Paediatric Gastroenterology and Hepatology.
Paediatric Cardiology and Intensive Medicine, Hannover Medical School, Hannover, Germany.
Liver Transpl. 2018 Jun;24(6):820-830. doi: 10.1002/lt.25076.
In adults, cirrhotic cardiomyopathy (CCM) has a significant incidence and impact on liver transplantation. For pediatric liver transplantation (pLT), data on liver-induced cardiac changes are scarce, and in particular, the comparison between cirrhotic and noncirrhotic liver disease has not been investigated. We retrospectively evaluated cardiac changes associated with CCM by echocardiography and 12-lead electrocardiogram in 198 pLT-candidates (median age 4.1 years) 4.2 before and 12 months after pLT. Results were correlated with the stage of liver fibrosis and cholestasis before transplantation. The left ventricular end-diastolic diameter (LVIDd) z score, left ventricular mass z score, and left ventricular mass index were significantly higher in cirrhotic patients (-0.10 versus 0.98, P < 0.001; -1.55 versus -0.42, P = 0.001; 78.99 versus 125.64 g/m , P = 0.001, respectively) compared with children with noncirrhotic liver disease. Pathological z scores (>2SDS) for the LVIDd occurred more frequently in cirrhotic patients compared with patients with noncirrhotic liver disease (31/169 versus 1/29; P = 0.03) and were significantly associated with cholestasis. All observed cardiac changes were reversible 1 year after pLT. Pathological LVIDd z scores correlated highly with intensive care unit (ICU) stay (9.6 days versus 17.1 days, respectively, P = 0.002) but not with patient survival pre-LT or post-LT. In contrast to other studies, prolonged QTc time was not associated with liver cirrhosis in our patients. In conclusion, CCM-associated cardiac changes in pLT candidates with cirrhotic liver disease are frequent, mild, and associated with cholestasis and reversible after pLT. They may impact peritransplant care and posttransplant hospitalization time. Further prospective evaluation is warranted. In particular, for QTc time prolongation etiological factors, possible protective effects of ursodeoxycholic acid treatment and the use as a screening parameter for CCM should be verified. Liver Transplantation 24 820-830 2018 AASLD.
在成年人中，肝硬化性心肌病（CCM）的发病率和对肝移植的影响都很高。对于儿科肝移植（pLT），关于肝诱导的心脏变化的数据很少，特别是尚未对肝硬化和非肝硬化性肝病进行比较。我们通过超声心动图和 12 导联心电图回顾性评估了 198 名 pLT 候选者（中位年龄 4.1 岁）在 pLT 前 4.2 个月和后 12 个月的 CCM 相关心脏变化。结果与移植前肝纤维化和胆汁淤积的阶段相关。与非肝硬化性肝病患儿相比，肝硬化患者的左心室舒张末期直径（LVIDd）z 评分、左心室质量 z 评分和左心室质量指数明显更高（-0.10 与 0.98，P<0.001；-1.55 与-0.42，P=0.001；78.99 与 125.64 g/m，P=0.001）。与非肝硬化性肝病患儿相比，肝硬化患者的 LVIDd 病理 z 评分（>2SDS）更常见（31/169 与 1/29；P=0.03），且与胆汁淤积显著相关。pLT 后 1 年，所有观察到的心脏变化均是可逆的。病理 LVIDd z 评分与重症监护病房（ICU）住院时间密切相关（分别为 9.6 天和 17.1 天，P=0.002），但与 LT 前或 LT 后患者生存率无关。与其他研究不同的是，在我们的患者中，延长的 QTc 时间与肝硬化无关。总之，在患有肝硬化的 pLT 候选者中，CCM 相关的心脏变化很常见、轻微，与胆汁淤积有关，且在 pLT 后是可逆的。它们可能会影响移植前护理和移植后住院时间。需要进一步的前瞻性评估。特别是，应验证 QTc 时间延长的病因、熊去氧胆酸治疗的可能保护作用以及作为 CCM 筛查参数的使用。肝移植 24 820-830 2018 AASLD。
