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Structural Heart Defects (Tetralogy of Fallot (Features (Pulmonary…
Structural Heart Defects
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Bicuspid Aortic Valve
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Normal aortic valve has 3 cusps, bicuspid aortic valve has only 2
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Atrial Septal Defect
Increased blood flow into right heart and lungs, so patient is not blue = acyanotic
If left untreated, can lead to right heart dilation and increased risk of infective endocarditis
As there is slightly higher pressure in LA than RA, the shunt is left to right
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Investigations
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Chest X-ray: large heart, large pulmonary arteries
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Patent Ductus Arteriosus
Normally the ductus arteriosus closes within a few hours of birth in response to decreased pulmonary resistance
If the ductus remains open, there is an abnormal shunt from the aorta to the pulmonary artery
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Pulmonary vascular resistance is high in the foetus as the vessels are filled with fluid and right heart pressure > left heart pressure
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Ductus arteriosus is a temporary vessel in the foetus which connects the pulmonary artery to the aorta, so the blood bypasses the lungs (these have not been developed yet)
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Coarctation of the Aorta
Associated with Turner's syndrome, bicuspid aortic valve
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Net result is narrowing of the aorta just after the arch and stronger perfusion to the upper body compared to the lower
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Narrowing of the descending aorta, usually occurs at the site of insertion of the ductus arteriosus
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Tetralogy of Fallot
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Features
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Aorta overrides the VSD, accepting right heart blood
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Treatment
Without surgery, mortality rate is 95% by age 20
After repair, 85% of patients survive to 35 years. Common problem in adults is pulmonary regurgitation
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Pulmonary stenosis
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Severe case presents with RV failure as a neonate, collapse, poor pulmonary blood flow, RV hypertrophy, tricuspid regurgitation
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