[Disorders of the cardiovascular system in Turner's syndrome]

A group of 22 adults with Turner's syndrome, mean age 29.6 years, was subjected to a careful examination by one-dimensional, two-dimensional, pulsed and coloured Doppler echocardiography. The purpose was to assess the incidence and character of congenital and acquired abnormalities of the cardiovascular system which occur within the framework of this defined genetic syndrome. A quite normal echocardiographic finding was recorded in 13 patients, i. e. in 59.1%. In the remainder a wide spectrum of abnormalities was found such as prolapse of the mitral valve (in 13.6%), bicuspid aortal valve with a medium regurgitation (4.5%), hypoplasia of the coronary cusp of the aortal valve (4.5%), dilatation of the ascending aorta with a residual significant stenosis at the site after operation of coarctation of the thoracic aorta (4.5%), subaortal defect of the interventricular septum (4.5%) and slight left ventricular hypertrophy in patients with arterial hypertension (9.1%). Echocardiographic examination in Turner's syndrome makes early diagnosis of abnormalities of the cardiovascular system possible, incl. quantification of the haemodynamic impact. Some of these pathological changes (bicuspid aortal valve, dilatation of the root of the aorta) are for a long time clinically silent but may be nevertheless associated with serious complications. An echographic diagnosis made in time may be of decisive importance for the prevention of complications.