Division of Neonatology, The Children's Hospital of Philadelphia, Philadelphia, PA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania. Philadelphia, PA.
Department of Pediatric General Thoracic and Fetal Surgery, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania Philadelphia, Philadelphia, PA.
J Pediatr. 2024 Aug;271:114061. doi: 10.1016/j.jpeds.2024.114061. Epub 2024 Apr 16.
To describe the scope of left ventricular (LV) dysfunction and left heart hypoplasia (LHH) in infants with congenital diaphragmatic hernia (CDH), to determine associations with CDH severity, and to evaluate the odds of extracorporeal membrane oxygenation (ECMO) and death with categories of left heart disease.
Demographic and clinical variables were collected from a single-center, retrospective cohort of patients with CDH from January 2017 through May 2022. Quantitative measures of LV function and LHH were prospectively performed on initial echocardiograms. LHH was defined as ≥2 of the following: z score ≤ -2 of any left heart structure or LV end-diastolic volume <3 mL. LV dysfunction was defined as shortening fraction <28%, ejection fraction <60%, or global longitudinal strain <20%. The exposure was operationalized as a 4-group categorical variable (LV dysfunction +/-, LHH +/-). Logistic regression models evaluated associations with ECMO and death, adjusting for CDH severity.
One hundred eight-two patients (80.8% left CDH, 63.2% liver herniation, 23.6% ECMO, 12.1% mortality) were included. Twenty percent demonstrated normal LV function and no LHH (LV dysfunction-/LHH-), 37% normal LV function with LHH (LV dysfunction-/LHH+), 14% LV dysfunction without LHH (LV dysfunction+/LHH-), and 28% both LV dysfunction and LHH (LV dysfunction+/LHH+). There was a dose-response effect between increasing severity of left heart disease, ECMO use, and mortality. LV dysfunction+/LHH + infants had the highest odds of ECMO use and death, after adjustment for CDH severity [OR (95% CI); 1.76 (1.20, 2.62) for ECMO, 2.76 (1.63, 5.17) for death].
In our large single-center cohort, patients with CDH with LV dysfunction+/LHH + had the highest risk of ECMO use and death.
描述先天性膈疝（CDH）婴儿左心室（LV）功能障碍和左心发育不全（LHH）的范围，确定其与 CDH 严重程度的关系，并评估左心疾病各分类与体外膜肺氧合（ECMO）和死亡的相关性。
从 2017 年 1 月至 2022 年 5 月，对一家单中心回顾性 CDH 患者队列进行了人口统计学和临床变量的收集。在最初的超声心动图检查中，对 LV 功能和 LHH 进行了定量测量。LHH 定义为以下 2 种或以上情况：任何左心结构的 z 评分≤-2 或 LV 舒张末期容积<3ml。LV 功能障碍定义为缩短分数<28%，射血分数<60%或整体纵向应变<20%。暴露因素被操作化为 4 组分类变量（LV 功能障碍+/-, LHH +/-）。使用逻辑回归模型，在调整 CDH 严重程度后，评估与 ECMO 和死亡的相关性。
共纳入 1082 例患者（80.8%左侧 CDH，63.2%肝疝，23.6%ECMO，12.1%死亡率）。20%的患者表现为正常 LV 功能且无 LHH（LV 功能障碍-/LHH-），37%的患者表现为正常 LV 功能伴 LHH（LV 功能障碍-/LHH+），14%的患者 LV 功能正常但伴有 LHH（LV 功能障碍+/LHH-），28%的患者同时存在 LV 功能障碍和 LHH（LV 功能障碍+/LHH+）。随着左心疾病严重程度、ECMO 使用和死亡率的增加，存在剂量反应关系。在调整 CDH 严重程度后，LV 功能障碍+/LHH+婴儿的 ECMO 使用和死亡的几率最高[ECMO 使用的比值比（OR）（95%CI）；1.76（1.20，2.62），死亡的 OR（95%CI）；2.76（1.63，5.17）]。
在我们的大型单中心队列中，LV 功能障碍+/LHH+的 CDH 患者 ECMO 使用和死亡的风险最高。
