congenitalheartdiseaseskhybermedicaluniversity先天性心脏病开伯尔医学大学.pptxVIP

Congenital Heart Diseases;Congenital heart diseases abnormalities of the heart or great vessels that are present at birth faulty embryogenesis during gestational weeks 3 through 8 major cardiovascular structures develop. Congenital malformations of the heart encompass a broad spectrum of defects, severe anomalies that cause death in the perinatal period, mild lesions that produce only minimal symptoms, even in adult life. Generally accepted incidence is approximately 1% of live births higher in premature infants and in stillborns. Most common type of heart disease among children. ;Patients surviving with congenital heart disease is increasing rapidly Because of clinical advances by 2020 there will be an estimated 750,000 adults with congenital heart disease. Surgery can correct the hemodynamic abnormalities repaired heart may still not be completely normal Although adaptive initially, such changes can elicit late-onset arrhythmias, ischemia, or myocardial dysfunction, sometimes many years after surgery Myocardial hypertrophy and a cardiac remodeling brought about by the congenital defect may be irreversible. ;General concepts regarding the etiology of congenital malformations unknown in almost 90% of cases. Environmental factors, congenital rubella infection, are causal in many instances. Genetic factors are also clearly involved, as evidenced by familial forms of congenital heart disease well-defined associations with certain chromosomal abnormalities (e.g., trisomies 13, 15, 18, and 21 and Turner syndrome). ;Cardiac morphogenesis involves multiple genes tightly regulated to ensure an effective embryonic circulation. Key steps involve specifying cardiac cell fate, morphogenesis and looping of the heart tube, segmentation and growth of the cardiac chambers, cardiac valve formation, and connection of the great vessels to the heart. The molecular pathways controlling such cardiac development provide a foundation for understanding the basis of some