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...

Patients who develop a severe stenosis in biological pulmonary conduits previously implanted for pulmonary outflow trunk reconstructions are treated either by surgical re-replacement, or by transcatheter stent-valve implantation through a femoral vein access. A catheter-based sub-xyphoidian access through the right ventricle for stent-valve positioning in a pulmonary conduit has rarely been pro...

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...

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...

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...

Patients with rheumatic mitral stenosis often have no pulmonary oedema despite considerably increased pulmonary venous pressure. Pulmonary microvascular permeability was measured non-invasively by a previously validated method of double isotope scintigraphy with indium-113m and technetium-99m. This permits calculation of an index reflecting transferrin efflux and thus, indirectly, the microvasc...

Pulmonary hypertension frequently complicates mitral stenosis. Increased pulmonary artery pressure results from raised left atrial pressure, pulmonary arteriolar constriction, and obliterative changes in the pulmonary vascular bed, and usually responds to surgical relief of mitral stenosis. However, severe pulmonary hypertension may persist after surgical treatment of mitral stenosis. We descri...

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...