Bansal Neha, Mahgerefteh Joseph, Lamour Jacqueline M, Kogan-Liberman Debora, Ovchinsky Michelle, Ganzburg Kayla, Choueiter Nadine
Division of Pediatric Cardiology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Ave-R1, Bronx, NY, 10467, USA.
Division of Pediatric Hepatology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, USA.
Pediatr Cardiol. 2024 Nov 22. doi: 10.1007/s00246-024-03673-9.
Cirrhotic cardiomyopathy is a complication of cirrhosis resulting in cardiac dysfunction. It remains poorly characterized in children. The aim of this study was to assess relationship of pre-liver transplant (LT) conventional and novel parameters of biventricular function with post-LT clinical course. This is a retrospective study of pre-LT echocardiograms performed on patients < 18 years of age with cirrhosis at a single center, who received a LT. Demographic, clinical, and echocardiographic data were collected. Speckle tracking echocardiography (STE) analysis was performed by a single observer using TomTec system. Descriptive data were expressed as mean (SD) and number (%). The relationship between clinical data and echocardiographic variables were assessed using Spearman correlation coefficient. Significance was set at < 0.05. Thirty-five patients (median age 6.5; IQR 14.2 years) underwent LT between 2010 and 2020. Pre-LT diagnosis was biliary atresia in 14 (40%) patients and 7 (20%) patients were listed as status 1A/1B. Their median natural pediatric/model end-stage liver disease score was 13 (IQR 9). Their pre-LT echocardiogram showed normal left ventricular systolic (LV) function by ejection fraction and strain parameters. Right ventricular (RV) function was abnormal in 74% of patients as measured by RV GLS (23 ± 3%). There was correlation between echocardiographic parameters with pre-transplant clinical disease and post-operative LT course (length of stay and duration of mechanical ventilation). Children undergoing liver transplant have RV dysfunction as evidenced by abnormal RV GLS on STE. There is echocardiographic parameter correlation between clinical liver disease and post-LT clinical course. This evidence highlights the importance of using novel technology like STE in assessment of children undergoing evaluation for liver transplant.
肝硬化性心肌病是肝硬化的一种并发症，可导致心脏功能障碍。其在儿童中的特征仍不明确。本研究的目的是评估肝移植前（LT）双心室功能的传统参数和新参数与肝移植后临床病程的关系。这是一项对在单一中心接受肝移植的18岁以下肝硬化患者进行的肝移植前超声心动图回顾性研究。收集了人口统计学、临床和超声心动图数据。由一名观察者使用TomTec系统进行斑点追踪超声心动图（STE）分析。描述性数据以均值（标准差）和数量（百分比）表示。使用Spearman相关系数评估临床数据与超声心动图变量之间的关系。显著性设定为<0.05。2010年至2020年间，35例患者（中位年龄6.5岁；四分位间距14.2岁）接受了肝移植。肝移植前诊断为胆道闭锁的患者有14例（40%），7例（20%）患者被列为1A/1B状态。他们的中位自然儿科/终末期肝病模型评分是13（四分位间距9）。他们的肝移植前超声心动图显示，通过射血分数和应变参数评估，左心室（LV）收缩功能正常。通过右心室GLS（23±3%）测量，74%的患者右心室（RV）功能异常。超声心动图参数与移植前临床疾病及肝移植后病程（住院时间和机械通气持续时间）之间存在相关性。接受肝移植的儿童存在右心室功能障碍，STE上右心室GLS异常证明了这一点。临床肝病与肝移植后临床病程之间存在超声心动图参数相关性。这一证据凸显了使用STE等新技术评估接受肝移植评估儿童的重要性。
