17. Shone JD, Sellers RD, Anderson RC, Adams P Jr, Lillihei CW, Edwards JE: The developmental complex of "parachute mitral valve," supravalvar ring of the left atrium, subaortic stenosis and coarctation of the aorta. Am J Cardiol 11: 714, 1963 18. Ellison RC, Restieaux NJ: Vectorcardiography in Congenital Heart Disease. Philadelphia, W.B. Saunders Co, 1972, p 196 19. Heath D, Edwards JE: The pa...

Williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the sy...

The recent rapid development of cardiac surgery now demands accurate diagnosis of the rarer types of congenital heart disease; the exact anatomy of congenital valve stenosis, for example, must be determined. The occurrence of valvar, subvalvar, and supravalvar forms of either aortic or pulmonary stenosis is well known. With respect to congenital mitral stenosis, a rarer cardiac anomaly, we are ...

The recent rapid development of cardiac surgery now demands accurate diagnosis of the rarer types of congenital heart disease; the exact anatomy of congenital valve stenosis, for example, must be determined. The occurrence of valvar, subvalvar, and supravalvar forms of either aortic or pulmonary stenosis is well known. With respect to congenital mitral stenosis, a rarer cardiac anomaly, we are ...

Of 3,022 patients with congenital heart disease studied in the heart clinic of the Royal Liverpool Children's Hospital during the last five years, 120 presented with signs of uncomplicated aortic stenosis giving an incidence of 4% of all cases of congenital heart disease. Swan, Wilkinson, and Blount (1958) noted an incidence of 5% of all patients in their congenital heart disease clinic. It has...

A youth of 16 years of age died suddenly and quite unexpectedly while walking to school. The necropsy disclosed a quadricuspid aortic valve with complete isolation of the left coronary artery by an adherent aortic valve cusp. The left ventricular myocardium showed subendocardial contraction band necrosis suggesting that critical ischaemia had triggered a state of hypercontraction, in keeping wi...

AIMS Williams syndrome (WS) is a microdeletion syndrome affecting cardiovascular and connective tissue as well as the endocrine and central nervous systems in 1 in 10,000 live births. This study aims to identify and evaluate cardiovascular abnormalities (CVAs) in 45 WS patients. PATIENTS AND METHODS We retrospectively reviewed a cohort of WS patients who were followed at our institution from ...

Cardiovascular malformations in patients with a Turner's phenotype and a normal chromosomal karyotype (Noonan's syndrome) have been reported in several recent publications (Noonan and Ehmke, I963; Chaves-Carballo and Hayles, I966; Celermajer, Bowdler, and Cohen, I968; Noonan, I968). Congenital heart disease is present in 48 per cent (Smith, I970) of the patients, the most common abnormality bei...