Glucose-6-phosphatase gene mutations in 20 adult Japanese patients with glycogen storage disease type 1a with reference to hepatic tumors.

BACKGROUND

The increased survival of patients with congenital heart disease over the last three decades has been associated with improvements in diagnosis and treatment. This study aimed to evaluate therapeutic interventional catheterization, outcomes and complications of these procedures in neonates and premature infants.

METHODS

In this study, therapeutic catheterization procedures performed on neonates and premature infants with congenital heart disease at a university hospital between February 2000 and October 2019 were retrospectively evaluated.

RESULTS

A total of 322 procedures were performed on 279 neonates and 26 premature infants. Of the patients, 217 (67.4%) were male. The median age of the patients was 8 days (interquartile range [IQR] 2-20) and the median body weight was 3050 g (IQR 2900-3600). The most common procedures were balloon atrial septostomy, balloon aortic angioplasty, balloon pulmonary valvuloplasty and balloon aortic valvuloplasty (35.4%, 20.8%, 18.3% and 12.4% respectively). The most common diagnoses were transposition of the great arteries, coarctation of the aorta, pulmonary stenosis and aortic stenosis (26.7%, 19.3%, 15.2% and 11.5% respectively). Most procedures, 274 (85.1%), were successful. Complications were observed in 74 procedures (23%). Of these complications, 45 (14%) were minor and 29 (9%) were major. The most common complication was transient dysrhythmia (6.9%). There was no significant relationship between body weight, age and the rate of complications. However, longer procedure time and fluoroscopy time were associated with higher complication rates (p < 0.05). Four procedurerelated deaths were observed.

CONCLUSION

Procedure-related complications are higher in the neonatal period. Although the complication rate varies according to the type of procedure, longer fluoroscopy time and procedure duration are associated with an increased complication rate. Procedures performed with the right indications, appropriate equipment and by experienced teams will play a key role in reducing complication rates.