Adults with Tetralogy of Fallot - Repaired, Yes; Cured, No
The New England Journal of Medicine - August 26, 1993 - Vol. 329, No. 9 (Editorial)
[for Professionals mainly]
There are approximately 500,000 adults with congenital heart disease in the United States, and each year another 10,000 children who have undergone surgical repair reach adulthood (1)
Largest diagnostic category among patients undergoing repair is isolated ventricular septal defect, followed by tetralogy of Fallot
The 32-year actuarial survival rate of 86 percent contrasts with the 52 percent cumulative survival for all patients born with tetralogy of Fallot who were initially evaluated during approximately the same period (1952 through 1963)
Preliminary results indicate that repair in infancy substantially improves cumulative survival, reduces the frequency of complications, and enhances the quality of life
Majority of adults with repaired tetralogy of Fallot are physically and emotionally well, a substantial number have residual problems and sequelae that require medical or surgical therapy and continued close surveillance
Major cardiac problems in adults with repaired tetralogy of Fallot are rhythm and conduction disorders
The most troublesome late complications are the rhythm and conduction disorders
Tetralogy of Fallot
Mayo Clinic Rochester
[Support Groups]
... First described by a French physician, Dr. Etienne Fallot, in 1888. There are about 3000 babies born each year in the United States with Tetralogy of Fallot accounting for 10% of all congenital heart defects ...
Tetralogy of Fallot
Victor A. McKusick, OMIM, Johns Hopkins University, January 23, 2008
Digilio et al. (1997) calculated empiric risk figures for recurrence of isolated tetralogy of Fallot in families after exclusion of del (22q11). The investigation covered relatives of 102 patients. Their results showed that the frequency of congenital heart defect was 3% in sibs, 0.5% in parents, 0.3% in grandparents, 0.2% in uncles or aunts, and 0.6% in first cousins
Tetralogy of Fallot
Anne J. L. Chun, M.D., MedLine Plus, June 27, 2006
Prenatal factors that
Include maternal rubella or other viral illnesses during pregnancy, poor prenatal nutrition, maternal alcoholism, mother over 40 years old, and diabetes. There is a higher incidence of Tetralogy of Fallot in children with Down Syndrome
22q11 Deletion Syndrome
Donna M. McDonald-McGinn, MS CGC, Gene Reviews, December 16, 2005
Includes: Shprintzen Syndrome, DiGeorge Syndrome (DGS), Velocardiofacial Syndrome (VCFS)
Individuals with 22q11 deletion syndrome (del 22q11) have a range of findings, including congenital heart disease (74% of patients), particularly conotruncal malformations (tetralogy of Fallot, interrupted aortic arch, and truncus arteriosus); palatal abnormalities (69%), particularly velopharyngeal incompetence (VPI), submucosal cleft palate, and cleft palate; characteristic facial features (present in the majority of individuals); and learning difficulties (70 - 90%). Hypocalcemia and immune deficiency are typical but less common
Del 22q11 is diagnosed in individuals with
In situ hybridization (FISH) using DNA probes from the DiGeorge chromosomal region (DGCR)
Del 22q11 is inherited as a deletion syndrome. About 94% of probands have a de novo deletion of 22q11 and 6% have inherited the 22q11 deletion from a parent. Prenatal testing is possible for fetuses determined to be at 50% risk by family history
Tetralogia de Fallot no Neonato
Edmar Atik, São Paulo, Arquivos Brasileiros de Cardiologia 83(4):, 2004 (Editorial)
[for Professionals mainly] [Portuguese]
Os avanços do manejo clinico-cirúrgico da tetralogia de Fallot resultaram de medidas terapêuticas mais apropriaadas e, principalmente, da progressiva diminuição da época da correção operatória, hoje factível até no periodo neonatal
Tetralogy of Fallot
I.B.I.S. Birth Defects, February 5, 2002
[Ukrainian]
Factsheet in Ukrainian
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Last Updated: 2008/3/3
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