Tetralogy of Fallot is a congenital Heart defect and is understood to involve four anatomical abnormalities [though only three of them are always present].

It is the most common cyanotic heart defect and is one of the more common causes of the “blue baby syndrome

Causes a low oxygen of the blood.

Blood with oxygen [oxygenated] mixes with blood without oxygen [deoxygenated]

This condition is seen in approximately 3-6 births out of 100,00.

 

The 4 anatomical abnormalities are:

1.)  Ventricular Septal Defect
2.)  Pulmonary Stenosis
3.)  Overriding aorta
4.)  Right ventricle hypertrophy (enlargement)

 

Symptoms

  • Low oxygen
  • Cyanosis
  • Low birth weight
  • Referred to as a Pink tet
  • Heart murmur
  • Difficulty in feeding
  • Difficulty for infant to gain weight
  • Clubbing of toes
  • Syncope

 

Additional Anomalies that can be seen

In addition, tetralogy of Fallot may present with other anatomical anomalies, including:

  1. Stenosis of the left pulmonary artery [40-50% of patients]
  2. A bicuspid pulmonary valve [40% of patients]
  3. Right-sided aortic arch [25% of patients]
  4. Coronary Artery changes [15% of patients]
  5. Foramen Ovale
  6. Atrial Septal Defect
  7. Atrioventricular Septal Defect
  8. Anomalous Pulmonary venous return [partially or completely]
  9. Forked ribs
  10. Scoliosis

 

Treatment

Medicine
. Propranolol [Beta-Blocker]
. Morphine
. Phenylephrine – increases blood pressure

. Oxygen
. Palliative surgery
. Total Surgical repair
. Another surgery may be required around age 20
. Antibiotic treatment to limit possible Endocarditis

Without treatment mortality increases dramatically each year of survival