Below are the four heart malformations that present together in tetralogy of Fallot:.
There is anatomic variation between the hearts of individuals with tetralogy of Fallot. Presumably, this arises from an unequal growth of the aorticopulmonary septum aka pulmonary outflow septum. This then prevents ventricular wall closure, therefore VSD, and this increases the pressures on the right side, and so the R ventricle becomes bigger to handle the work.
In addition, tetralogy of Fallot may present with other anatomical anomalies, including:  . Tetralogy of Fallot with pulmonary atresia pseudotruncus arteriosus is a severe variant  in which there is complete obstruction atresia of the right ventricular outflow tract, causing an absence of the pulmonary trunk during embryonic development. The lungs are perfused via extensive collaterals from the systemic arteries, and sometimes also via the ductus arteriosus.
Congenital heart defects are now diagnosed with echocardiography , which is quick, involves no radiation, is very specific, and can be done prenatally. Many patients are diagnosed prenatally. Color Doppler type of echocardiography measures the degree of pulmonary stenosis. Additionally, close monitoring of the ductus arteriosus is done in the neonatal period to ensure that there is adequate blood flow through the pulmonary valve.
In certain cases, coronary artery anatomy cannot be clearly viewed using echocardiogram. In this case, cardiac catheterization can be done. Before more sophisticated techniques became available, chest x-ray was the definitive method of diagnosis. The abnormal " coeur-en-sabot " boot-like appearance of a heart with tetralogy of Fallot is classically visible via chest x-ray, although most infants with tetralogy may not show this finding.
Lung fields are often dark absence of interstitial lung markings due to decreased pulmonary blood flow.
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Electrocardiography shows right ventricular hypertrophy RVH , along with right axis deviation. Tet spells may be treated with beta-blockers such as propranolol , but acute episodes require rapid intervention with morphine or intranasal fentanyl  to reduce ventilatory drive, a vasopressor such as phenylephrine , or norepinephrine to increase systemic vascular resistance, and IV fluids for volume expansion.
This allows more blood flow to the lungs by decreasing shunting of deoxygenated blood from the right to left ventricle through the VSD. There are also simple procedures such as squatting and the knee chest position which increase systemic vascular resistance and decrease right-to-left shunting of deoxygenated blood into the systemic circulation. If the spells are refractory to the above treatments, people are usually intubated and sedated. Total surgical repair of TOF is a curative surgery.
Different techniques can be used in performing TOF repair. However, a transatrial, transpulmonary artery approach is used for most cases. This open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful resection of muscle and to repair the VSD. Timing of surgery in asymptomatic patients is usually between the ages of 2 months to one year. Potential surgical repair complications include residual ventricular septal defect, residual residual outflow tract obstruction, complete atrioventricular block, arrhythmias, aneurysm of right ventricular outflow patch, and pulmonary valve insufficiency.
Total repair of tetralogy of Fallot initially carried a high mortality risk, but this risk has gone down steadily over the years. Post surgery, most patients enjoy an active life free of symptoms. Initially surgery involved forming a side to end anastomosis between the subclavian artery and the pulmonary artery -i. The first Blalock-Thomas-Taussig shunt surgery was performed on month-old Eileen Saxon on November 29, with dramatic results.
The Potts shunt  and the Waterston-Cooley shunt   are other shunt procedures which were developed for the same purpose.
These are no longer used. Currently, palliative surgery is not normally performed on infants with TOF except for extreme cases. Untreated, tetralogy of Fallot rapidly results in progressive right ventricular hypertrophy due to the increased resistance caused by narrowing of the pulmonary trunk. Mortality rate depends on the severity of the tetralogy of Fallot. Patients who have undergone total surgical repair of tetralogy of Fallot have improved hemodynamics and often have good to excellent cardiac function after the operation with some to no exercise intolerance New York Heart Association Class I-II.
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Tetralogy of Fallot occurs approximately times per million live births . Tetralogy of Fallot was initially described in by Niels Stensen. The first surgical repair was carried out in at Johns Hopkins. Taussig , with Vivien Thomas also providing substantial contributions and listed as an assistant.
Critical Care of Children with Heart Disease: Basic Medical and Surgical Concepts - PDF Drive
The first total repair of tetralogy of Fallot was done by a team led by C. Walton Lillehei at the University of Minnesota in on an year-old boy.
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Tetralogy of Fallot
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Bringing care to the doorstep in Cuddalore with Miot Hospital 24 Jul, Cuddalore is a district in the Southern state of Tamil Nadu, an old port city, famous for its temples. The power of a community 26 Jun, Five-month old Satyam and his family originally hail from Gaya, Bihar. A universal language 7 May, Nestled on the Eastern banks of the Godavari river lies a small city called Rajahmundry in the Indian state of Andhra Pradesh.
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