ObjectiveTo evaluate the early- and mid-term outcomes of surgical repair of persistent truncus arteriosus in children in a single institution of China.MethodsThe clinical data of 27 consecutive patients with persistent truncus arteriosus undergoing surgical repair in Guangzhou Women and Children’s Medical Center from November 2009 to May 2018 were retrospectively reviewed. There were 14 males and 13 females. Median age was 3.0 months (range: 13 days -11 years), of whom 10 (37.0%) were older than 6 months. ResultsThere were three early deaths with a mortality of 11.1%. The main complications included VSD partial repair in 2 patients, complete atrioventricular block in one patient. The mean follow-up time was 24.5±19.3 months (range: 1–76 months). There were three late deaths, and two patients lost follow. Echocardiology showed seven patients of right heart outflow tract obstruction, including three in pulmonary artery trunk, and four of pulmonary artery branches. One patient showed moderate aortic valve regurgitation. None required re-intervention during the follow-up. Survival estimates for the entire cohort following surgery were both 76.1% (95%CI 59.2% to 92.9%) at 1 year and 5 years.ConclusionThe surgical repair of persistent truncus arteriosus (PTA) remains challenges. The early- and mid-term outcomes of surgical repair of persistent truncus arteriosus are acceptable. For older children with severe pulmonary artery hypertension and/or trunk valve regurgitation, the risk of death is still higher. Some children have the higher risk of late right heart obstructive lesions.
Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation resulting from the failed development or premature involution of the sixth aortic arch during embryogenesis, leading to a failure to establish a connection with the main pulmonary artery. Currently, there is a notable lack of consensus regarding the surgical management of UAPA in China. Drawing upon the latest clinical research, this consensus aims to summarize surgical approaches and techniques to improve the clinical management of UAPA patients and serve as a scientific reference for physicians specializing in pediatric cardiology and structural heart disease. This consensus aims to promote the standardization of UAPA diagnosis and treatment, thereby facilitating improved patient outcomes and long-term management, and stimulating the continuous development and innovation of surgical treatment for this condition in China.