Question
A newborn infant presented with cyanosis.On examination:a systolic ejection murmur is heard over the pulmonic area and left sternal border.Chest X ray findings are shown in the image.The following features are true for this condition except:
A. Overriding Aorta
B. Pulmonary Stenosis
C. Right ventricular hypertrophy
D. Atrial Septal Defect
Show Answer
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Correct Answer » D Explanation |
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Ans:D. Atrial Septal Defect.
The patient is suffering from tetralogy of Fallot.
Cor-en-sabot/Boot shaped Heart. is seen in the X ray
Tetralogy of Fallot (TOF)
- It is a congenital heart defect that is present at birth.
- Classically there are four defects:
- a ventricular septal defect,
- pulmonary stenosis,
- right ventricular hypertrophy,
- an overriding aorta,
- The overall heart size in tetralogy of Fallot is usually Normal or small in size.
- Signs and symptoms
- Tetralogy of fallot is associated with Right ventricular out flow tract obstruction due to Pulmonic Stenosis.The clinical consequences depends primarily on the severity of subpulmonic stenosis.Typically this is significant, resulting in cyanosis evident in the neonatal period, as a consequence of the right to left shunt across the VSD. If the subpulmonary stenosis is mild, the abnormality resembles an isolated VSD, and the shunt may be leftto-right, without cyanosis (pink tetralogy)
- Failure to thrive
- Episodes of bluish pale skin during crying or feeding (ie, “Tet” spells)
- Exertional dyspnea, usually worsening with age
- Physical findings include the following:
- Most infants are smaller than expected for age
- Cyanosis of the lips and nail bed is usually pronounced at birth
- After age 3-6 months, the fingers and toes show clubbing
- A systolic thrill is usually present anteriorly along the left sternal border
- A harsh systolic ejection murmur (SEM) is heard over the pulmonic area and left sternal border
- During cyanotic episodes, murmurs may disappear
- In individuals with aortopulmonary collaterals, continuous murmurs may be auscultated
- RV predominance on palpation
- A bulging left hemithorax
- Aortic ejection click
- Squatting position (compensatory mechanism)
- Scoliosis (common)
- Tetralogy of fallot is associated with Right ventricular out flow tract obstruction due to Pulmonic Stenosis.The clinical consequences depends primarily on the severity of subpulmonic stenosis.Typically this is significant, resulting in cyanosis evident in the neonatal period, as a consequence of the right to left shunt across the VSD. If the subpulmonary stenosis is mild, the abnormality resembles an isolated VSD, and the shunt may be leftto-right, without cyanosis (pink tetralogy)
- Risk factors include:
- a mother who uses alcohol, has diabetes, is over the age of 40, or gets rubella during pregnancy. It may also be associated with Down syndrome.
- Congenital heart defects are now diagnosed with echocardiography, which is quick, involves no radiation, is very specific, and can be done prenatally.
- Plain radiograph
- Chest radiographs may classically show a “boot-shaped” heart with an upturned cardiac apex due to right ventricular hypertrophy and concave pulmonary arterial segment.
- Pulmonary oligaemia due to decreased pulmonary arterial flow. Right-sided aortic arch is seen in 25%..
- Treatment:
- Most infants with tetralogy of Fallot require some type of surgical procedure. Surgery is preferably done at or about 12 months of age.
