De volwassen patiënt met een tetralogie van Fallot: Behandeling van restletsels

Almost all adult patients with a tetralogy of Fallot have been operated upon in infancy or childhood. With the surgical technique that was most widely used - a transannular patch for the relief of the right ventricular outflow tract obstruction - a severe pulmonary regurgitation developed unavoidably. Until adolescent life even a severe pulmonary regurgitation is often tolerated remarkably well, probably as a result of a good systolic function of the right ventricle. There is however, an increasing evidence that, when the patient becomes older, the systolic right ventricular function deteriorates, leading to progressive right ventricular dilatation. This in turn will lead to a further decline of the right ventricular function with, eventually, right ventricular failure. Therefore, severe pulmonary regurgitation is considered the most important sequel after childhood repair of tetralogy of Fallot. Pulmonary valve replacement, using a bioprosthesis, is the therapy. The disadvantage of any bioprosthesis is its limited life span, with a mean of 10-15 years. Pulmonary valve replacement early in life will therefore dictate several more pulmonary valve replacements in a lifetime, with a not neglectable cumulative surgical risk. This could constitute an argument in favour of postponing a pulmonary valve replacement as long as possible. However, from recent studies it has become evident that the right ventricular function may not recover after pulmonary valve replacement, when the right ventricular function has deteriorated too much. Therefore, an earlier pulmonary valve replacement is advocated, but the optimal moment - definitely not too late, but also not too early - is still unclear.