonary blood flow decreases resulting in severe cyanosis. Exercise capacity displayed in a reduced peak oxygen uptake (peak VO2) and ventilatory inefficiency estimated from the steepness of the V̇E/V̇CO2 slope is the worst seen in the cohort of patients with congenital heart disease [2], [3], [4].
Despite similar symptoms in these two groups, the patho-physiologic background is different. In patients with PS the fixed stenosis in the pulmonary artery increases intracardiac right-to-left shunting during exercise resulting in an increase in cyanosis and ventilatory inefficiency. [5], [6] In ES patients, it is the pulmonary vascular remodelling that causes fibrosis, inhomogeneous perfusion, and increased pulmonary vascular resistance, in addition to their intracardiac right-to-left shunt.
Hence, we aimed to define differences from a single cardiopulmonary exercise test (CPET) between the groups of PS and ES patients and whether they are related to the quality of life.
Section snippets
Study subjects
We retrospectively analyzed all patients aged fourteen years or older with major intra cardiac right-to-left shunts, referred to our exercise laboratory as part of their routine follow-up, with progressive cyanosis under exercise. Patients with major aorto-pulmonary collateral arteries were excluded. All CPET were performed between October 2001 and February 2009.
In total fifty-eight patients (28 male, 30 female, aged 14–55 years) could be studied. Twenty-three of them had hemodynamic relevant
Cardiopulmonary exercise test
CPET could be performed even in these severely symptomatic patients without any incident. Ventilatory threshold could not be determined properly in 18 patients with ES and two patients with PS. Results are shown in Table 1.
Exercise capacity displayed as peak V̇O2 was severely reduced (13.0 ml/min/kg corresponding to 38.6% of age and sex related reference values) and ventilatory efficiency expressed as V̇E/V̇CO2 slope was markedly increased, while PETCO2 was diminished substantially.
Pulse
Discussion
This study showed that patients with cyanotic congenital heart diseases have a severely diminished exercise capacity, ineffective ventilation at rest that even worsens under exercise. Furthermore, they have a curtailed quality of life predominantly in the physical domains. Pulse oxymetric oxygen saturation at rest seems to predict exercise capacity and the functional status of patients in this study cohort, but not quality of life.
In most of the investigated criteria, patients with Eisenmenger
Conclusion
Patients with cyanotic congenital heart disease have diminished exercise capacity, impaired ventilatory efficiency and a reduced quality of life. Moreover, oxygen saturation at rest is an important parameter for cyanotic patients to indicate the functional status of the patient and to predict exercise capacity and ventilation efficiency.
Despite showing similar cyanosis at rest and under exercise and also reporting on similar symptoms like dyspnoea and fatigue, patients with Eisenmenger syndrome
Acknowledgement
The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology. [35]
References (35)
- et al.
Long-term survival of patients with univentricular heart not treated surgically
J Thorac Cardiovasc Surg
(2002) - et al.
Mixed-expired and end-tidal CO2 distinguish between ventilation and perfusion defects during exercise testing in patients with lung and heart diseases
Chest
(2007) German translation and psychometric testing of the SF-36 Health Survey: preliminary results from the IQOLA Project. International quality of life assessment
Soc Sci Med
(1995)- et al.
Assessment of symptoms and exercise capacity in cyanotic patients with congenital heart disease
Chest
(2004) - et al.
Aerobic capacity in adults with various congenital heart diseases
Am J Cardiol
(2001) - et al.
Natural history of exercise capacity after the Fontan operation: a longitudinal study
Ann Thorac Surg
(2008) - et al.
Can maximal cardiopulmonary capacity be recognized by a plateau in oxygen uptake?
Chest
(1989) - et al.
Clinical correlates and prognostic significance of the ventilatory response to exercise in chronic heart failure
J Am Coll Cardiol
(1997) - et al.
Ventilatory efficiency and aerobic capacity predict event-free survival in adults with atrial repair for complete transposition of the great arteries
J Am Coll Cardiol
(2009) Ethical authorship and publishing
Int J Cardiol
(2009)
Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication
Circulation
Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease
Cardiol J
Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival
Circulation
Gas exchange detection of exercise-induced right-to-left shunt in patients with primary pulmonary hypertension
Circulation
Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease
Eur Heart J
Gas exchange response to exercise in children
Am Rev Respir Dis
Exercise testing and interpretation; a practical approach
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