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TOF Repair: Symptoms, Classification, Diagnosis & Recovery

A very rare, complex congenital heart disease, tetralogy of Fallot affects approximately 5 out of every 10,000 babies. It involves four different heart issues. It usually presents with ventricular septal defect, pulmonary stenosis, right ventricular hypertrophy, and overriding aorta, affecting how blood flows through the heart and to the rest of the body. Babies with this condition may have Cyanosis and appear blue or gray due to low oxygen levels. Doctors usually identify it during pregnancy or shortly after birth. In some cases, if symptoms are mild, they may remain unnoticed until adulthood. Treatment involves surgery, and lifelong checkups are necessary for those diagnosed with this condition.

The specific cause of tetralogy of Fallot is uncertain, but certain factors may heighten the chances of a baby being born with it. These risk factors include
  • A family history
  • Exposure to viruses like rubella (German measles) during pregnancy
  • Alcohol consumption
  • Poor nutrition
  • Smoking during pregnancy
  • Mother's age above 35
  • Presence of Down syndrome or DiGeorge syndrome in the baby.

The symptoms of Tetralogy of Fallot vary based on how much blood flow is restricted from leaving the heart for the lungs. Signs may include

  • Bluish or gray skin
  • Difficulty breathing and fast breaths, especially during activities
  • Trouble gaining weight
  • Getting tired during play
  • Irritability
  • Prolonged crying
  • Occasional fainting

Tetralogy of Fallot is typically identified shortly after a baby is born. Signs include the baby's skin having a bluish or grayish tint and the presence of a heart murmur, a whooshing sound heard with a stethoscope.

Diagnostic tests for tetralogy of Fallot comprise:

  1. Oxygen Level Check: A small sensor known as a pulse oximeter placed on a finger or toe measures the blood's oxygen level.
  2. Echocardiogram: This test uses sound waves to create moving pictures of the heart, providing insights into its structure and valve function.
  3. Electrocardiogram (ECG or EKG): By recording the heart's electrical activity, this test, involving sticky patches on the chest, arms, or legs, helps assess the heart's rhythm and detect irregularities.
  4. Chest X-ray: This shows the heart and lung shape, often showcasing a boot-shaped heart on an X-ray, indicating enlargement of the right lower chamber.
  5. Cardiac Catheterization: In this procedure, thin tubes called catheters are inserted into a blood vessel in the groin and guided to the heart. It helps in diagnosis and treatment planning.

These tests play a crucial role in understanding and addressing the tetralogy of Fallot.

There are two main approaches for treating tetralogy of Fallot: complete intracardiac repair and temporary or palliative repair.

Complete Intracardiac Repair (Open-heart Surgery): This surgery focuses on ensuring proper blood flow to the lungs. The passageway between the right ventricle and the pulmonary artery is widened to facilitate a smooth blood flow, supplying oxygen-rich blood to the entire body. Additionally, a patch is placed to cover the Ventricular Septal Defect, preventing the mixing of oxygen-rich and oxygen-poor blood between the ventricles. These two repairs correct the heart valve defects and ensure a consistent supply of oxygen-rich blood throughout the body.

Temporary or Palliative Repair: This approach is for infants who might not be strong enough for full surgery. It temporarily corrects the blood flow to the lungs. The surgeon inserts a shunt between a large artery branching off the aorta and the pulmonary artery. This creates an extra pathway for blood to reach the lungs for oxygen. As the child grows, a complete intracardiac repair is performed, and the shunt is removed, resolving the heart defects.

After tetralogy of Fallot treatment, your doctor may suggest measures to maintain heart health. These could include:

The patient is monitored closely for a week or more. Since the surgery is an open heart procedure extra care should be given while taking care of the patient. The incision for the surgery heals completely in 6 weeks and proper care should be taken to ensure that it doesn’t infect.

Activity Guidelines: Depending on the severity of the heart condition, some individuals may need to restrict exercise or sports activities. It's important to consult with your healthcare team to determine which activities are safe.

Antibiotics for Heart Protection: In cases where serious heart issues increase the risk of heart lining or valve infections (endocarditis), antibiotics might be recommended, In case of Temporary repair, the patient may need medicines to keep the shunt open while waiting for the full repair. These medicines are stopped after the shunt is removed.

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