Part of the book: Advances in the Diagnosis of Coronary Atherosclerosis
Bicuspid aortic valve (BAV) is one of the most common congenital diseases, affecting 1–2% of the general population. Although most of them are sporadic, some familial cases have also been detected. BAV is a complex developmental and progressive pathology, which may present with various clinical findings from newborn to adulthood. It may be suspected during cardiac auscultation or may be diagnosed by echocardiography incidentally. Some BAV cases may remain symptomless for years, with findings like valvular stenosis, insufficiency, or dilatation in the ascending aorta, whereas some others may present with early severe aortic valve dysfunction, premature congestive heart failure, and aortic aneurysms even in the newborn period. Such heterogeneous presentations of BAV phenotypes may be associated with congenital, genetic, and/or connective tissue abnormalities. The natural course of BAV is nonpredictable, it may lead to severe morbidity and mortality.
Part of the book: Structural Insufficiency Anomalies in Cardiac Valves
Ebstein’s anomaly (EA), a rare congenital heart disease, results from the failure of delamination of tricuspid valve (TV) leaflets from the endocardium of the right ventricle (RV) and apical displacement, particularly of the septal and posterior leaflets of TV. The most commonly accompanying cardiac malformation is atrial septal defect. Most EA cases are sporadic; familial ones are rare. EA patients may present at any age. Symptoms result from TV regurgitation, RV dysfunction, inadequate left ventricular filling owing to ventricular septal bowing, inadequate pulmonary flow, and arrhythmias. Atrial tachyarrhythmias are the most common late complications. There have been more techniques of tricuspid repair reported in the literature than any other congenital or acquired cardiac lesion. Neonatal operation has a higher risk of mortality than the operations performed beyond infancy. Late survival rate and the quality of life for hospital survivors are excellent.
Part of the book: Structural Insufficiency Anomalies in Cardiac Valves