Persistent Truncus Arteriosus: Twenty Years Experience in a Tertiary Care Center in Taiwan

存動脈幹：台灣一醫學中心之二十年經驗分析

孫麗娟(Li-Chuan Sun) ； 王主科(Jou-Kou Wang) ； 林銘泰(Ming-Tai Lin) ； 吳恩婷(En-Ting Wu) ； 呂立(Frank L. Lu) ； 呂鴻基(Hung-Chi Lue) ； 張重義(Chung-I Chang) ； 陳益祥(Yih-Sharng Chen) ； 邱英世(In-Su Chiu) ； 吳美環(Mei-Hwan Wu)

《Acta Paediatrica Taiwanica》 46卷1期 (2005/02) Pp. 6-10

https://doi.org/10.7097/APT.200502.0006

並列摘要

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 Al 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.

並列關鍵字

persistent truncus arterisus ； chromosome 22q11 ； deletion