Pulmonary lymphangioleiomyomatosis has been observed almost exclusively in women, usually in their reproductive years. Exacerbations with pregnancy and after hormonal manipulation have been documented, and it has been suggested that its pathogenesis is due to the influence of hormonal(estrogenic) stimulus. The clinical, roentgenographic, and histopathologic features of this case of pulmonary ly...

Supravalvular aortic stenosis is a less common form of left ventricular outflow tract obstruction (LVOTO); commonest being the valvular aortic stenosis followed by valvular and subvalvular forms respectively. Most of the supravalvular aortic stenosis is associated with Williams syndrome; isolated supravalvular aortic stenosis is further rarer. We present a case of isolated SVAS with infective e...

Obstruction to pulmonary blood flow may occur in the pulmonic valve, in the infundibulum or in both. Cardiac catheterization aids in the determination of the site of obstruction. Criteria for the differentiation at operation of valvular and infundibular pulmonic stenosis are enumerated, and the usefulness of accurate pressure tracings during operation is emphasized. The accurate identification ...

Fourteen infants, all under 6 months of age, underwent surgery for the relief of severe valvar pulmonary stenosis. A modified Brock (transinfundibular valvotomy) procedure was performed in all cases. Dilatation of the pulmonary valve with a mosquito clamp and biliary dilator is the basis of the modified operation. All infants survived the operation. In two patients there is residual, significan...

SIGNIFICANT outflow obstruction of both ventricles on a congenital basis is rare. Only 11 cases have been reported1-7 and, in most instances, the stenosis was either valvular or subvalvular on both sides. Supravalvular stenosis occurring in the pulmonary arteries or in the root of the aorta, although uncommon, has been reported frequently in recent years. The association of these two lesions in...

critical pulmonary valve stenosis (cpvs) and atrial septal defect (asd) is an uncommon form of congenital heart disease. concurrent transcatheter pulmonary valvuplasty and closure of secundum atrial septal defect appears to be an interesting alternative to surgical correction. we present the simultaneous balloon valvuloplasty of critical pulmonary stenosis (ps) with supra systemic right ventric...

Six patients with early systolic closure of the aortic valve are described with mitral regurgitation, double outlet right ventricle, left ventricular diverticulum, congestive cardiomyopathy, Eisenmenger ventricular septal defect, and aortic regurgitation with an aneurysmal ascending aorta, respectively. None had evidence of subaortic stenosis. Early sytolic closure of the aortic valve is thus n...