目标:尽管有几十年的经验,法洛四联症合并肺动脉狭窄(TOF)的治疗仍存在争议.从业者必须考虑更新,用有限的数据不断发展的治疗策略来指导决策。因此,TOF临床实践标准委员会受美国胸外科协会的委托,提供了关于这一主题的框架,专注于干预措施的时机和类型,高危患者的管理,干预期间的技术考虑,以及评估干预措施结果的最佳实践。此外,该小组的任务是为未来的调查确定相关的研究问题。人们认识到,机构经验的可变性可能会影响该框架在临床实践中的应用。
方法:TOF临床实践标准委员会是一家跨国公司,具有TOF专业知识的多学科心脏病专家和外科医生小组。在医学图书管理员的协助下,在PubMed中进行引文搜索,Embase,Scopus,和WebofScience是使用与TOF及其管理相关的关键字进行的;搜索仅限于英语和2000年或以后。与肺动脉闭锁有关的文章,肺动脉瓣缺失,房室间隔缺损,成人TOF患者被排除在外,以及评论文章等非主要来源。这产生了近20,000个结果,其中包括163个。更多地考虑了最近的研究,更大的研究,和那些使用随机化或倾向评分匹配的比较组。使用改进的德尔菲法开发了具有建议类别和证据水平的专家共识声明,要求80%的成员投票,每份声明有75%的同意。
结果:在无症状婴儿中,在3至6个月之间进行完整的手术矫正是合理的,以减少住院时间,不良事件发生率,需要一个跨环贴片。在大多数有症状的新生儿中,姑息治疗和原发性完全手术矫正都是有用的治疗选择.考虑低出生体重或早产的人是合理的,小的或不连续的肺动脉,染色体异常,其他先天性异常,或其他合并症,如颅内出血,脓毒症,或其他终末器官受损作为高风险患者。在这些高危患者中,姑息治疗可能是首选;并且,在解剖结构适合的患者中,基于导管的手术可能比手术缓解更有利。
结论:正在进行的研究将进一步深入了解基于导管的干预措施的作用。为了完成手术矫正,经心房和经心室入路都是有效的;然而,应尽可能使用最小的脑室切开术。如果可能,肺动脉瓣应该幸免;如果无法挽救,可以考虑重建。手术结束时,应确认右室流出道梗阻的充分缓解,和确定一个显著的固定的解剖阻塞应提示进一步干预。鉴于我们目前的知识和发现的差距,我们提出了几个关键问题,由未来的研究和潜在的TOF注册表来回答:何时减轻或继续进行完整的手术矫正,以及理想的姑息治疗类型;完全修复的最佳手术方法,以实现右心室功能的最佳长期保留;以及实用性,功效,各种肺动脉瓣保存和重建技术的耐久性。
Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice.
The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert
consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement.
In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both
palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients,
palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical
palliation.
Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of
palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.