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Tetralogy of Fallot (TOF) (Signs and symtomps (The arterial oxygen…
Tetralogy of Fallot (TOF)
Treatment
Oxygen supplement
focuses on determining whether
the child's oxygen levels are in a safe range.
PGE1
a prostaglandin infusion is usually initiated to keep the ductus arteriosus open which will provide additional pulmonary blood flow and increase the child's oxygen level.
Surgery
Corrective repair of TOF
Ventricular septal defect. With significant obstruction in the right ventricular outflow tract, blood will shunt from right to left, bypassing the lungs and leading to cyanosis
Right ventricular hypertrophy (thickened muscle wall) secondary to higher pressure load on this chamber.
Right ventricular outflow obstruction. Typically multi-level: small, malformed pulmonic valve, hypoplastic proximal pulmonary artery, and muscular narrowing below the valve
The aorta "overrides" the VSD.
Muscular right ventricular outflow obstruction has been cut away as part of the repair.
Patch closure of the VSD.
Right ventricular outflow patch to address all levels of obstruction.
Palliative surgery
Blalock-Taussig (BT) shunts
BT shunts, are used for defects that affect the flow of blood from the right ventricle, through the pulmonary artery, and to the lungs.
Shunts are usually used for four to five months, until the child outgrows them and a second operation or definitive repair is needed.
The BT shunt is a palliative procedure. It does not correct the defect, but it helps to resolve symptoms until the child is older and/or the defect itself can be repaired. It is also called the blue baby operation.
Definite
Tetralogy of Fallot (TOF) is a cardiac anomaly that refers to a combination of four related heart defects that commonly occur together. The four defects are
Ventricular septal defect (VSD)
Overriding aorta
Pulmonary stenosis
Right ventricular hypertrophy
Diacnosis of TOF
significant cyanosis is first seen,
often placed in supplemental oxygen
Failure to respond to this "hyperoxia test" is often the first clue to suspect a cyanotic cardiac defect. Infants with tetralogy of Fallot can have normal oxygen levels if the pulmonary stenosis is mild (referred to as "pink" tetralogy of Fallot).
Echocardiography
Cardiac Catheterization
Signs and symtomps
Rapid breathing
response to low oxygen levels and reduced pulmonary blood flow can occur. The heart murmur, which is commonly loud and harsh, is often absent in the first few days of life.
Cyanosis
The degree of cyanosis is proportional to lung blood flow and thus depends upon the degree of narrowing of the outflow tract to the pulmonary arteries
Heart murmur
The most often diagnosed in the first few weeks of life due to either a loud murmur or cyanosis. Babies with tetralogy of Fallot usually have a patent ductus arteriosus at birth that provides additional blood flow to the lungs, so severe cyanosis is rare early after birth.
The arterial oxygen saturation of babies with tetralogy of Fallot can suddenly drop markedly
results from a sudden increased constriction of the outflow tract to the lungs so that pulmonary blood flow is further restricted
A tetralogy spell
A tetralogy spell can sometimes be treated by comforting the infant and flexing the knees forward and upward.