one manifestation of the idiopathic hypercalcaemia of infancy syndrome, and the possible aetiologies are discussed. Another presentation of supravalvar aortic stenosis is in the so-called familialform, but there may exist considerable confusion between these two forms, as less than 50 per cent of those without physical stigmata have a positive family history, and the severeform of the idiopathi...

The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aortic stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal...

The cardiovascular findings in 9 patients with Turner's syndrome and 9 patients with Noonan's syndrome are described. Of the 9 patients with Turner's syndrome, 4 had coarctation of the aorta, 4 aorta stenosis, and the remaining patient both these lesions. All patients with Noonan's syndrome had pulmonary valve stenosis. In addition, 2 children had an atrial septal defect and 1 an atrial septal ...

The results of investigation in 5 children with congenital deformities of the mitral valve region characteristic of the 'parachute mitral valve complex' were reviewed. Three patients had parachute mitral valve, i had supravalvar mitral ring, and i had both these lesions. Associated anomalies included ventricular septal defect, double outlet right ventricle, subaortic stenosis, bicuspid aortic v...

BACKGROUND Supravalvar pulmonary stenosis (SVPS) is frequently observed after arterial switch. Traditionally the coronary arteries are removed from the neopulmonic root by excising the entire sinus of Valsalva. As a result, reconstruction of the neopulmonic root requires a pericardial patch encompassing two-thirds of the anastomosis between the neopulmonic root and pulmonary artery. We present ...

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...

Advances in technology have led to a changing role for MRI in the evaluation of the thoracic vasculature in children. MRI, especially with 3D gadolinium-enhanced MR angiography, can clearly demonstrate the morphology of the aortic and pulmonary vascular supply. In patients with nonvalvar obstructive lesions of the aorta (i.e., coarctation, interruption of the aortic arch, and supravalvar stenos...

Williams syndrome (WS) is a congenital developmental disorder affecting the connective tissue, neurologic, and cardiovascular (CV) systems in 1 in 8,000 live births. The characteristic features of WS, including facial dysmorphisms, cognitive traits, and CV abnormalities, are known to arise from the deletion of approximately 28 genes on chromosome 7q11.23. Structural CV abnormalities occur most ...