Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study.
Long-term functional health status and exercise test variables for patients with pulmonary atresia with intact ventricular septum: a Congenital Heart Surgeons Society study.
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室间隔完整的肺动脉闭锁患者的长期功能健康状况和运动测试变量：先天性心脏病外科医生协会的一项研究。
DOI：
10.1016/j.jtcvs.2012.11.092
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Mc
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文献类型：
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作者：
Karamlou,Tara;Poynter,JeffreyA;Walters3rd,HenryL;Rhodes,Jonathan;Bondarenko,Igor;Pasquali,SaraK;Fuller,StephanieM;Lambert,LindaM;Blackstone,EugeneH;Jacobs,MarshallL;Duncan,Kim;Caldarone,ChristopherA;Williams,WilliamG;Mc
对于室间隔完整的肺动脉闭锁（PAIVS）患者，在修复途径的选择方面存在有利于双心室（BV）修复的偏倚。我们试图确定的影响，移动边界候选人的BV路线在晚期功能健康状况（FHS）和运动能力（EC）。METHODS在1987年和1997年之间，448名新生儿PAIVS被纳入一个多机构的研究。晚期EC和FHS在修复后（平均14年）使用标准化运动试验和3个经验证的FHS仪器进行评估。评价了FHS、EC、形态学和3种终末状态（即BV、单心室[UV]或1.5心室修复[1.5V]）之间的关系。与年龄和性别匹配的正常对照组相比，参与者在身体功能领域的FHS评分较低（P <0.001），但在几乎所有的心理社会领域得分均显着较高。1.5V修复患者的EC更高（P = .02），而BV修复患者的离散FHS测量值更高。所有组的峰值耗氧量均较低，且与较大的初始三尖瓣z评分呈正相关（P < .001），BV修复组内的效果增强。CONCLUSIONSLate患者感知的物理FHS和测量的EC减少，无论PAIVS修复途径如何，PAIVS患者认为他们做得很好，尽管存在重要的物理障碍。对于初始三尖瓣z评分较小的患者，BV修复术的生存率可能以运动能力的晚期缺陷为代价，强调边缘患者采用1.5V或UV修复策略可能获得更好的结局。
BACKGROUNDA bias favoring biventricular (BV) repair exists regarding choice of repair pathway for patients with pulmonary atresia with intact ventricular septum (PAIVS). We sought to determine the implications of moving borderline candidates down a BV route in terms of late functional health status (FHS) and exercise capacity (EC).METHODSBetween 1987 and 1997, 448 neonates with PAIVS were enrolled in a multi-institutional study. Late EC and FHS were assessed following repair (mean 14 years) using standardized exercise testing and 3 validated FHS instruments. Relationships between FHS, EC, morphology, and 3 end states (ie, BV, univentricular [UV], or 1.5-ventricle repair [1.5V]) were evaluated.RESULTSOne hundred two of 271 end state survivors participated (63 BV, 25 UV, and 14 1.5V). Participants had lower FHS scores in domains of physical functioning (P < .001) compared with age- and sex-matched normal controls, but scored significantly higher in nearly all psychosocial domains. EC was higher in 1.5V-repair patients (P = .02), whereas discrete FHS measures were higher in BV-repair patients. Peak oxygen consumption was low across all groups, and was positively correlated with larger initial tricuspid valve z-score (P < .001), with an enhanced effect within the BV-repair group.CONCLUSIONSLate patient-perceived physical FHS and measured EC are reduced, regardless of PAIVS repair pathway, with an important dichotomy whereby patients with PAIVS believe they are doing well despite important physical impediments. For those with smaller initial tricuspid valve z-score, achievement of survival with BV repair may be at a cost of late deficits in exercise capacity, emphasizing that better outcomes may be achieved for borderline patients with a 1.5V- or UV-repair strategy.