法洛四联症患者的护理：文献系统综述。
完整摘要 · 中文译文
背景：法洛四联症（ToF）是最常见的发绀型先天性心脏病，发病率约为每3,500名活产婴儿中1例，占所有先天性心脏缺陷的7-10%。心脏外科和重症监护的进步使生存至成年的比例超过85%，导致成人 repaired 先天性心脏病患者群体不断增长，而在资源有限的环境中，还存在未经治疗或既往未确诊的成人患者。在此背景下，专科护理在整个连续护理过程中发挥着关键作用。 目的：系统评价现有关于法洛四联症患者护理的科学证据，特别关注术前和术后阶段的护理干预，以及不同临床环境所需的能力。 材料与方法：采用PRISMA方法学，于2025年11月至2026年3月期间在PubMed、Cochrane Library和CINAHL中进行系统性文献检索。检索策略为：care AND tetralogy of Fallot AND nurs*。在最初确定的56条记录中，去除重复后筛选出33篇文章。在应用纳入标准和采用乔安娜·布里格斯研究所（JBI）批判性评估清单进行方法学质量评价（纳入阈值≥75%）后，最终纳入4项研究。所选研究包括2篇病例报告和2篇叙述性综述。 结果：纳入的四项研究共同涵盖了法洛四联症（ToF）患者护理路径的主要阶段：新生儿术前稳定期（Tran et al., 2022）、儿科重症监护病房的术后管理（Forman et al., 2019）、未修复法洛四联症成人的护理管理（Araújo et al., 2017）以及既往未确诊法洛四联症的成人患者的围麻醉期评估（Edwards et al., 2025）。主要得出四个主题：将系统性临床监测作为预防并发症的策略；高发绀（Tet）发作的处理；使用标准化的NANDA-I、NIC和NOC分类体系实施护理程序；以及将家庭支持作为整体护理的重要组成部分。 讨论：对纳入研究的比较表明，在法洛四联症患者护理的核心原则方面，包括系统性监测、并发症预防、个体化护理和以家庭为中心的支持，各项研究具有高度一致性。这些原则根据护理阶段、患者年龄和临床环境进行调整。研究特别关注了法洛四联症成人患者的管理，无论是手术修复后还是既往未确诊者，这成为专科护理实践中日益重要的挑战。不断扩大的先天性心脏病成人人群要求护士具备临床评估、批判性思维和文化敏感性方面的高级能力。 结论：护士在法洛四联症患者的护理路径中发挥着关键且不可替代的作用。专科临床专业知识、循证系统方法、早期临床评估以及以家庭为中心的护理，构成了针对这一复杂疾病提供高质量护理的基石。应将在成人先天性心脏病护理领域发展专科教育路径视为优先事项，以确保为这一持续增长的患者群体提供安全、公平且高质量的护理。
完整摘要 · 英文原文
BACKGROUND: Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease, with an incidence of approximately 1 in 3,500 live births, accounting for 7-10% of all congenital heart defects. Advances in cardiac surgery and intensive care have increased survival into adulthood to over 85%, resulting in a growing population of adults with repaired congenital heart disease and, in resource-limited settings, adults with untreated or previously undiagnosed disease. In this context, specialist nursing care plays a pivotal role throughout the continuum of care. OBJECTIVE: To systematically review the available scientific evidence on nursing care for patients with tetralogy of Fallot, with particular emphasis on nursing interventions during the preoperative and postoperative phases and on the competencies required across different clinical settings. MATERIALS AND METHODS: A systematic literature review was conducted between November 2025 and March 2026 using PubMed, the Cochrane Library, and CINAHL, following the PRISMA methodology. The search strategy was: care AND tetralogy of Fallot AND nurs*. Of the 56 records initially identified, 33 articles were screened after duplicate removal. Following the application of the eligibility criteria and methodological quality assessment using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist, with an inclusion threshold of ≥75%, four studies were included in the review. The selected studies comprised two case reports and two narrative reviews. RESULTS: The four included studies collectively addressed the major stages of the care pathway for patients with ToF: neonatal preoperative stabilization (Tran et al., 2022), postoperative management in the pediatric intensive care unit (Forman et al., 2019), nursing management of adults with unrepaired ToF (Araújo et al., 2017), and peri-anesthetic assessment of an adult patient with previously undiagnosed ToF (Edwards et al., 2025). Four principal themes emerged: systematic clinical monitoring as a strategy for preventing complications; management of hypercyanotic (Tet) spells; implementation of the nursing process using the standardized NANDA-I, NIC, and NOC taxonomies; and family support as an integral component of comprehensive nursing care. DISCUSSION: Comparison of the included studies demonstrated strong consistency regarding the core principles of nursing care for patients with ToF, including systematic monitoring, prevention of complications, individualized care, and family-centered support. These principles are adapted according to the stage of care, patient age, and clinical setting. Particular attention emerged regarding the management of adults with ToF, whether surgically repaired or previously undiagnosed, representing an increasingly important challenge for specialist nursing practice. The expanding population of adults with congenital heart disease requires nurses with advanced competencies in clinical assessment, critical thinking, and cultural sensitivity. CONCLUSIONS: Nurses play a key and irreplaceable role in the care pathway of patients with tetralogy of Fallot. Specialist clinical expertise, an evidence-based systematic approach, early clinical assessment, and family-centered care constitute the cornerstones of high-quality nursing care for this complex condition. The development of specialized educational pathways in adult congenital cardiology nursing should be considered a priority to ensure safe, equitable, and high-quality care for this continuously growing patient population.