Ellis-Van Creveld syndrome is a very rare congenital disorder which its principal features are polysyndactyly, chondrodysplasia, cardiac abnormalities and ectodermal dysplasia. We report a 10-year-old girl with major manifestations of this syndrome who also had multiple brownish macules and patches on trunk and extremities with aortic and pulmonary stenosis in echocardiographic evaluations.

Quadricuspid aortic valve is a rare congenital condition that occurs not only as an isolated anomaly, but also with other cardiac defects. We describe a 10-year-old boy whose aortic stenosis was diagnosed during infancy. Transthoracic echocardiography revealed dilation of the left ventricle, valvular and subvalvular aortic stenosis, bicuspid aortic valve, aortic regurgitation, and mitral valve ...

there is a paucity of literature regarding iatrogenic aortic valve perforation after cardiac operation is performed in the vicinity of the aortic valve. this report describes the clinical, echocardiographic, and angiocardiographic recognition of iatrogenic aortic valve perforation in a patient who had previously under gone membranous ventricular septal defect and pulmonary stenosis. five days a...

Multiple-lead surface potential maps were compared throughout ventricular activation in nine patients with mild-to-moderate pulmonic stenosis and in 12 patients with mild-to-moderate aortic stenosis. Abnormal patterns of potential distribution were found in aortic stenosis, including three patients without electrocardiographic evidence of left ventricular enlargement. When related to the onset ...

The disease conditions which cause functional change in the aortic valve include aortic regurgitation and aortic stenosis. The aortic sclerosis represents a thickening of the aortic valve, which could progress to the complete stenosis of the aortic valve if not managed. Aortic sclerosis is also considered as one of the markers for coronary atherosclerosis. The treatment involves conservative ma...

OBJECTIVES The objective of this study was to test the effect of a 3-hydroxy-3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitor on the progression of moderate to severe aortic stenosis as measured by echocardiography. BACKGROUND Recent retrospective studies support the hypothesis that statins slow the progression of aortic stenosis. METHODS We performed an open-label, prospective stu...

To examine factors that might affect progression in valvular aortic stenosis (AS), we reviewed serial hemodynamic studies in 50 adult patients. Seven patients had congenital, 22 rheumatic and 21 degenerative-calcific aortic stenosis. The patients with calcific aortic stenosis were older (62 ± 6 vs 51 ± 9 years, p < 0.001) and had onset of murmur later in life. For all patients average values at...

Congenital aortic stenosis is a relatively common disease. It is the one malformation predisposing to sudden death in children. Surgical attempts at correction of critical congenital aortic stenosis have been reported within recent years, but there are no large series with adequate longterm follow up of the post-operative course of children with aortic stenosis. The present report is based on o...

Severe pulmonary or aortic valve stenosis may be lethal to the neonate if untreated. Two patients suffering from pulmonary stenosis and four with aortic stenosis were relieved surgically with the use of full cardiopulmonary bypass. Both neonates with pulmonary stenosis did well, making them the youngest recorded survivors of such treatment. Two of the four patients with aortic stenosis have don...

Transcatheter aortic valve implantation is increasingly being used to treat severe aortic stenosis in patients with high operative risk. In an aging population the incidence of aortic stenosis is rising, and increasing numbers of elderly patients are undergoing aortic valve replacement with bioprosthetic valves. Therefore, there is a corresponding increase in prosthetic degeneration. This prese...