PRESENTATION AND ATTRITION IN COMPLEX PULMONARY ATRESIA
PRESENTATION AND ATTRITION IN COMPLEX PULMONARY ATRESIA
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DOI：
10.1016/0735-1097(94)00364-v
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发表时间：
1995-02-01
影响因子：
24
通讯作者：
TY, E
中科院分区：
文献类型：
--
作者：
BULL, K;SOMERVILLE, J;TY, E
目标。这项研究总结了复杂性肺闭锁的表现和磨损模式。背景。评估复杂肺闭锁外科治疗策略的潜在影响需要了解患者发病前的年龄和生理学变化情况。我们对来自两个机构的218名先天性心脏病患者的发病年龄、转诊来源、肺动脉和侧枝解剖以及一生的手术史进行了回顾。大约65%的肺闭锁出现在婴儿期，其中50%的患者出现严重的青紫症状，25%的患者出现心力衰竭。与那些未确诊的患者相比，二次转诊至专科治疗的患者在第一次就诊时往往年龄较大，在根据他们的生存经验概括疾病的自然历史时必须小心。包括手术效果在内的总体精算生存率表明，婴儿期患者60%(95%可信区间[CL]43~73)存活到1周岁，1岁存活患者存活到10岁，16%(95%可信区间[CL]5~31)存活到35岁。新的手术方法通常应用于婴儿期以后，根据患者在这种疾病的最大磨损期的存活率来选择他们。除非在有症状的婴儿中成功应用，否则我们不能认为这些连续和复杂的手术会对大多数复杂肺闭锁患者的外观产生重大影响。
Objectives. This study summarized patterns of presentation and attrition in complex pulmonary atresia.Background. Assessment of the potential impact of surgical strategies for managing complex pulmonary atresia requires information about variability in age and physiology at preseotation of the condition.Methods. We performed a retrospective review of age at presentation, referral source, pulmonary artery and collateral anatomy and surgical history of 218 patients from two institutions dealing with congenital heart disease throughout life.Results. Approximately 65% of pulmonary atresia appears in infancy, with 50% of patients severely symptomatic from cyanosis and 25% from heart failure. Compared with those presenting undiagnosed, patients referred secondarily for specialist management tend to be older when first seen, and care must be taken when generalizing about the natural history of the condition from their survival experience. Overall actuarial survival, including the effects of operation, suggests that 60% (95% confidence limits [CL] 43 to 73) of patients presenting in infancy survive to their first birthday, 65% (95% CL 51 to 74) of those alive at 1 year old survive to the age of 10, and 16% (95% CL 5 to 31) of those alive at 10 years old survive to age 35.Conclusions. Novel surgical approaches have generally been applied beyond infancy in patients selected by their survival through the period of greatest attrition for this disease. Unless successful application in symptomatic infants is demonstrated, we cannot assume that these serial and complicated operations will have a major impact on the outlook of most patients with complex pulmonary atresia.