Exercise performance and quality of life is more impaired in Eisenmenger syndrome than in complex cyanotic congenital heart disease with pulmonary stenosis

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Abstract

Objective

Patients with cyanotic congenital heart disease without corrective surgery or palliation survive into adulthood, if they have a balanced pulmonary blood flow facilitated by pulmonary stenosis (PS) or Eisenmenger syndrome (ES). Both groups show cyanosis, diminished exercise performance and impaired quality of life. This study aimed to compare the functional outcome of those two cohorts directly.

Patients and methods

In total fifty-eight cyanotic patients with cardiac shunts (28 male, 30 female, aged 14–55 years) were investigated, twenty-three of them with PS and thirty-five of them with ES. They completed the health related quality of life questionnaire SF-36 and performed a symptom limited cardiopulmonary exercise test.

Results

At exercise, oxygen saturation decreased severely and similarly in both groups (PS: 90% to 65% vs. ES: 87 % to 64%). Moreover, hemoglobin levels were comparable in both subgroups. Exercise capacity was markedly reduced, but more diminished in ES (PS: 20.3 (11.9;24.6) ml/min/kg vs. ES: 11.3 (9.7;14.5) ml/min/kg; p < 0.001) and ventilatory inefficiency expressed as E/CO2 slope was more enhanced in ES (PS: 45.7 (37.6;52.9) vs. ES: 54.6 (43.4;68.7); p = 0.005). Oxygen saturation at rest was correlated to peak O2 (r = 0.436; p = 0.001) and E/CO2 slope (r =  0.388; p = 0.003).

Self estimated quality of life was poor, with worse results in physical and psychosocial domains in ES group.

Conclusions

Despite similar cyanosis, patients with ES show less exercise performance, more ventilation–perfusion-mismatch and a worse quality of life compared to complex cyanotic congenital heart disease patients with PS. Moreover, oxygen saturation at rest predicts exercise capacity and ventilatory efficiency in this cohort.

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 E/CO2 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 E/CO2 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]

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