Introduction

Truncus arteriosus (TA) is a congenital (developed from birth) heart condition in which there is one main artery that carries blood from the heart rather than two (normally, the pulmonary artery carries deoxygenated blood to the lungs and the aorta carries oxygenated blood to the rest of the body). Within the first month of birth (the neonatal period), babies present symptoms such as less efficient breathing and heart failure. However, neonatal repair is crucial in treating this defect.¹ Neonatal repair and other surgical interventions aim to separate the oxygenated and deoxygenated blood being carried from the heart to various organs to ensure that this blood does not mix. Neonatal repair is the most successful form of surgery in terms of lowering the number and severity of side effects that result from this condition.² Tubes known as conduits are used to redirect blood into the right ventricle, where the aorta is typically situated.

The outcomes are generally positive if surgical intervention takes place in the early stages, with quality of life being generally unaffected.³ Although a slightly delayed operation on the arterial trunk can lead to positive results, this is only possible when complications are not present, which is before adulthood in the majority of cases.⁴ This article is going to delve into how the quality of life differs between adults and children with TA who have undergone surgery. Future techniques and treatments for this condition will also be explored.

Understanding truncus arteriosus

This congenital heart defect arises when there is some sort of ventricular septal defect, which means that there is a hole between the left and right ventricles, thus leading to the oxygenated and deoxygenated blood mixing. This is also a result of issues regarding the aorticopulmonary trunk, such as with the pulmonary artery; thus, blood travelling from the heart to the rest of the body is not appropriately separated.⁵ Symptoms include cyanosis, in which the skin is discoloured (turns grey or blue) due to a lack of oxygenated blood as well as breathing difficulties. Heart murmurs, in which there are strange sounds when the heart is beating, also occur as the pulmonary arteries are under very high pressure, leading to hypertension.⁶

There are a few different types of surgical techniques that are utilised to treat instances of TA. These include aortic and pulmonary homografts in which a cadaver is used to carry out reconstruction of the ventricles. Cardiopulmonary bypasses use another blood vessel to effectively correct blood flow, and in neonatal repair, conduits redirect blood flow.⁷ 

Initially, treatments were experimental and different types of homografts and conduits were tested to correct blood flow in TA cases. More recently, neonatal repairs and interventions are possible due to the development of technology and a greater understanding of the condition. As a result, the prognosis of TA patients has improved over time.⁸

Quality of life: definition and key factors

Defining quality of life is difficult; from a medical perspective, the psychometric approach involves using different dimensions such as pain, function and social interactions to calculate the overall quality of life, in which physical and mental health can be separated. A different approach called decision theory does not assume that all issues are equal and is a more holistic approach in evaluating the quality of life of people suffering from TA.⁹ Due to respiratory difficulties and other issues, patients with congenital heart defects often have a poorer quality of life regarding physical function when compared to people without these conditions.¹⁰ There are also psychological issues, such as anxiety and depression, resulting from this condition, especially due to those with these conditions struggling to live a normal life in some instances. Hospitalisation over a prolonged period can also lead to depression, which is a major concern for those affected.¹¹

There are not many studies regarding educational attainment and how this is affected by congenital heart diseases; however, there is evidence suggesting that these diseases can lead to improper brain development. This is due to symptoms such as cyanosis and treatments such as bypasses. There will be more studies in the future on this subject.¹² Heart defects also lead to a lower overall exercise capacity and tolerance compared to people without these conditions.¹³

Quality of life in children with repaired truncus arteriosus

Children who have undergone TA repairs do have a generally lower quality of life for many reasons. Firstly, many children who undergo TA repairs are treated using conduits with a small diameter, and this requires reintervention in the future as the child grows over time. This is impractical and carries some risks.¹⁴ Many children also have lower growth rates, and developmental stages do not occur at the same rates as children without TA.¹⁵ Some studies have also demonstrated that the amount of oxygen that can be taken up during exercise is low. However, performance is not significantly affected.¹⁶ Depression and anxiety are also likely, regardless of the repairs, as children with TA who exhibit chest pain can develop anxiety. It is important to know that this condition also interferes with school performance, and symptoms can limit exam

attainment in some instances.¹⁷ Some children are also advised not to take part in all sporting activities and may not choose to do so, which limits extracurricular activity participation as a whole. The family and friends of those affected must provide adequate support to ensure proper integration in curricular and extracurricular activities.¹⁸

Quality of life in adults with repaired truncus arteriosus

This lower quality of life generally continues into adulthood for those affected by TA. There are some complications and long-term health effects associated with surgery, such as pulmonary hypertension, particularly in those treated later in life. Pulmonary hypertension also leads to potential failure in other treatment methods, such as utilising homografts.¹⁹ Due to aspects of life such as physical and social function being limited, the mental health of individuals suffering from TA can worsen; support systems must be readily available to help adults navigate these issues.²⁰ There are also risks regarding employment, with some adults not being fit to work in a desired field or employers not wanting to risk employing those with heart defects. This affects TA patients socially and can impact their career prospects and inevitably, their relationships with people in general.²¹

Comparative analysis

There are some differences between children and adults affected by TA regarding their quality of life. Patients who are children often undergo more surgical reinterventions when compared to adults due to smaller conduits.²² Effects regarding mental health and physical health are similar, though children may find it more difficult in school compared to career prospects for adults. Neonatal procedures are generally effective and widely utilised for TA.¹

It is important to know that many deaths tend to take place within one year of a child being operated hence continuous monitoring following operations is crucial.²³ Over the years, there have been many new technological advancements regarding surgical interventions, such as the progression from homografts to neonatal reapers, which have improved patient outcomes.²⁴

Challenges and unmet needs

Although TA cases are managed, there are some challenges regarding the management and treatment of this condition. Firstly, there is a lack of research; the rarity of this condition means the long-term implications have not been widely studied. This has led to healthcare services not being able to adequately monitor the long-term health of patients in some instances. There was previously a gap in psychological help for the patients and families of those with TA.

However, there has been more of an emphasis on providing effective counselling in recent times, so patients are more aware of treatment options and outcomes.²⁵ People from a worse socioeconomic background tend to have lower accessibility to specialist cardiac care for congenital heart diseases such as TA. It is of utmost importance that this barrier becomes less of an issue over time.²⁶

Strategies to enhance quality of life

There are a few ways to increase the quality of life for those with TA. Utilising a multidisciplinary team to manage the various unique issues associated with TA - such as physiological issues - is important to optimise treatment. Geneticists are very useful alongside cardiac specialists. The need for educators, especially for younger patients, cannot be understated.²⁵ Due to physical activity being limited for those with TA, this can lead to obesity and a high body mass index (BMI). Therefore, it is important to encourage those affected to engage in some safe physical activities.²⁷ Implanting strategies to manage mental health, such as appropriate counselling and check-ups, is beneficial for patients suffering from mental health issues.²⁸ As mentioned, there have been developments in surgery types and treatment for TA due to better research, and this will hopefully improve patient outcomes over time.²⁴

Summary

To summarise, the quality of life for adults and children with repaired TA is affected negatively regarding physical and emotional health, alongside a major social impact that is often overlooked. There are no major differences in these effects regardless of age and all instances should be treated with equal severity. Even though there is some evidence to suggest that repaired TA may have a higher educational impact on children due to decreased attendance and academic performance, this will need to be further evaluated. Research into TA is rapidly improving and increasing awareness of issues associated with TA and management strategies will prove extremely beneficial to patients in the near future.