Persistent truncus arteriosus: twenty years experience in a tertiary care center in Taiwan.
BACKGROUND
Persistent truncus arteriosus (PTA) is a rare congenital heart disease. The disease spectrum and outcome in the Oriental are still unclear.


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.
Sun
Li-Chuan
LC
Department of Pediatrics, National Taiwan University Hospital and National Taiwan University, College of Medicine, Taipei, Taiwan.
Wang
Jou-Kou
JK
Lin
Ming-Tai
MT
Wu
En-Ting
ET
Lu
Frank L
FL
Lue
Hung-Chi
HC
Chang
Chung-I
CI
Chen
Yih-Sharng
YS
Chiu
In-Su
IS
Wu
Mei-Hwan
MH
D000367:Age Factors; D001013:Aorta, Thoracic / Q000002:abnormalities / Q000601:surgery; D006328:Cardiac Catheterization; D002675:Child, Preschool; D003331:Coronary Vessels / Q000601:surgery; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000601:surgery; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D005791:Patient Care; D016019:Survival Analysis; D013624:Taiwan / Q000453:epidemiology; D016896:Treatment Outcome; D014339:Truncus Arteriosus, Persistent / Q000145:classification / Q000453:epidemiology / Q000601:surgery*