Abstract

Congenital heart defects arise in approximately 1% of all live births, independent of ethnic and geographical considerations. With the development of new surgical procedures and current technologies a large number of these heart lesions can be surgically corrected in infancy. In the majority of cases patients evaluated some 10 to 20 years after surgery are asymptomatic and can lead a normal life. Despite their satisfactory clinical outcome patients may, nevertheless, show an abnormal pattern of physiological responses when submitted to dynamic exercise. This paper reviews the scientific literature concerning the exercise capabilities and the cardiorespiratory adjustments to exercise in patients surgically corrected for 4 of the most common congenital heart lesions: isolated atrial septal defect, isolated ventricular septal defects, pulmonary stenosis and tetralogy of Fallot. The maximal exercise tolerance of postoperative congenital heart defect patients may usually be related to: (a) the age of the patients at the time of surgery; (b) the severity of the lesions remaining after surgery; and (c) the age of the patients at the time of investigation. Although normal maximal exercise capabilities may be found in a good number of patients operated for either of the 4 lesions considered, this does not imply normal exercise haemodynamics. A general observation made in these 4 groups of patients is that of a subnormal exercise cardiac output which may or may not be fully compensated by an increase in peripheral oxygen extraction. The limitation in exercising cardiac output may, in turn, be attributed to either a subnormal stroke volume or a limitation in the chronotropic response to exercise or a combination of both factors. Residual pulmonary stenosis, increased pulmonary vascular resistance, increased myocardial stiffness are all factors that may contribute to the cardiac output limitation. A thorough explanation of underlying causes for the abnormal haemodynamic response to exercise, however, still remains to be provided.

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