Williams syndrome is a genetic disorder associated with characteristic facies, supravalvar aortic stenosis, peripheral pulmonary stenosis, mental retardation, hypertension, premature aging of skin, and congenital cardiac defects. Many cardiac defects such as bicuspid aortic valve, mitral valve regurgitation, coarctation of the aorta, and ventricular or atrial septal defects are linked to the sy...

A 56-year-old female with congestive heart failure was transferred to our institution. Aortography demonstrated aortic valve stenosis (AS) with a congenitally bicuspid valve and dilatation of the ascending aorta. Preoperative coronary angiography showed a left single coronary artery. Replacement of the aortic valve and ascending aorta was performed. She had an uneventful postoperative course. W...

The numerous reports appearing in current British, American and French journals testify to the interest aroused by recent advances in cardiac surgery. It may, therefore, be helpful to recall certain landmarks in its history and to review the present status of those procedures which have proved of value. Congenital Heart Disease Although the surgical treatment of pulmonary stenosis is now soundl...

no commissural fusion. The obstructive mechanism is related to markedly thickened, immobile cusps, characterized by the presence of disorganized myxomatous tissue. Several clinical features tend to distinguish this form of pulmonary stenosis. In each of 16 patients studied, a pulmonary ejection murmur was present, but this was not associated with an ejection click. Other features suggestive of ...

We describe the case of a newborn with 22q11 deletion and an unusual form of common arterial trunk with the main pulmonary artery arising anteriorly at the level of the common arterial valve sinus. Case reports: We report the case of a term newborn male who presented to our institution with a prenatal diagnosis of congenital heart disease. His mother was a 29-year-old woman with 22q11 deletion ...

OBJECTIVE Noonan syndrome is a clinically homogeneous but genetically heterogeneous condition. Type 1 Noonan syndrome is defined by the presence of a mutation in the PTPN11 gene, which is found in approximately 40% of the cases. Phenotype descriptions and cardiac defects from cohorts with Noonan syndrome were delineated in the "pregenomic era." We report the heart defects and links to gene dysf...

This report describes 3 cases of pulmonary stenosis in the recipient twin in twin-twin transfusion syndrome. Fetal echocardiography showed cardiomegaly, tricuspid valve regurgitation, and increased reverse flow in the inferior vena cava, as signs of congestive heart failure in all 3 cases. We diagnosed 2 cases of pulmonary stenosis by fetal echocardiography prenatally and confirmed our findings...

Cor triatriatum dexter is a rare congenital malformation in which a membrane divides the right atrium into two chambers. The membrane represents a persistence of the right sinus venosus valve (RSV). Normally the RSV regresses between the 9th and 15th week of gestation, as the cephalic portion forms the crista terminalis and the caudal portion develops into the Eustachian and Thebesian valve. An...

Scope of the Problem The population of children and adults who have undergone successful palliation of various forms of congenital heart disease (CHD) continues to increase and live longer. The reasons are multiple, and include expanded indications, early, more definitive surgery and other interventional techniques, and improved surgical, interventional, catheterization, electrophysiological, a...