Tetralogy of Fallot is one of the most common congenital heart diseases. Congenital heart diseases are ones which are as a result of the malformation of the heart’s septum, valves and the large arteries (Hayes-Lattin & Salmi, 2020). Tetralogy of Fallot comprises of four malformations which include pulmonary stenosis, ventricular septal defect, right ventricular hypertrophy and an overriding aorta (Smith et al., 2019). In pulmonary stenosis, there exists an obstruction in the right ventricle at the pulmonary valve or the narrowing of the pulmonary valve, which results to a decline in blood flow. The ventricular septal defect presents as an opening between the lower chambers of the heart that allows blood in the left and right ventricles to mix (van der Ven et al., 2019). Overriding aorta occurs as a shift of the aorta to the right side in manner that it is on top of the ventricular septal defect. The right ventricular hypertrophy presents as an increase in the right ventricle as an adaptational mechanism to pump blood with more energy against the pulmonary obstruction (Smith et al., 2019).
The cause of this condition has been linked to several causes such as genetic due to environmental exposure, chromosomal abnormality or a defect in the gene responsible for the development of the heart (Ali, 2015). In addition, other causes such as maternal use of alcohol leads to the development of fetal alcohol syndrome which is highly associates with tetralogy of Fallot, mothers who are on antiepileptic medication during pregnancy period and those with phenylketonuria are also more likely to give birth to babies with the malformation. However there remains no clear cause for tetralogy of Fallot as in most cases the condition occurs spontaneously with no attributable cause (Smith et al., 2019).
The most prevalent clinical feature for tetralogy of Fallot is cyanosis. The bluish discoloration occurs as a result of mixing of deoxygenated blood and the oxygenated blood through the ventricular septal defect (Wilson et al., 2019). The child appears blue in the extremities, particularly on the lips, toes, fingers, nose an ear. Secondly if not treated early, at the age of 2 years, the child develops significant finger clubbing which is a sign of cardiorespiratory problem (Wilson et al., 2019). Third the child is often seen assuming a squatting position after engaging in little activities which indicates exertional dyspnea. Overall, there is delayed growth and development which are mostly observed as delayed developmental milestones and stunted growth (Khan et al., 2019).
Evaluations for tetralogy of Fallot are based on the signs and symptoms of the baby, physical examination results, and tests and procedures. The physical exam entails assessing the cardiopulmonary system with stethoscopes, assessing for signs and symptoms related to heart failure and the general appearance (Ali et al., 2018). The diagnostic tests conducted include echocardiography, where the soundwaves are used to produce a live photo of the heart. During this test, it is possible to visualize the suspected defects on the structure of the heart through which a diagnosis for tetralogy of Fallot can be made upon the confirmation of the four defects (Ali et al., 2018). An EKG test is also conducted to assess the electrical activity of the heart and reveal various parameters such as the heart rates, regularity of the rates as well as in determining whether there is an enlargement of the right ventricle. A chest X-Ray helps visualize the structure in the chest including the heart. During this chest, the nurse or the doctor can visualize whether the heart is enlarged or if there are other signs such as pulmonary edema which might indicate that the patient has developed heart failure. A pulse oximetry is used to actively monitor the oxygen levels in the blood (Puri et al., 2017).
Nursing diagnosis: decreased cardiac output related to the structural impairment of the heart
Cardiac output is the total amount f blood that is pumped out of the heart per minute expressed in liters per minute. Although it is a common feature among the elderly due to the reduced compliance of the ventricles, it is also a common problem among people with congenital heart diseases such as tetralogy of Fallot, myocardial infarction, hypertension, valvular heart disease and electrolyte imbalances (Oliveira et al., 2016)
Assessment data: subjective
In most cases patients complain of difficulty in breathing upon engaging in any exercise activities, which in this case, the mother of a child with could report of the child stopping to breastfeed to gasp for air, exercise intolerance whereby the kid stops playing and takes a rest while squatting, fatigue, insomnia and reports of chest pain (Oliveira et al., 2016).
Assessment data: objective
Nursing assessment is crucial in order to help the nurse to distinguish problems related to decreased cardiac output. To begin with, the nurse should assess for skin color, temperature and moisture. A skin that is cold, pale and clammy is often associated with low oxygen levels and impaired tissue perfusion. More importantly, the nurse should assess for signs of alterations in the level of consciousness as decreased cerebral perfusion and hypoxia secondary to decreased cardiac output leads to signs of difficulties in concentrating, irritability and altered consciousness. Presence of weak pulses and slow capillary refill of more than 3 seconds indicates reduced stroke volume. Also, the nurse should assess the heart sounds for gallops and murmurs which could be indicative of structural defects in the heart. Moreover, a decreased urine output is indicative of the inability of the heart to supply the kidneys with sufficient blood for filtering. For this reason, the nurse should have a close monitoring of an input output chart to determine the extent of the decreased cardiac output and possible help prevent renal failure (Rojas Sánchez et al., 2016).
The desired outcome for this diagnosis is that the child should demonstrate normal and adequate cardiac output which will be evidenced by normal hemodynamics such as normal blood pressure, normal pulse rate, strength, volume and rhythm, ability to participate in physical activities without symptoms such as dyspnea, fatigue, chest pain or syncope.
Nursing diagnosis: activity intolerance, related to an imbalance in oxygen supply and demand possibly evidence by the need to rest after short period of play, exertional dyspnea, and abnormal heart rate
Activity intolerance can affect any person despite of their age or any other factor, it can be related to other factors such as physical impairment, cognitive impairment, pain, weakness in the muscles, insufficient sleep, prolonged bed rest, sedentary lifestyle, cardiac problems, respiratory problems as well as metabolic problems (Kılıç, 2017).
Nursing assessment: Subjective data
A patient with activity intolerance can verbalize of pain upon motion, general weakness of the body, shortness of breath upon exertion, chest tightness and altered consciousness. However, in a patient with tetralogy of Fallot, the most prominent subjective data include shortness of breath upon exertion, and feeling dizzy after engaging in little activities such as climbing stairs (Kılıç, 2017).
Nursing assessment: Objective data
Typically, a patient with TOF will have similar presentations as one with heart failure as the heart has generally been incapacitated to perform its functions in an optimal manner. For this reason, the nurse should assess for signs of elevated heart rates, elevated blood pressure, signs of shortness of breath such as labored breathing and use of accessory muscles when breathing, low oxygen saturation, changes in EKG, and frequent refusal to engage in physical activities (Pereira et al., 2016). In addition, the nurse should investigate the nutritional status of the patient as energy is required to engage in exercises therefore poor nutrition might be a possible cause for the activity intolerance. The nurse should also monitor and observe the patients sleep patterns. This is because inadequate sleep can significantly affect the activity level of a person therefore the nurse needs to identify any sleep deprivation for a probable cause of activity intolerance (Pereira et al., 2016).
Other nursing diagnoses for TOF include:
Impaired gaseous exchange related to altered pulmonary blood flow.
This is because the pulmonary stenosis impairs the movement of blood into the lung. For this reason, the nurse should place the child in a knee-chest position while monitoring oxygen saturation levels and arterial blood gases, knee-chest position, or squatting is a compensatory mechanism that can be utilized by the nurse to increase the peripheral vascular resistance in a child and ultimately reduce the magnitude of the left-to-right shunting across the sepal defect. The resulting effect is increased gaseous exchange and minimal mixing of oxygenated and the deoxygenated blood. Monitoring of arterial blood gasses helps in detecting any metabolic complication which might occur as the body tries to compensate for decreased oxygen in the blood or increased carbon dioxide in the blood.
Imbalanced nutrition inadequate for the child’s nutritional requirements related to decreased energy for sucking and chewing
As mentioned earlier, activity intolerance makes it hard for the child to breasfeed and engage in other feeding activities. On top of activity intolerance, other symptoms such as shortness of breath makes it difficult for the child to breastfeed continuously as they get tired. More importantly, parental anxiety due to the condition of the child can influence mild production and her ability to feed her child effectively. Also, the parent may have deficient knowledge about her child’s nutritional requirements due to the health condition, which might influence how the child gets fed (Ali, 2015).
Treatment and management
Tetralogy of Fallot, requires to be repaired with open heart surgery soon after birth or later during infancy. The aim of the surgical management is to repair the defects and to ensure that the heart returns to being as normal as possible (Puri et al., 2017). However, other treatment modalities including pharmacological can also be employed in the management of the condition. The pharmacological aspect of management entails the administration of propranolol at 1mg/kg four times a day to reduce pulmonary spasms while intravenous Prostaglandin E1 help increase pulmonary blood flow therefore improving the oxygenation of the arterial blood (van der Ven et al., 2019). The surgical management of the condition entails palliative surgery and corrective surgery. Traditionally, palliative surgery was conducted temporarily to help improve blood flow to the lungs. Later on in childhood, another surgical procedure was conducted to completely repair the condition. In modern days, children with tetralogy of Fallot often undergo full repair in their infancy period. The palliative surgery is aimed at improving blood flow as mean of enabling the child to grow strong as they prepare for the corrective surgery (van der Ven et al., 2019).
With the recent advances in healthcare and technology, the outlook for children with tetralogy of Fallot has become much better. However there still remains the need for long-term home-based care. For babies with the condition, feeding and nutrition can be a little stressful. For this reason, small but frequent meals that the baby can handle can significantly improve the baby’s outcomes. Moreover, supplemental nutrition is helpful for providing the baby with extra calories, vitamins and other nutrients that are required for recovery and other developmental nutritional needs (Smith et al., 2019).
In babies whose tetralogy of Fallot has not been corrected yet, tet spells are more often. Mothers should lower the anxiety or any predisposing stress which to help prevent the baby from having the spells. Activity restrictions may be required for some children, although the restrictions vary with each child. Children with tetralogy of Fallot also require frequent medical care such as hear checkups with pediatric cardiologists, routine health exams with pediatrician and adherence to medications as prescribed (Smith et al., 2019).
Being a common congenital heart disease encompassing four defects, tetralogy of Fallot ought to be diagnosed early and treatment initiated as soon as possible. Diagnostic techniques include EKG, echocardiography and chest X-rays. The condition primarily affects the cardiopulmonary system therefore a close monitoring of the relevant parameters is required. More importantly, the babies with the condition should undergo surgical management for correction of the defects and supportive care to help improve outcomes later in life.
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