The bicuspid aortic valve is known to be the most common congenital cardiac malformation, with an approximate incidence rate of 1-2% in the general population. Most patients are unaware of the disease until the onset of infective endocarditis, which is a life-threatening complication that may affect a heart valve or other cardiac structures at the site of endothelial damage. A 22-year-old man p...

The use of antiepileptic drugs increases the risk of major congenital malformations during pregnancy. Here, we report an infant who had a history of in-utero carbamazepine exposure and who was born with a cardiac malformation. The infant was born at 39 weeks of gestation vaginally to an epileptic mother who had been treated with carbamazepine throughout her pregnancy. He was referred due to car...

OBJECTIVE To assess the pattern and progression of aortic valve dysfunction by serial Doppler echocardiographic examinations in ambulatory adult patients with congenital bicuspid aortic valve. DESIGN AND SETTING Retrospective analysis of patients referred for Doppler echocardiography over a four year period. SUBJECTS Fifty one adult patients with echocardiographic diagnosis of congenital bi...

Congenital aortic valve anomalies are the cause of premature aortic stenosis in pediatric and younger adult populations. Despite being very rare, unicuspid aortic valves account for approximately 5% of isolated aortic valve replacements. Patients with aortic stenosis, present with the same symptomatology independent of leaflet morphology. However, the presence of bicuspid and unicuspid aortic s...

Bicuspid aortic valves calcify at a significantly higher rate than normal aortic valves, a process that involves increased inflammation. Because we have previously found that bicuspid aortic valve experience greater stretch, we investigated the potential connection between stretch and inflammation in human aortic valve interstitial cells (AVICs). Microarray, quantitative PCR (qPCR), and protein...

BACKGROUND Bicuspid aortic valve regurgitation can be caused by a defect in the valve itself or by dysfunction of one or more components of the aortic root complex. A successful repair thus requires correction of all aspects of the problem simultaneously. We review our experience addressing both the valve and the aortic root when correcting bicuspid valve regurgitation. METHODS AND RESULTS Be...

A young male presented with multiple xanthomas and xanthelesmas, progressive breathlessness and angina on exertion. Investigations confirmed diagnosis of familial hypercholesterolaemia, bicuspid aortic valve with severe aortic stenosis and significant obstructive coronary artery disease.

BACKGROUND Hereditary spherocytosis is a genetic, frequently familial hemolytic blood disease characterized by varying degrees of hemolytic anemia, splenomegaly, and jaundice. There are few reports on adult open-heart surgery for patients with hereditary spherocytosis. CASE PRESENTATION We report a rare case of an adult open-heart surgery associated with hereditary spherocytosis. A 63-year-ol...

A 22-year-old male patient was admitted to our institution because of repeated chest pain and syncope. Physical examination was unremarkable except for a systolic murmur on cardiac auscultation. Transesophageal echocardiogram showed a large, very mobile cystic mass (21×17 mm) attaching to the bicuspid aortic valve, which caused severe left ventricular outflow tract obstruction with normal ventr...

Background: Bicuspid aortic valve (BAV) is the most frequent congenital heart disease affecting 1% to 2% of population, with thoracic aneurysm (TAA) formation being its second complication after dysfunction (stenosis or insufficiency). Aortic replacement (AVR) mechanical biologic prostheses has long been procedure choice. Although prosthetic AVR effective in correcting hemodynamic problem, ther...