From the Guidelines
The prognosis of transposition of the great vessels has dramatically improved with modern surgical interventions, with a survival rate exceeding 90% into adulthood, as reported in the most recent study 1. Without treatment, most infants with this condition would not survive beyond the first year of life. However, with early diagnosis and appropriate surgical correction, the outcome is significantly improved. The primary surgical approach is the arterial switch operation (ASO), ideally performed within the first few weeks of life, which involves switching the great arteries back to their normal positions and reimplanting the coronary arteries, as described in 1. Some key points to consider in the management and prognosis of transposition of the great vessels include:
- Long-term follow-up is essential as patients may develop complications including coronary artery stenosis, aortic root dilation, aortic valve regurgitation, and right ventricular outflow tract obstruction, as noted in 1.
- Most patients who undergo successful repair can expect to lead relatively normal lives with good exercise tolerance, though they require lifelong cardiac monitoring.
- Neurodevelopmental outcomes are generally favorable but may be affected by factors such as prolonged hypoxemia before repair, complications during surgery, or associated genetic syndromes.
- Women with repaired transposition of the great vessels can usually tolerate pregnancy well, though specialized cardiac obstetric care is recommended, as indicated in 1. It's also important to consider that the quality of life and health status of children after ASO are similar to those of normal children and significantly better than those of children who have undergone the atrial baffle procedure, as reported in 1. Overall, the prognosis for transposition of the great vessels is favorable with modern surgical interventions and appropriate long-term care, as supported by the evidence from 1.
From the Research
Prognosis of Transposition of the Great Vessels
The prognosis of transposition of the great arteries has improved significantly with the development of early medico-surgical strategies, including balloon atrioseptostomy, prostaglandin infusion, and the arterial switch operation within the first days of life 2.
Mortality and Complications
- Early postoperative mortality rates have been reported to be around 2.8% to 15.8% in different studies 3, 4, 5.
- Late complications of the arterial switch operation include coronary artery stenosis, neoaortic root dilation, neoaortic insufficiency, and neopulmonic stenosis 3.
- Risk factors for early death and late reintervention have been identified, including resection of left ventricular outflow tract obstruction, weight less than 2.5 kg, associated aortic arch obstruction, and the need for postoperative extracorporeal membrane oxygenation 5.
Long-term Outcomes
- Long-term outcomes of the arterial switch operation have been reported to be excellent, with freedom from at least moderate neoaortic valve regurgitation of 98.7% at 20 years 5.
- Patients with associated ventricular septal defect and aortic arch obstruction require close follow-up due to a higher risk of reintervention 5.
- The introduction of balloon atrial septostomy has dramatically improved early survival of infants with transposition of the great arteries, but there is still considerable attrition before definitive repair 6.