Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.
Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.
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患有左心发育不全和左心室功能障碍的先天性膈疝患者死亡率最高。
DOI：
10.1016/j.jpeds.2024.114061
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发表时间：
2024
期刊：
影响因子：
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通讯作者：
Avitabile,CatherineM
中科院分区：
文献类型：
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作者：
Fraga,MaríaV;Hedrick,HollyL;Rintoul,NatalieE;Wang,Yan;Ash,Devon;Flohr,SabrinaJ;Mathew,Leny;Reynolds,Tom;Engelman,JennyL;Avitabile,CatherineM
目的描述先天性膈疝 (CDH) 婴儿的左心室 (LV) 功能障碍和左心发育不全 (LHH) 的范围，确定与 CDH 严重程度的关联，并评估体外膜肺氧合 (ECMO) 和左心疾病类别死亡的几率。研究设计从 2017 年 1 月至 5 月的单中心、回顾性 CDH 患者队列中收集人口统计学和临床变量2022 年。前瞻性地对初始超声心动图进行了 LV 功能和 LHH 的定量测量。 LHH 定义为以下各项中的 ≥2 项：任何左心结构的 zscore ≤ −2 或 LV 舒张末期容积 <3 mL。左室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%。暴露被操作为 4 组分类变量（左心室功能障碍 +/-、LHH +/-）。 Logistic 回归模型评估了 ECMO 与死亡的关联，并调整了 CDH 的严重程度。结果 纳入了 182 名患者（80.8% 左侧 CDH，63.2% 肝疝，23.6% ECMO，12.1% 死亡率）。 20% 的人左室功能正常但无 LHH（左室功能障碍-/LHH-），37% 的左室功能正常伴 LHH（左室功能障碍-/LHH+），14% 的左室功能障碍无 LHH（左室功能障碍+/LHH-），28% 的人左室功能障碍和 LHH 均存在（左室功能障碍+/LHH+）。左心疾病严重程度的增加、ECMO 的使用和死亡率之间存在剂量反应效应。在调整 CDH 严重程度后，左心室功能障碍 +/LHH + 婴儿使用 ECMO 和死亡的几率最高 [OR (95% CI)； ECMO 为 1.76 (1.20, 2.62)，死亡为 2.76 (1.63, 5.17)]。结论在我们的大型单中心队列中，左室功能障碍 +/LHH + + 的 CDH 患者使用 ECMO 和死亡的风险最高。
ObjectivesTo 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.Study designDemographic 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:zscore ≤ −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.ResultsOne 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].ConclusionsIn our large single-center cohort, patients with CDH with LV dysfunction+/LHH + had the highest risk of ECMO use and death.