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...

OBJECTIVE To determine the incidence and prognosis of congenital aortic valve stenosis in the five Health Districts of Liverpool that make up the Merseyside area. DESIGN The records of the Liverpool Congenital Malformations Registry and the Royal Liverpool Children's Hospital identified 239 patients (155 male, 84 female) born with aortic valve stenosis between 1960 and 1990. Patients were tra...

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.

We report the case of a 16-year-old pregnant patient with severe aortic stenosis and pulmonary congestion clinically uncontrolled, in whom percutaneous balloon aortic valvuloplasty was used as the first choice of treatment in an emergency procedure. The clinical findings, pathophysiology, diagnostic features, and indications for percutaneous treatment are reported. Severe congenital aortic sten...

It has frequently been stated that congenital aortic valve stenosis cannot be distinguished with any confidence from discrete subvalvar aortic stenosis on clinical grounds, and that their separation rests on the results of hmmodynamic investigation and angiocardiography (Nadas, 1957; Ongley et al., 1958; Dotter et al., 1961; Braunwald et al., 1963; Engle and Ehlers, 1964). Our experience has le...

Severe aortic stenosis in pregnancy has been known to have a high maternal and perinatal mortality, documented at 17 and 32%, respectively, in 1978. More recent reviews still indicate significant maternal morbidity and unfavourable fetal outcomes. Severe aortic stenosis with or without symptoms leads the table of ‘high maternal and fetal risk’ in valvular heart disease in pregnancy. We report t...

bicuspid aortic valve is traditionally considered an innocuous congenital anomaly. due to a better and widespread availability of non-invasive imaging techniques, it has come to the fore that 30% of these cases develop complications, viz., valve abnormality (aortic regurgitation and stenosis), and aneurysm of aortic root and ascending aorta. sinus of valsalva aneurysm is an uncommon complicatio...

Congenital kinking of the aorta is an uncommon anomaly consisting of elongation of the aortic arch with kinking at the level of the ductal ligament. Herein we report a case of congenital kinking of the aorta with calcified aortic valve stenosis. The combination of a kinked aorta with severe calcified valve stenosis is very unusual.

Various surgical techniques have been described to repair congenital supravalvular aortic stenosis, but the best technique is still controversial. The three-patch repair reconstructs a symmetric aortic root and is supposed to restore a normal physiology. We present two successfully operated patients who had congenital supravalvular aortic stenosis using symmetric three-patch repair. We will dis...