Obstruction to ventricular outflow may be either discrete and fixed, or dynamic and variable. A fixed obstruction is most common at valvar level but occasionally it may be subvalvar or supravalvar. Dynamic or functional obstruction is associated with hypertrophy of ventricular muscle and is invariably subvalvar. This latter type of functional obstruction was first recognized in the right ventri...

transluminal balloon valvuloplasty is an alternative to surgical valvotomy for congenital pulmonary valve stenosis. the aim of this study was to evaluate the long term results (to 13.5 years) of balloon pulmonary valvuloplasty. from june 1998 to january 2012, percutaneous balloon pulmonary valvuloplasty for congenital pulmonary valve stenosis was performed in 98 patients (50 males, 48 females, ...

Williams-Beuren syndrome is a multisystem genetic disorder caused by hemizygous deletion on chromosome 7q11.23, encompassing about 28 genes including the elastin gene, ELN. Cardiovascular abnormalities are frequent and are related to elastin insufficiency. These abnormalities include supravalvular aortic stenosis (SVAS) in 70% of case, pulmonic valve stenosis, and renal artery stenosis. Definit...

conclusions full echocardiographic examination of the right ventricle, including rvot is essential for the diagnosis of this disease. surgical correction appears to be a successful strategy for management of this rare condition in adults. case presentation we report a 40-year-old man who was referred to cardiology clinic for work-up of dyspnea and a heart murmur found on his physical examinatio...

Between 1956 and 1976, 18 patients underwent surgery for supravar aortic stenosis at the The Children's Hospital Medical Center, Boston. Discrete obstruction, present in 11, was treated by insertion of a prosthetic gusset placed across the area of narrowing and extending into the noncoronary sinus of Valsalva. There was one operative death. Residual gradients (measured in five patients) ranged ...

cutis laxa is rare and hetrogenous group of disorders related to abnormalities in elastic tissue. it may be autosomal recessive, autosomal dominant, x linked or acquired. acquired cutis laxa has developed after a febrile illness, inflammatory skin disease such a lupus eryhymatosis or erythema multiform, amyloidosis, hypersensitivity reaction to penicillin and in infants born from women who were...

The authors present a case of echocardiographic diagnosis of supravalvar mitral ring (a fibromembranous structure that arose from the atrial surface of the mitral leaflets) in a child with a parachute mitral valve, a ventricular septal defect, and mild narrowing of the aortic isthmus. The supravalvar mitral stenosis is a typical but very infrequently detected element of the complex of anatomica...

oxygen delivery per unit of TTI was significantly lowered and Endo ECGs showed ischemia (ST elevation) when Endo/Epi flow ratios (microsphere method) fell below 1.0 (P < 0.001). Ischemia occurred without anemia (Hgb > 10 g %) in severe aortic stenosis; with mild anemia (Hgb 5-10 g %) and mild aortic stenosis; and with severe anemia (Hgb < 5 g %) without aortic stenosis. Ischemic Endo ECGs occur...