From the Guidelines
The prognosis for congenital truncus arteriosus is significantly improved with modern surgical techniques, but it remains a serious heart defect requiring lifelong care, with long-term survival into adulthood now common, though life expectancy may still be somewhat reduced compared to the general population 1. The standard surgical approach involves separating the pulmonary arteries from the truncal vessel, closing the ventricular septal defect, and creating a connection between the right ventricle and pulmonary arteries using a conduit. However, these patients will need multiple operations throughout life as the conduits do not grow with the child and eventually deteriorate. Some key points to consider in the management of truncus arteriosus include:
- Regular follow-up with a cardiologist with expertise in adult congenital heart disease (ACHD) is necessary, with particular emphasis on the function of the systemic ventricle, maintenance of sinus rhythm, and function of the pulmonary conduit or prosthesis 1.
- Lifelong cardiology follow-up is essential to monitor for complications such as conduit stenosis or insufficiency, arrhythmias, heart failure, and truncal valve problems.
- The quality of life can be good, though many patients have exercise limitations and may need medications such as ACE inhibitors, beta-blockers, or diuretics to manage heart function.
- Early diagnosis, prompt surgical intervention, and consistent specialized cardiac care are the key factors that improve long-term outcomes. It is also important to note that pulmonary hypertension may be present in repaired patients, and unifocalization of the pulmonary arteries may be necessary in very complex cases, as stated in the 2018 AHA/ACC guideline for the management of adults with congenital heart disease 1.
From the Research
Prognosis for Congenital Truncus Arteriosus
The prognosis for congenital truncus arteriosus is generally poor without surgical intervention, with most patients dying within the first year of life 2. However, with advances in medical technology and surgical techniques, the outlook for patients with this condition has improved.
Survival Rates
- The intention-to-treat survival rate for patients with truncus arteriosus communis (TAC) who undergo surgery is around 86%, while the overall survival rate is approximately 55% 3.
- A study on early primary repair of TAC reported a hospital mortality rate of 3.4% and a 6-year actuarial survival rate of 93% 4.
- Without surgical treatment, patients with truncus arteriosus rarely survive beyond the first year of life, although some cases of prolonged survival without intervention have been reported 2.
Factors Affecting Prognosis
- The presence of associated cardiac and extracardiac anomalies, as well as genetic syndromes, can affect the prognosis for patients with truncus arteriosus 3.
- The development of pulmonary hypertension and congestive heart failure can also impact the prognosis 2.
- Early surgical intervention is crucial for improving long-term outcomes, and a coordinated multidisciplinary approach from prenatal diagnosis to postnatal care is essential for optimizing results 3.
Long-term Outcomes
- Patients with truncus arteriosus who undergo surgical repair may require repeated interventions, such as percutaneous balloon dilation or conduit replacement, to manage complications such as right ventricular outflow tract obstruction or aortic valve regurgitation 4.
- Long-term follow-up studies are necessary to monitor potential complications and guide future management strategies 3.