What is the most common complication found in patients with tetralogy of Fallot?

ABSTRACT

Introduction: We report a series of operated tetralogy of Fallot (TOF) patients focusing on complications and outcomes. Materials and Methods: Data from TOF patients seen at our centre’s adult congenital heart disease clinic was analysed. Results: There were 21 patients: the mean age was 32.2 ± 12.4 years; the age at first operation was 9.0 ± 7.9 years; the mean postoperative follow-up duration was 23.5 ± 12.1 years; and the current New York Heart Association (NYHA) status: I, 82%; II, 4%; III, 14%. Fourteen patients had complete operative notes. All these patients underwent total TOF correction; 2 had staged aortopulmonary shunt with total correction at a mean of 3.2 years later, pulmonary artery patch augmentation in 8 patients and pulmonary valvotomy in 8 patients. Three patients required pulmonary valve homograft replacement for severe pulmonary regurgitation (PR) at 13, 28 and 36 years after the initial corrective operation. Current investigations: RBBB on ECG (91%), QRS duration 137 ± 29 ms. Echocardiography showed dilated right ventricular end-diastolic (RVED) diameters (3.2 ± 0.8 cm); severe PR (67%), residual right ventricular outflow tract obstruction (RVOTO) (42%) and VSD patch leakage (9%). Cardiac magnetic resonance (CMR) (8 patients) showed dilated RVED volumes 252.6 ± 93.8 mL, indexed RV volume 165.7 ± 34.8 mL; RV systolic function was preserved in most patients with a RV ejection fraction of 49.5 ± 5.7%. One patient had atrial tachycardia and another had frequent non-sustained ventricular tachycardia that required radiofrequency ablation. Conclusion: Patients with TOF who had full corrective surgery during childhood are now surviving into adulthood. Many challenges arising from complications in the postoperative period remain. It is imperative that adult TOF patients should have regular follow-up to monitor development and subsequent management of these complications.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease with an incidence of approximately 0.5/1000 live births (5% to 7% of congenital heart lesions). In Singapore with a live birth of over 37, 000 per year,1 the expected number of new TOF cases will be approximately 19 per year. Based on data obtained from the National Birth Defects Registry, there were 164 TOF cases amongst all live and stillbirths between 1994 and 2000.2 In classical TOF, an anterior and cephalic displacement of the infundibular septum results in a large ventricular septal defect (VSD) and the development of infundibular pulmonary stenosis. Right ventricular (RV) hypertrophy is associated with both pulmonary stenosis and VSD (Fig. 1). Without surgical intervention, most patients die in childhood with a rate of survival of 66% at 1 year of age, 40% at 3 years, 11% at 20 years and 3% at 40 years .3 The advent of surgical repair which includes closure of the VSD and relief of RV outflow tract (RVOT) obstruction has greatly improved the long-term survival of TOF patients. Complete repair of TOF in early childhood is now routinely available in tertiary centres in Singapore. High survival rates in TOF patients who underwent complete repair have been reported.4,5

This article is available only as a PDF. Please click on “Download PDF” to view the full article.

What is tetralogy of Fallot?

Tetralogy of Fallot (TOF) is a set of 4 congenital heart defects that occur together. "Tetralogy" refers to the number 4. Congenital means that a baby is born with them. The set of 4 defects are:

• Narrowing (stenosis) of the pulmonary artery. This is the artery that carries blood from the heart to the lungs to pick up oxygen.

• Ventricular septal defect. This is an opening in the wall between the 2 lower chambers of the heart (right and left ventricles). 

• Overriding aorta. The artery that carries oxygen-rich blood to the body (aorta) is shifted toward the right side of the heart. It should be on the left side. In this condition, the aorta sits over the ventricular septal defect.

• Enlargement (hypertrophy) of the right ventricle. The right lower chamber of the heart (ventricle) is bigger than normal.

In a healthy heart, oxygen-poor (blue) blood returns to the right chamber of the heart (right atrium) from the body. Next, it travels through the tricuspid valve to the right ventricle of the heart. Then it’s pumped through the pulmonary valve to the pulmonary artery and into the lungs for oxygen. Oxygen-rich (red) blood comes back to the left upper chamber of the heart (left atrium) from the lungs. Then it passes through the mitral valve and into the left ventricle of the heart. Finally, it’s pumped through the aortic valve to the aorta and out to the body.

Some children with TOF may only have slightly lower than normal oxygen levels in their blood. These children have minimal narrowing of the pulmonary artery. They don’t usually have bluish skin (cyanosis). Other children with TOF will have low oxygen levels in their blood. These children have more severe narrowing of the pulmonary artery. They have bluish skin. This is from the low oxygen levels in their blood.

What causes tetralogy of Fallot?

Genes and family history may play a part in TOF. It may also be caused by Down syndrome or 22q11.2 deletion syndrome (DiGeorge syndrome). Most of the time, this heart defect occurs by chance, with no cause.

What are the symptoms of tetralogy of Fallot?

Symptoms can show up a bit differently in each child. The most common symptom is a bluish color of the skin, lips, and nail beds. This may come on in sudden spells, called TET spells. It happens when blood oxygen level drops quickly. During the spell, babies may have a hard time breathing. They may also be tired and fussy. In the most severe cases, they may lose consciousness.

The symptoms of TOF may be similar to symptoms caused by other problems. Make sure that your child sees a healthcare provider for a diagnosis.

How is tetralogy of Fallot diagnosed?

Your child will need to see a heart doctor (pediatric cardiologist.) They will check your baby and listen to their heart and lungs. The details about your child’s heart murmur will also help the doctor make the diagnosis.

Tests may be done to confirm the diagnosis. The tests your child has depends on their age and condition, and the doctor’s preferences.

Echocardiogram (echo)

An echo uses sound waves to make a moving picture of the heart and heart valves. This test will show structural changes caused by TOF.

Chest X-ray

A chest X-ray may show changes in the heart and lungs caused by TOF. The heart on an X-ray may be enlarged or have the shape of a boot.

ECG

This test records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias or dysrhythmias) and spots heart muscle stress. These issues may be caused by TOF.

Cardiac catheterization (cardiac or heart cath)

A cardiac catheterization gives detailed information about the structures inside the heart. In this test, a small, thin, flexible tube (catheter) is put into a blood vessel in your child’s groin. Then the healthcare provider guides it to your child’s heart. They will inject your child with contrast dye to see their heart more clearly. This test measures your child’s blood pressure and oxygen in the 4 chambers of the heart. It also measures blood pressure and oxygen in the pulmonary artery and aorta. Your child will get medicine to help them relax and prevent pain (sedation).

How is tetralogy of Fallot treated?

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.

Some children will need a small tube called a shunt put into the pulmonary artery. This helps create stable pulmonary blood flow until a permanent repair can be done at a later age.

All children with TOF need to have surgery to fix it. Most children have it before they turn 1 year old. It’s often done around age 6 months. A team of heart surgeons will do your child’s surgery. To fix TOF, a surgeon may use a patch to close the ventricle septal defect (VSD). The surgeon will enlarge the right ventricular outflow tract. This can be done by removing excess heart muscle or using a patch to enlarge narrowed pulmonary arteries.

Fixing the heart defects will allow oxygen-poor blood to travel its normal route. This is through the pulmonary artery to the lungs to pick up oxygen.

What are possible complications of tetralogy of Fallot?

If left untreated, it can cause these problems:

• Blood clots (which may be in the brain causing stroke)

• Infection in the lining of the heart and heart valves (bacterial endocarditis)

• Abnormal heart rhythms (arrhythmias)

• Heart failure

• Death

Living with tetralogy of Fallot

The heart doctor may give your child antibiotics to prevent infections after leaving the hospital. Your child may also need medicine before other surgeries or dental tests.

After surgery, your child may become tired easily and sleep more. Over time, most children are able to be active. Most children’s appetite and growth become normal soon after surgery. Some children who had surgery for TOF can have problems learning or growing normally.

Most children who have surgery for this condition will live healthy lives. They may need more surgeries throughout their lives. Your child might need a pulmonary valve replacement surgery when they are an adult. This will help prevent heart complications. These include enlargement of the right ventricle, abnormal heart rhythms, and heart failure. Women who want to have children should be checked by a heart doctor before they get pregnant.

Ask your child's healthcare provider about your child’s outlook.

When should I call my child's healthcare provider?

Call your child’s healthcare provider if they have trouble breathing, eating, or being active.

Key points about tetralogy of Fallot

• Tetralogy of Fallot is a set of 4 congenital heart defects. Children are born with this condition.

• This condition causes problems with the heart’s ability to pump oxygen-rich blood to the body.

• All children with TOF need to have surgery to fix it.

• After surgery, most children will live healthy lives.

• Most children who have had surgery for TOF will need more surgery when they are an adult.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.

• Before your visit, write down questions you want answered.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.

• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.

• Ask if your child’s condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if your child does not take the medicine or have the test or procedure.

• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.

What is the most common complication of tetralogy of Fallot?

What are the possible complications of tetralogy of Fallot?

How does tetralogy of Fallot affect the body?

What syndromes are associated with tetralogy of Fallot?