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The pathophysiology of TOF together with pulmonary arteriosus is uniquely attributable to defects of the pulmonary arteries. Even after operative care, these patients remain at higher risk for pulmonary arterial stenoses and pulmonary hypertension .
The condition has been called a severe form of Tetralogy of Fallot. [ 18 ] [ 19 ] [ 9 ] [ 20 ] [ 21 ] [ 12 ] [ 11 ] If deformed blood vessels coming from the thoracic aorta appear alongside this condition, the phenotype is renamed to pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals.
The most common cause of right-to-left shunt is the Tetralogy of Fallot, a congenital cardiac anomaly characterized by four co-existing heart defects. Pulmonary stenosis (narrowing of the pulmonary valve and outflow tract, obstructing blood flow from the right ventricle to the pulmonary artery)
Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension [1] [2] and eventual reversal of the shunt into a cyanotic right-to-left shunt.
Ventricular septal defect (VSD), atrial septal defect (ASD), and tetralogy of Fallot (ToF) are the most common congenital heart defects seen in the VACTERL association. [19] Less common defects in the association are persistent truncus arteriosus and transposition of the great arteries .
The severity of symptoms depends on the type of TGV, and the type and size of other heart defects that may be present (ventricular septal defect, atrial septal defect, or patent ductus arteriosus). Most babies with TGA have blue skin color (cyanosis) in the first hours or days of their lives, since dextro-TGA is the more common type.
The most typical form of APVS is a tetralogy of Fallot variant, [4] however, case studies have linked APVS to several different congenital cardiac syndromes, such as agenesis of ductus arteriosus, [5] persistent ductus arteriosus, [6] atrioventricular septal defect, [7] pulmonary branching abnormalities, [8] [9] transposition of the great arteries, [10] and type B interrupted aortic arch. [11]
Tetralogy of Fallot is actually the most common cause of cyanotic congenital heart defects, accounting for about 50-70%, as well as about 10% of all congenital heart defects in general. Now, it’s not quite clear why some babies develop TOF but it is associated with chromosome 22 deletions and DiGeorge syndrome.