Department of Neonatology, Royal Hospital for Children, Glasgow, United Kingdom.
Department of Pediatric Surgery, McGovern Medical School at The University of Texas Health Science Center at Houston and Children's Memorial Hermann Hospital, Houston, Texas.
Am J Respir Crit Care Med. 2019 Dec 15;200(12):1522-1530. doi: 10.1164/rccm.201904-0731OC.
Congenital diaphragmatic hernia (CDH) is an anomaly with a high morbidity and mortality. Cardiac dysfunction may be an important and underrecognized contributor to CDH pathophysiology and determinant of disease severity. Our aim was to investigate the association between early, postnatal ventricular dysfunction and outcome among infants with CDH. Multicenter, prospectively collected data in the CDH Study Group (CDHSG) registry, abstracted between 2015 and 2018, were evaluated. Ventricular function on early echocardiograms, defined as obtained within the first 48 hours of life, was categorized into four hierarchical groups: normal function, right ventricular dysfunction only (RV), left ventricular dysfunction only (LV), and combined RV and LV dysfunction (RV&LV). Univariate, multivariate, and Cox proportional hazards regression analyses were performed. Cardiac function data from early echocardiograms were available for 1,173 (71%) cases and categorized as normal in 711 (61%), RV in 182 (15%), LV in 61 (5%), and combined RV&LV in 219 (19%) cases. Ventricular dysfunction was significantly associated with prenatal diagnosis, CDHSG stage, intrathoracic liver, and patch repair (all < 0.001). Survival varied by category: normal function, 80%; RV, 74%; LV, 57%; and RV&LV, 51% ( < 0.001). The adjusted risk of death (hazard ratio) for cases with LV was 1.96 (95% confidence interval [CI], 1.29-2.98; = 0.020) and for cases with RV&LV was 2.27 (95% CI, 1.77-2.92; = 0.011). All cardiac dysfunction categories were associated with use of extracorporeal membrane oxygenation ( < 0.005). Early ventricular dysfunction occurs frequently in CDH and is an independent determinant of severity and clinical outcome.
先天性膈疝 (CDH) 是一种发病率和死亡率均较高的畸形。心脏功能障碍可能是 CDH 病理生理学的一个重要且被低估的因素，也是疾病严重程度的决定因素。我们的目的是研究 CDH 患儿出生后早期心室功能障碍与结局之间的关系。对 2015 年至 2018 年期间 CDH 研究组 (CDHSG) 注册中心前瞻性收集的数据进行了评估。早期超声心动图（定义为出生后 48 小时内获得）的心室功能分为四个层次组：正常功能、仅右心室功能障碍 (RV)、仅左心室功能障碍 (LV) 和 RV 和 LV 联合功能障碍 (RV&LV)。进行了单变量、多变量和 Cox 比例风险回归分析。1173 例 (71%) 患儿的早期超声心动图心脏功能数据可用，其中 711 例 (61%) 正常、182 例 (15%) RV、61 例 (5%) LV 和 219 例 (19%) RV&LV。心室功能障碍与产前诊断、CDHSG 分期、胸腔内肝和补片修补术显著相关（均 < 0.001）。存活率按类别不同而异：正常功能为 80%；RV 为 74%；LV 为 57%；RV&LV 为 51%（ < 0.001）。LV 病例死亡风险（风险比）的调整值为 1.96（95%置信区间 [CI]，1.29-2.98； = 0.020），RV&LV 病例为 2.27（95% CI，1.77-2.92； = 0.011）。所有心脏功能障碍类别均与体外膜肺氧合（ECMO）的使用相关（ < 0.005）。CDH 患儿早期常出现心室功能障碍，是严重程度和临床结局的独立决定因素。
