aly characterized by the absence of the pulmonary valve. The absence of the pulmonary valve, along with the hypoplasia of the pulmonary annulus, leads to aneurysmal dilatation of the pulmonary artery and its branches. Other consequences include pulmonary stenosis and pulmonary insufficiency (1). The 3-day-old baby presented with cyanosis and tachypnea. He had to and fro murmurs at the upper lef...

CXCR7 (RDC1), a G-protein-coupled receptor with conserved motifs characteristic of chemokine receptors, is enriched in endocardial and cushion mesenchymal cells in developing hearts, but its function is unclear. Cxcr7 germline deletion resulted in perinatal lethality with complete penetrance. Mutant embryos exhibited aortic and pulmonary valve stenosis due to semilunar valve thickening, with oc...

in this study, trans-pulmonic valvular gradient of 51 patients with congenital pulmonary stenosis measured by cw-doppler and cardiac catheterization were compared to find whether they are comparable and if doppler method is accurate enough for interventional decision-making. results showed that findings in both methods were comparable and doppler method is accurate and enough for clinical decis...

Critical congenital pulmonary stenosis with intact interventricular septum is a cyanotic and potentially lethal neonatal cardiac anomaly. Percutaneous transcatheter balloon valvuloplasty for critical often presents technical difficulties due to supra-systemic pressure in right ventricle. Using transjugular approach instead of the femoral vein helps dealing this issue. We describe successful use...

Twenty two children (age range 4 months-20 years) with pulmonary valve stenosis and ten children (age range 1 month-11 years) with coarctation of the aorta underwent balloon dilatation in the 29 month period between October 1983 and February 1986. Number 5-9 French catheters with 5-20 mm balloons were used according to the size of the angiographically measured pulmonary valve annulus or coarcte...

Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitr...

T he number of adults in North America who have congenital heart disease (CHD) has steadily increased over the past 30 years. Although this increase is partly because of the growth of the adult population, it may be more attributable to improvements in surgical management, which have resulted in a greater than 90 percent 10-year survival rate in persons with CHD. Between 1979 and 1997, mortalit...