The eisenmenger syndrome: A clinical and physiologic reappraisal

Abstract

Ninety-one cases of congenital heart disease with bidirectional or reversed central shunt and high total pulmonary vascular resistance are presented. The clinical examination including history, physical examination, electrocardiogram and chest roentgenogram is of little help in determining the site of the shunt. Cardiac catheterization aids in localizing the lesion, but a second defect may be present even though 1 defect has been clearly demonstrated. Careful follow-up examinations are therefore necessary, and postoperative catheterizations mandatory. Routine use of tolazoline hydrochloride is indicated to assess reactivity of the pulmonary vascular bed, and no physiologic study is considered complete without this procedure. Management is predicated on the reactivity of the pulmonary vascular bed, and it is stressed that the term Eisenmenger syndrome does not necessarily connote inoperability. Surgery, either correction of the defect or pulmonary arterial banding, is indicated in the patient whose level of total pulmonary resistance falls to normal or near normal with tolazoline. Supportive medical management is provided for patients with fixed pulmonary vascular changes.