Tetralogy of Fallot (TOF) is a congenital heart condition characterized by four defects:
- Ventricular septal defect: A hole between the heart’s lower chambers (ventricles)
- Pulmonary stenosis: A blockage between the heart and the lungs due to the narrowing of the main pulmonary artery and valve
- Overriding of the aorta: The enlarged aortic valve opens from both ventricles rather than just the left, as it would in a normal heart. The ventricular septal defect is directly under the aortic valve.
- Ventricular hypertrophy: The right ventricle, the lowest chamber of the heart, has a thicker muscular wall than usual.
Because of these structural flaws, blood is poorly oxygenated when pumped to the rest of the body. Because the blood does not contain enough oxygen, infants may display a bluish discoloration (cyanosis) of the skin, nails, and lips.
Tetralogy of Fallot is commonly diagnosed when the child is still a newborn or shortly after. It can sometimes go undetected until adulthood, depending on the severity of the abnormalities and symptoms.
Tetralogy of Fallot is a complex condition linked to the following: untreated maternal diabetes, poor maternal nutrition, alcohol consumption during pregnancy, and genetics.
Tetralogy of Fallot symptoms include the following:
- A baby who becomes cyanotic when crying, feeding, or when agitated. This is known as a “tet spell” when an infant’s blood supply cannot keep up with their need for oxygen.
- Squatting in young children when short of breath to increase blood flow to the lungs.
- Clubbing of the fingers
- Poor weight gain
- Tiring easily during play
- Heart murmur
MRI, ECG, CT scan, and echocardiograms are practical tests for the diagnosis and evaluation of TOF.
A pediatrician, cardiologist, cardiac surgeon, radiologist, and nurse work as an interprofessional team to diagnose and treat TOF. Surgery is necessary for all children with TOF for normal growth and development. Most infants with TOF undergo a primary repair during the first year of life. The results for TOF patients are good, although a significant portion will need pulmonary valve replacement after 20 years.
Most children who have surgery do not experience any side effects. It is crucial to teach the parents that surgery for TOF is palliative rather than curative. Additional surgeries may be needed in adulthood, and lifelong management is required.
Health teaching must include regular follow up with a cardiologist. The nurse must emphasize the need to take medication as prescribed, complete follow-ups and routine testing, and adhere to lifestyle modifications such as activity restrictions.
Nursing Care Plans
Decreased Cardiac Output
Ineffective tissue perfusion associated with Tetralogy of Fallot can be caused by decreased blood flow to tissues.
Nursing Diagnosis: Decreased Cardiac Output
- Lack of oxygenated blood
- Poor perfusion to tissues and organs
- Reduced blood flow
- Mixed oxygenated and deoxygenated blood
As evidenced by:
- Murmur upon auscultation
- Tet spells
- Clubbing of fingers
- Poor developmental growth
- Patient will demonstrate an oxygen saturation level within expected limits
- Patient will be able to tolerate playing or exercise without dyspnea, fatigue, or fainting
1. Assess cardiac status.
Reduced cardiac output and stroke volume are indicators of weak pulses. Capillary refill may be sluggish. Presence of abnormal heart sounds such as S3 and S4. S3 indicates left ventricular failure and denotes diminished left ventricular ejection. The left ventricle’s lower compliance in S4 compromises diastolic filling.
2. Monitor blood pressure and heart rate.
As a result of the lower cardiac output, most cardiac patients experience compensatory tachycardia and abnormally low blood pressure.
3. Note for other cardiovascular symptoms.
Note complaints of fatigue, decreased activity tolerance, skin color, temperature, and moisture. These are indicators of decreased cardiac output and oxygen desaturation.
4. Monitor for Tet spells.
Tet spells describe newborns with deep blue skin, lips, and nails after crying, feeding, defecating, or becoming restless. A rapid decline in blood oxygen levels brings on Tet spells.
5. Review RBC and hemoglobin levels.
As the body works to remedy the tissues’ shortage of oxygen, complete blood count may reveal increased red blood cell count and hemoglobin.
6. Obtain ECG.
Low perfusion, acidosis, or hypoxia caused by low cardiac output can all lead to cardiac dysrhythmias. Cardiovascular blood flow can also be impaired by tachycardia, bradycardia, and ectopic beats.
7. Verify with echocardiography.
The degree of pulmonary stenosis, the ventricular septal defect, a hole between the left and right ventricles, and other unexpected anomalies will be visible using echocardiography.
8. Assist in cardiac catheterization.
Cardiac catheterization may further measure the blood pressure and oxygen in the pulmonary artery and aorta and can open narrowed arteries.
1. Place the patient on their left side, knees to chest.
Positioning the patient on the left side with knees to the chest reduces the blood flow through the septal hole from the right ventricle and improves blood circulation to the lungs. The increase in the aortic and left ventricular pressure increases oxygenated blood flow to the tissues.
2. Cautiously administer oxygen therapy as prescribed.
When a child has Tetralogy of Fallot, breathing more oxygen minimizes their oxygen levels. Cautiously provide oxygen with only the prescribed amount via a face mask to improve blood oxygen levels.
3. Manage Tet spells.
Morphine, propranolol (or metoprolol), or, in difficult situations, phenylephrine may be administered to the child. These medications lessen tet spells’ incidence and severity.
4. Prepare for surgical management.
Surgical management can include a palliative shunt or complete repair:
The shunt directs blood flow from the left or right subclavian artery to the pulmonary arteries. It enables the infant to reach an appropriate size for a complete surgical repair.
The right ventricular pulmonic stenosis is removed, and the opening in the ventricular septum (between the ventricles) is patched up.
5. Refer to a cardiologist.
Cardiologists specialize in preventing, detecting, and treating congenital cardiac diseases such as TOF. The patient will need lifelong follow-ups with a cardiologist who will monitor and manage the condition.
Compromised Family Coping
Compromised family coping associated with Tetralogy of Fallot can be caused by situational family crises and developmental crises in the infant.
Nursing Diagnosis: Compromised Family Coping
- Situational family crisis
- Developmental crisis
- Inadequate information or resources
- Lack of support
- Change in family roles
As evidenced by:
- Verbalization of concern and fear about the condition
- Overprotective behavior of the parents/family member
- Expression of inadequate knowledge
- Ineffective coping mechanisms
- Family will be able to use coping mechanisms towards the crisis effectively
- Parents/family will be able to verbalize acceptance of the current situation
- Parents/Family will be able to verbalize a positive outlook on the situation
1. Identify family coping mechanisms.
Determine how the family has coped in the past and the necessity of learning new coping mechanisms if attempts fail to alter behavior.
2. Assess the family’s knowledge about the condition.
Accurate information relieves anxiety and reduces/eliminates the fear of the condition and the future.
3. Observe verbal and nonverbal behaviors.
Verbal and nonverbal behaviors provide essential details about the family’s feelings, how they process information, and how to approach them.
4. Consider cultural factors.
Cultural differences may affect how the family copes, communicates, processes, and makes decisions.
1. Assist the family in identifying a suitable coping mechanism.
Assist the family in developing and employing coping mechanisms to deal with the crisis and take control of the situation.
2. Encourage to maintain open communication.
Encourage social communication and expression of feelings. Feelings of anxiety, exhaustion, and loneliness are expected and can be managed.
3. Instruct on the effects of overprotective behavior.
Teach that being overprotective can impede a child’s growth and development during their early years. It can result in risk aversion, parental dependence, a higher chance of psychological problems, a deficiency in practical coping skills, and long-term anxiety.
4. Refer to a family counselor.
Family therapy or counseling in a group setting can instruct on how the behaviors of all family members affect not only the relationships between family members but the family as a whole. Strategies can then be developed on effective teamwork and coping.
Risk for Infection
Risk for infection associated with Tetralogy of Fallot is associated with lifelong treatment, surgical procedures, and hospitalizations.
Nursing Diagnosis: Risk for Infection
- Long-term treatment
- Compromised immune system
- Surgical procedures
- Prolonged hospitalization
As evidenced by:
A risk for diagnosis is not evidenced by signs and symptoms as the problem has not yet occurred, and nursing interventions are aimed at prevention.
- Patient will be able to maintain an infection-free health status
- Patient will be able to identify infection prevention measures
- Patient will be able to enumerate the signs and symptoms of infection
1. Assess risk factors.
Risk factors include medications, surgical procedures, compromised immune systems, and other comorbidities.
2. Consider the patient’s age.
Infants and young children are frequently sick due to a delicate immune system. They may be around other children in school or daycare settings, contributing to the risk of contagious illnesses.
3. Assess vital signs.
The most accurate and sensitive symptoms for identifying severe bacterial infections are fever, tachycardia, and tachypnea.
4. Review white blood cell count.
A high white blood cell count can identify an underlying infectious process.
1. Follow standard precautions.
Standard precautions are infection control measures intended to stop the spread of diseases that can be contracted by contact with blood, bodily fluids, non-intact skin, and mucous membranes. Healthcare professionals should maintain strict handwashing protocols and wear gloves or other protective equipment as necessary.
2. Maintain sterile technique in IV procedures.
When accessing an IV, use sterile methods to prevent microorganisms from entering the body.
3. Provide adequate nutrition and rest.
The best way to prepare the body to fight infection and boost the immune system is with a balanced diet rich in vitamins and minerals, adequate sleep, regular exercise, and stress reduction.
4. Administer prophylactic antibiotics.
Children and adults with heart defects may need to take antibiotics prophylactically before dental or surgical procedures to prevent infection.
5. Ask the parents to list the signs and symptoms of infection.
Nurses should educate parents on detecting infection symptoms and when to alert their providers.
- American Heart Association. (2018, January 19). Tetralogy of Fallot. www.heart.org. https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/tetralogy-of-fallot
- Centers for Disease Control and Prevention. (2022). Facts about tetralogy of Fallot. https://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html
- Doenges, M. E., Moorhouse, M. F., & Murr, A. C. (2019). Nurse’s pocket guide: Diagnoses, interventions, and rationales (15th ed.). F A Davis Company.
- Diaz-Frias, J., & Guillaume, M. (2022). Tetralogy of Fallot. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK513288/
- Mayo Clinic. (2021). Tetralogy of Fallot – Symptoms and causes. https://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/symptoms-causes/syc-20353477