Persistent truncus arteriosus: Twenty years experience in a tertiary care center in Taiwan
Journal
Acta Paediatrica Taiwanica
Journal Volume
46
Journal Issue
1
Pages
6-10+50
Date Issued
2005
Abstract
Background. Persistent truncus arteriosus (PTA) is a rare congenital heart disease. The disease spectrum and outcome in the Oriental are still unclear. Methods and Results. A total of 35 patients with PTA were identified from the Pediatric Cardiology Database of this institution, giving an incidence of 0.47%. According to the Van Praagh classification, we found type A1 in 16, A2 in 10, A3 in 4 and A4 in 5 patients. The most common truncal valves were still tricuspid (57.1%) and quadricuspid (28.5%), with the latter being associated with moderate to severe truncal regurgutation. Eleven patients had not received surgery and all died. We classified the era of operation as early (between 1980 and 1995) or late (1996-2001), and further classified the type of PTA as simple (type A1 or A2) or complex (interrupted aortic arch -A4, absent orifice of one PA from truncal root - A3 or moderate truncal valve insufficiency). The overall surgical mortality was 67%. Statistical analysis revealed that age at operation (older than 6 months), early operation era and complex PTA were risk factors for survival. Conclusions. PTA is a rare form of congenital heart disease in Taiwan and probably also in the Oriental. The surgical reparation seems to improve with experience. Early operation may prevent pulmonary vasculopathy. However, the results in complex PTA remain poor.
SDGs
Other Subjects
aorta arch; arterial trunk; article; clinical article; disease classification; female; human; incidence; male; mortality; outcomes research; risk factor; statistical analysis; survival; Taiwan; time series analysis; tricuspid valve; vascular disease; Age Factors; Aorta, Thoracic; Child, Preschool; Coronary Vessels; Female; Heart Catheterization; Heart Septal Defects, Atrial; Humans; Incidence; Infant; Infant, Newborn; Male; Patient Care; Survival Analysis; Taiwan; Treatment Outcome; Truncus Arteriosus, Persistent
Type
journal article