الفهرس | Only 14 pages are availabe for public view |
Abstract Summary Congenital heart disease is present in 0.9% of living births; currently, 90% of those affected will reach adulthood because of recent progress made in pediatrics, cardiology, surgery and resuscitation. Among what are commonly called ‘‘grown-up congenital heart diseases’’ (GUCHs) or, more recently, ‘‘adult congenital heart diseases’’ (ACHDs), tetralogy of Fallot (TOF) has a preponderant place, because of its relatively high prevalence and because it is possible to have corrective surgery. Tetralogy of Fallot (TOF) is a cardiac abnormality that occurs during embryogenesis. TOF has been known as one of the most common causes of cyanotic heart disease during first ages of life that occurs in 3 of every 10,000 live births and constitutes approximately 7%–10% of all congenital defects. It is one of the most common causes of cyanotic heart disease beyond the neonatal age. Males are affected slightly more often than females. The etiology is multifactorial and has been associated with maternal intake of retinoic acid, untreated maternal diabetes, and phenylketonuria. chromosomal anomalies including trisomies 21, 18, and 13 have also been associated with TOF. More frequent, however, are microdeletions of chromosome 22. There is also an association between Alagille syndrome with JAG1mutations and TOF. The risk of death in the first year of life is very high, after the first year it is constant until age 25 years, and then increases |