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
- Genetics
In this article:
- Symptoms
- Nursing Process
- Nursing Care Plans
- Compromised Family Coping
- Impaired Gas Exchange
- Ineffective Tissue Perfusion
- Risk for Infection
Symptoms
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
- Fainting
MRI, ECG, CT scan, and echocardiograms are practical tests for the diagnosis and evaluation of TOF.
Nursing Process
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
Once the nurse identifies nursing diagnoses for tetralogy of Fallot, nursing care plans help prioritize assessments and interventions for both short and long-term goals of care. In the following section you will find nursing care plan examples for tetralogy of Fallot.
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
Related to:
- Lack of oxygenated blood
- Poor perfusion to tissues and organs
- Reduced blood flow
- Mixed oxygenated and deoxygenated blood
As evidenced by:
- Cyanosis
- Murmur upon auscultation
- Tet spells
- Clubbing of fingers
- Squatting
- Dyspnea
- Fatigue
- Poor developmental growth
Expected outcomes:
- Patient will demonstrate an oxygen saturation level within expected limits.
- Patient will be able to tolerate playing or exercise without dyspnea, fatigue, or fainting.
Assessment:
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.
Interventions:
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:
Palliative shunt:
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.
Complete 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
Related to:
- 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
- Anxiety
- Withdrawal
- Ineffective coping mechanisms
Expected outcomes:
- 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.
Assessment:
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.
Interventions:
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.
Impaired Gas Exchange
Impaired gas exchange associated with Tetralogy of Fallot can be caused by a lack of oxygenated blood supply reaching the lungs.
Nursing Diagnosis: Impaired Gas Exchange
Related to:
- Inadequate gas exchange
- Poor production of oxygenated blood
- Insufficient oxygenated blood circulation
- Poor perfusion to pulmonary tissues
- Reduced blood flow
- Inadequate blood supply
- Mixed oxygenated and deoxygenated blood
As evidenced by:
- Difficulty breathing
- Coughing
- Rapid breathing
- Fatigue
- Use of accessory muscles
- Nasal flaring
- Headache
- Alteration in the level of consciousness
- Cyanosis
- Tachycardia
- Palpitations
- Anxiety
Expected outcomes:
- Patient will maintain oxygen saturation within normal limits.
- Patient will present an alert, conscious, and coherent level of consciousness.
- Patient will be able to play or tolerate activities with no complaints of difficulty breathing.
Assessment:
1. Assess respiratory status.
Pediatric patients may exhibit signs and symptoms of respiratory distress or cyanosis during crying, eating, or playing. It’s important to rule out other possible causes, such as bronchiolitis, pneumonia (viral/bacterial), or respiratory syncytial virus (RSV).
2. Determine the oxygen saturation.
In babies with tetralogy of Fallot, the arterial oxygen saturation may abruptly decline. A tetralogy spell occurs when the blood vessels to the lungs suddenly become more constricted, further reducing pulmonary blood flow.
3. Review ABG analysis.
ABG analysis has a significant prognostic value for patients undergoing TOF repair.
4. Obtain chest X-ray.
An X-ray of the chest can reveal the heart and lung structure. A heart with a boot shape is seen on the X-ray due to right ventricle enlargement.
Interventions:
1. Assist patient in knee-chest position.
Children may squat to increase pulmonary blood flow and relieve dyspnea and cyanosis.
2. Prepare the patient for surgery.
Open heart surgery to repair the flaws or temporary shunts to enhance blood flow to the lungs are the surgical treatments for patients with tetralogy of Fallot.
3. Promote aerobic exercise.
Moderate exercise training increases aerobic capacity and gas exchange in people with corrected TOF.
4. Administer oxygen therapy as ordered.
A baby with severe cyanosis is given supplemental oxygen when initially diagnosed. In situations of lung compromise, the additional oxygen helps the infant’s oxygen levels. Oxygen therapy produces pulmonary vasodilation and systemic vasoconstriction. However, in children with Tetralogy of Fallot, consuming more oxygen has minimal impact on the child’s oxygen levels.
5. Administer prostaglandin therapy.
Prostaglandin treatment may be necessary for neonates with severe right ventricular outflow blockage who exhibit extreme hypoxemia and cyanosis to preserve ductal patency and pulmonary flow before surgery.
6. Collaborate with RT.
Respiratory therapists carry out prescribed breathing exercises and drug treatments. When choosing the best oxygen therapy for each patient, they are the specialist to consult.
Ineffective Tissue Perfusion
Tetralogy of Fallot results in structural defects that disrupt oxygenated blood reaching the rest of the body.
Nursing Diagnosis: Ineffective Tissue Perfusion
Related to:
- Insufficient oxygenated blood circulation
- Poor perfusion to tissues and organs
- Structural heart defects
- Mixed oxygenated and deoxygenated blood
As evidenced by:
- Altered blood pressure
- Decreased peripheral pulses
- Increased heart rate
- Dysrhythmias
- Decreased oxygen saturation
- Difficulty breathing
- Alteration in the level of consciousness
- Fainting
- Fatigue
- Activity intolerance
- Cold and clammy skin
- Prolonged capillary refill time
- Pale or cyanotic color of the membranes
- Clubbing to the fingers
Expected outcomes:
- Patient will maintain peripheral pulses and capillary refill time within normal limits.
- Patient will not experience cyanosis or pallor to the skin or nails.
- Patient will verbalize no complaints of dizziness or fainting with activity.
Assessment:
1. Assess the cardiovascular status.
Due to structural heart defects, deoxygenated blood circulates to the rest of the body. High pressure to the right side of the heart may cause arrhythmias. A heart murmur (a “whooshing” sound from inadequate blood flow through the heart) may be heard on auscultation.
2. Assist the patient with echocardiography.
Echocardiography is the gold standard for identifying the location and number of ventricular septal defects, the structure and severity of right ventricular outflow obstruction, and any related abnormalities with the coronary arteries and aortic arch. This test will determine the defects that may affect tissue perfusion in the body.
Interventions:
1. Enhanced blood flow.
The following medications enhance blood flow and perfusion:
- Morphine and intravenous beta-blockers alleviate the right ventricular outflow blockage by relaxing the heart muscle
- Intravenous phenylephrine raises systemic afterload
- Digoxin and loop diuretics are effective pharmacological treatment options if heart failure develops
2. Administer IV fluids.
Administer intravenous fluids to enhance pulmonary and ventricular filling that can increase blood supply and tissue perfusion.
3. Anticipate a complete repair surgery.
In a complete repair, the aorta is repaired to the left ventricle, the restricted pulmonary tract is widened, and the hole between the ventricles is sealed with a patch. A complete repair relieves the right ventricle’s volume and pressure overload, minimizing cyanosis, reducing right ventricular hypertrophy, and decreasing the likelihood of arrhythmias. This surgery is typically carried out between 4-6 months of age, though it can be performed as early as three months.
4. Advise parents on symptom prevention.
Parents can help their children prevent symptoms and “Tet spells” by ensuring they are drinking enough fluids and not becoming dehydrated, not overexerting themselves, and adhering to their treatment plan.
5. Complete follow-up care.
Following a complete repair patients must follow up with a pediatric cardiologist regularly. Long-term complications after TOF repair include arrhythmias, leaking valves, and aneurysms. Adults should receive a cardiac evaluation every 1-2 years and tests such as ECGs, stress tests, and MRIs.
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
Related to:
- 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.
Expected outcomes:
- 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.
Assessment:
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.
Interventions:
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.
References
- 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
- National Center for Biotechnology Information. (2022, January 18). Tetralogy of Fallot – StatPearls – NCBI bookshelf. Retrieved February 2023, from https://www.ncbi.nlm.nih.gov/books/NBK513288/
