Williams J M, de Leeuw M, Black M D, Freedom R M, Williams W G, McCrindle B W
Division of Cardiology, University of Toronto, The Hospital for Sick Children, Ontario, Canada.
J Am Coll Cardiol. 1999 Aug;34(2):545-53. doi: 10.1016/s0735-1097(99)00227-2.
The purpose of this study was to identify trends and factors associated with outcomes of persistent truncus arteriosus (PTA).
Although there have been significant improvements, PTA continues to be associated with significant morbidity and mortality.
We undertook a review of all consecutive cases of PTA (n = 205) presenting at our institution from 1953 to 1997. Data were collected regarding demographics, anatomy, management (surgical palliation and repair) and outcomes (mortality and reoperation).
Significant trends (p < or = 0.001) related to groups defined by year of birth were as follows: number of cases (1953-1967, n = 13; 1968-1977, n = 42; 1978-1987, n = 69; 1988-1997, n = 81), median age at first assessment (8 months, 42 days, 7 days and 2 days, respectively), proportion who did not have any surgery (58%, 27%, 22% and 11%), proportion who had an initial palliative procedure (25%, 37%, 6% and 2%), proportion who underwent PTA repair (31%, 59%, 72% and 88%), median age at PTA repair (11.2 years, 1.1 years, 1.6 months and 12 days) and proportion dying before hospital discharge after repair (50%, 63%, 56% and 41%). Since 1995, mortality before hospital discharge after repair has further decreased to 2/11 (18%). Increasing time to initial conduit replacement in hospital survivors was significantly related to larger sized conduit at repair (p = 0.02) and use of pulmonary homografts (vs. aortic homografts or xenografts; p = 0.002). Interventional catheterization to address conduit obstructions significantly increased conduit longevity.
Significant improvements in PTA outcomes are evident with trends toward earlier age at assessment and complete repair.
本研究旨在确定与永存动脉干（PTA）预后相关的趋势和因素。
尽管已有显著改善，但PTA仍与较高的发病率和死亡率相关。
我们回顾了1953年至1997年在我院就诊的所有连续性PTA病例（n = 205）。收集了有关人口统计学、解剖结构、治疗（手术姑息治疗和修复）及预后（死亡率和再次手术）的数据。
与按出生年份定义的组相关的显著趋势（p≤0.001）如下：病例数（1953 - 1967年，n = 13；1968 - 1977年，n = 42；1978 - 1987年，n = 69；1988 - 1997年，n = 81），首次评估时的中位年龄（分别为8个月、42天、7天和2天），未接受任何手术的比例（分别为58%、27%、22%和11%），接受初始姑息治疗的比例（分别为25%、37%、6%和2%），接受PTA修复的比例（分别为31%、59%、72%和88%），PTA修复时的中位年龄（分别为11.2岁、1.1岁、1.6个月和12天）以及修复后出院前死亡的比例（分别为50%、63%、56%和41%）。自1995年以来，修复后出院前的死亡率进一步降至2/11（18%）。住院存活者中首次更换管道的时间增加与修复时使用更大尺寸的管道显著相关（p = 0.02）以及使用肺动脉同种异体移植物（与主动脉同种异体移植物或异种移植物相比；p = 0.002）。用于解决管道阻塞的介入性导管插入术显著延长了管道寿命。
PTA预后有显著改善，表现为评估年龄提前和完全修复的趋势。
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