[Angiocardiographic and vectorcardiographic findings in Noonan's syndrome (author's transl)]

Abstract

The angiocardiographic and vectorcardiographic findings in 18 patients with Noonan's syndrome are presented. The excentric hypertrophy of the left ventricle affecting the superior portion of the anterior wall, the posteroinferior portion and the septum was primarily investigated. The predominant lesion was left ventricular deformity. In one case only a hemodynamic burden was caused by the excentric hypertrophy. It could be demonstrated that angiocardiographically and vectorcardiographically differentiation between idiopathic hypertrophic subaortic stenosis and excentric hypertrophy in Noonan's syndrome is impossible. A good correlation however, existed between the morphological structures and the angiocardiographic aspect as well as the vectorcardiographic findings. If hemodynamic burden of the left ventricle is present, identical therapy is proposed for Noonan's syndrome as for idiopathic hypertrophic subaortic stenosis. Left heart catheterization is therefore indicated to prove excentric hypertrophy.