Tetralogy of Fallot

Tetralogy of Fallot (TOF) is a complex congenital heart defect that involves four structural abnormalities in the heart.

This condition impacts the heart’s anatomy and its ability to circulate oxygen-rich blood throughout the body.

TOF is a congenital condition, meaning your child is born with it. Usually, it happens by chance, without any known cause or risk factor. Detecting and treating TOF is important to ensure the health of a child’s heart.

What is Tetralogy of Fallot?

Tetralogy of Fallot is a combination of four congenital heart defects that occur together:

• Ventricular septal defect (VSD): A hole between the heart’s lower chambers
• Pulmonary stenosis: Narrowing of the pulmonary valve and artery
• Right ventricular hypertrophy: Thickening of the muscle of the right ventricle
• Overriding aorta: The aorta is positioned directly over the VSD, on the right side, instead of where it should be, on the left side of the heart

These defects cause oxygen-poor blood to flow out of the heart and into the rest of the body, leading to symptoms such as cyanosis (bluish skin), shortness of breath, and fatigue.

Congenital heart defects and Tetralogy of Fallot

Tetralogy of Fallot is present at birth and is one of the more severe congenital heart defects. The exact cause is unknown, but genetic and environmental factors may play significant roles. It is crucial for expectant parents to understand the potential for this condition if there is a family history of congenital heart issues.

Surgeons at WashU Medicine are experts in child-friendly care. They are nationally and internationally recognized for their expertise, offering world-class care for pediatric patients. The team of surgeons are adept in the latest surgical techniques and innovative technologies to treat the most complex cases, such as Tetralogy of Fallot.

Diagnosis and treatment

TOF is a rare condition that can be detected before birth or after. Treatment is complex, so finding a skilled team with experience in surgery for congenital heart defects is essential

Diagnosis

TOF is typically diagnosed either during an ultrasound in pregnancy, or within the first few weeks after birth.

Doctors may use various methods, such as:

• Echocardiogram: An ultrasound of the heart to view its structure and function
• Electrocardiogram (ECG): A test to measure the electrical activity of the heart
• Chest X-ray: Observes the heart’s size and any abnormalities in the lung fields
• Cardiac MRI: Provides detailed images of the heart and major blood vessels

Treatment

Treatment of TOF usually involves surgical interventions:

• Complete intracardiac repair: The most common surgery, usually performed in infancy, which involves closing the VSD and repairing the narrowed pulmonary artery. The procedure usually involves:
• Closure of the VSD: Using a synthetic patch to seal the hole between the heart’s lower chambers.
• Relief of pulmonary stenosis: Repairing or replacing the narrowed pulmonary valve and widening the pulmonary artery to improve blood flow to the lungs.
• Redirecting the aorta: Ensuring the aorta is correctly positioned over the left ventricle.
• Temporary or palliative surgery: Such as a shunt operation, to improve blood flow until a more definitive surgery can be performed.

Medications

While surgeries are the primary treatment, medications may be prescribed to manage symptoms before and after surgery. These may include drugs to control heart rhythm, improve heart function, or alleviate fluid buildup.

Potential complications

Complications from TOF and its treatments might include:

• Irregular heartbeats (arrhythmias)
• Leaking heart valves
• Residual VSD
• Heart failure
• The need for further operations or catheter-based interventions later in life

Postoperative care and long-term management

Comprehensive care doesn’t end with surgery.

Postoperative management is crucial for ensuring the best outcomes and includes:

• Intensive care monitoring: Close observation in the Pediatric Intensive Care Unit (PICU) or Cardiac Intensive Care Unit (CICU) immediately after surgery
• Medical management: Prescription of medicines to support heart function, manage arrhythmias, and prevent blood clots or infections
• Regular follow-ups: Lifelong cardiology follow-ups are recommended to monitor the patient’s heart health and detect any potential complications early
• Rehabilitation programs: Tailored physical and occupational therapy to aid recovery and improve quality of life

Living with Tetralogy of Fallot

Living with Tetralogy of Fallot requires ongoing medical care, but with advances in medical technology and surgical techniques, individuals with TOF can lead full and active lives.

It is essential for patients and their families to stay informed and proactive about their heart health.

Regular consultations with health care providers and adherence to medical advice will help achieve the best possible outcomes for those affected by this congenital heart condition.