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.

OBJECTIVE This article aims to present updated therapeutic options for fetal congenital heart diseases. METHODS Data source for the present study was based on comprehensive literature retrieval on fetal cardiac interventions in terms of indications, technical approaches and clinical outcomes. RESULTS About 5% of fetal congenital heart diseases are critical and timely intrauterine interventi...

OBJECTIVES The aim of the study was to analyze the long-term results of congenital subvalvar aortic stenosis (SAS) relief and the risk factors associated with recurrence and reoperations. METHODS Between January 1960 and March 2005, 190 patients underwent surgical correction for discrete (n=140) and tunnel (n=50) congenital subaortic stenosis. There were 115 male and 75 female patients rangin...

BACKGROUND Pulmonary valve stenosis (PVS) is one of the most important acyanotic congenital heart diseases in children. Over the last years, balloon pulmonary valvuloplasty (BPV) has become the treatment of choice for the relief of moderate to severe valvular pulmonic stenosis in all age groups. The aim of current study was to report our regional survey on the short term outcomes of BPV among p...

Eight patients with mild to moderate valvular pulmonary stenosis underwent serial physiological studies before surgical intervention. Average pulmonary valve area at the initial study was 0.59 cm.2 and at the second study (average 7.8 years later) was 1.09 cm.2, resulting in an average increase of 0.49 cm.2 (p greater than 0.05). In normal subjects during early years the pulmonary valve area in...

Balloon dilatation in infants with severe pulmonary valve stenosis may not be a straightforward procedure once the arterial duct has closed. Balloon dilatation was attempted in three neonates and infants. In an 11 week old infant hypotension and bradycardia developed shortly after a 5 French end hole catheter was passed through the severely stenosed pulmonary valve. An emergency Waterston shunt...

surgery and to enhance our understanding of the pathophysiology of mitral stenosis. Although surgery produced a significant increase in calculated mitral valve area, the degree of improvement varied markedly. The younger patients had the greater increase in mitral valve area. The change in cardiac index and pulmonary vascular resistance correlated well with the change in mitral valve area. Only...

Congenital pulmonary stenosis with intact ventricular septum is a relatively common malformation of the heart. The stenosis varies from almost complete obliteration to a minimal decrease in the diameter of the orifice. The stenosis is usually of the valve, but it may be either of the infundibulum or of the lower bulbar orifice. The atrial septum may be complete, imperfectly developed, or it may...

OBJECTIVE To assess the pattern and progression of aortic valve dysfunction by serial Doppler echocardiographic examinations in ambulatory adult patients with congenital bicuspid aortic valve. DESIGN AND SETTING Retrospective analysis of patients referred for Doppler echocardiography over a four year period. SUBJECTS Fifty one adult patients with echocardiographic diagnosis of congenital bi...