Nagdyman Nicole, Mebus Siegrun, Kügel Johanna, Zachoval Reinhart, Clevert Dirk-André, Braun Siegmund Lorenz, Haverkämper Guido, Opgen-Rhein Bernd, Berger Felix, Horster Sophia, Schoetzau Jörg, Salvador Claudia Pujol, Bauer Ulrike, Hess John, Ewert Peter, Kaemmerer Harald
Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany.
Department of Gastroenterology and Hepatology, Ludwig-Maximilians-University Munich, Munich, Germany.
Cardiovasc Diagn Ther. 2019 Oct;9(Suppl 2):S198-S208. doi: 10.21037/cdt.2019.07.10.
Adults with congenital heart disease and ventricular dysfunction are prone to liver congestion, leading to fibrosis or cirrhosis but little is known about the prevalence of liver disease in atrial switch patients. Liver impairment may develop due to increased systemic venous pressures. This prospective study aimed to assess non-invasively hepatic abnormalities in adults who underwent Senning or Mustard procedures.
Hepatic involvement was assessed non-invasively clinically by laboratory analysis, hepatic fibrotic markers, sonography, and liver stiffness measurements [transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)].
Overall, 24 adults who had undergone atrial switch operation (13 Senning, 11 Mustard; four female; median age 27.8 years; range 24-45 years) were enrolled. In liver stiffness measurements, only three patients had values within the normal reference. All other patients showed mild, moderate or severe liver fibrosis or cirrhosis, respectively. Using imaging and laboratory analysis, 71% of the subjects had signs of liver fibrosis (46%) or cirrhosis (25%).
Non-invasive screening for liver congestion, fibrosis or cirrhosis could be meaningful in targeted screening for hepatic impairment in patients with TGA-ASO. As expert knowledge is essential, patients should be regularly controlled in highly specialised centres with cooperations between congenital cardiologists and hepatologists.
患有先天性心脏病和心室功能障碍的成年人容易出现肝脏充血,导致肝纤维化或肝硬化,但关于心房调转术患者的肝脏疾病患病率知之甚少。肝功能损害可能由于体循环静脉压升高而发展。这项前瞻性研究旨在评估接受森宁或马斯塔德手术的成年人的肝脏非侵入性异常情况。
通过实验室分析、肝脏纤维化标志物、超声检查和肝脏硬度测量[瞬时弹性成像(TE)和声辐射力脉冲成像(ARFI)]对肝脏受累情况进行非侵入性临床评估.
总体而言,纳入了24例接受心房调转术的成年人(13例森宁手术,11例马斯塔德手术;4例女性;中位年龄27.8岁;范围24 - 45岁)。在肝脏硬度测量中,只有3例患者的值在正常参考范围内。所有其他患者分别表现为轻度、中度或重度肝纤维化或肝硬化。通过影像学和实验室分析,71%的受试者有肝纤维化(46%)或肝硬化(25%)的迹象。
对大动脉转位心房调转术患者进行肝脏充血、纤维化或肝硬化的非侵入性筛查,对于针对性筛查肝功能损害可能具有重要意义。由于专业知识至关重要,患者应在先天性心脏病专家和肝病专家合作的高度专业化中心定期接受检查。