Tetralogy of Fallot

Diagnosis

Tetralogy of Fallot is typically identified shortly after birth. The skin of your baby might seem bluish. When listening to the baby’s heart with a stethoscope, the doctor might hear an unusual whooshing sound (heart murmur).

Tetralogy of Fallot is diagnosed by the following tests:

• Pulse oximetry (oxygen level measurement). The amount of oxygen in the blood is measured using a tiny sensor that is attached to a finger or toe.
• Echocardiogram. Sound waves are used in an echocardiography to produce images of the beating heart. Structure, location, and function of the heart wall, heart chambers, aorta, heart and pulmonary valves can all be seen during an echocardiography.
• Electrocardiogram (ECG or EKG). When the heart beats, an electrocardiogram captures the electrical activity that occurs. On the chest, wrists, and ankles during this test, sticky patches containing wires (electrodes) are applied. The computer that the wires are connected to shows the heart’s rhythm. The presence of an irregular heartbeat (arrhythmia) and enlargement of the heart chambers can both be detected using an ECG.
• Chest X–ray. An X–ray of the chest can reveal the heart and lung architecture. A heart with a boot shape is a common X–ray finding of tetralogy of Fallot because the right ventricle is enlarged.
• Cardiac catheterization. This test may be used by medical professionals to assess the heart’s structure and devise surgical strategies. In this technique, a thin, flexible tube (catheter) is inserted into a blood vessel, typically in the groin, and is then guided to the heart.

To make the heart components easier to observe on X–rays, dye runs via the catheter. During the procedure, the doctor can check the blood arteries and chambers of the heart for pressure and oxygen levels.

Treatment

Corrective surgery by a cardiovascular surgeon is necessary for all babies diagnosed with tetralogy of Fallot. Without this treatment, the child may experience growth and developmental issues. The specific surgical procedure and timing will be determined by the child’s condition and health. Prior to surgery, certain children may require medication to sustain the flow of blood from the heart to the lungs. This medication helps in maintaining adequate blood circulation until the surgical intervention takes place.

Surgery or other procedures

Open heart surgery to repair the flaws (intracardiac repair) or a short–term therapy with a shunt are the two options for treating tetralogy of Fallot. The majority of infants and older kids get intracardiac repair.

Intracardiac repair

Multiple repairs are made during this open–heart procedure, which is typically performed within the first year of life. In adults with tetralogy of Fallot who did not receive surgical repair as children, this procedure is very uncommon.

A surgeon performing an intracardiac repair will:

• Close the opening between the ventricles with a patch to cover the ventricular septal defect.
• To improve blood flow to the lungs, repair or replace the narrowed pulmonary valve.

After this treatment, the right ventricle wall will return to its usual thickness since it won’t need to exert as much effort to pump blood. The blood’s oxygen content rises and symptoms subside after intracardiac repair.

Temporary shunt surgery

Sometimes babies need a brief (palliative) operation before an intracardiac repair to enhance blood flow to the lungs. If your child was born preterm or has hypoplastic (underdeveloped) pulmonary arteries, this operation may be performed.

A big artery that arises from the aorta and the pulmonary artery are connected by a bypass (shunt) created by the surgeon during this treatment.

The surgeon removes the shunt when your infant is prepared for intracardiac repair and performs the intracardiac repair operation.

Post surgery

People who have undergone tetralogy of Fallot surgery continue to have higher long–term survival rates.

After tetralogy of Fallot surgery, blood flow to the lungs might occasionally remain be reduced. Additional operations might be required. Adults who have had their tetralogy of Fallot fixed may later need to have their pulmonary valve replaced if they have a leaky pulmonary valve (pulmonary valve regurgitation).

Arrhythmias are a typical consequence of tetralogy of Fallot repair surgery. Your doctor may advise taking certain medications, having the ablation, or getting an implantable cardioverter–defibrillator, a unique pacemaker used to treat arrhythmias that are life–threatening.

Ongoing care

Patients with tetralogy of Fallot require ongoing care from a pediatric or adult cardiologist to monitor the effectiveness of their treatment and watch for any potential complications. As part of routine checkups, imaging exams are often performed to evaluate the patient’s progress and the overall success of their treatment.