Nicole Nagdyman1, Siegrun Mebus1, Johanna Kügel1, Reinhart Zachoval2, Dirk-André Clevert3, Siegmund Lorenz Braun4, Guido Haverkämper5, Bernd Opgen-Rhein5, Felix Berger5,6, Sophia Horster2, Jörg Schoetzau1, Claudia Pujol Salvador1, Ulrike Bauer7, John Hess1, Peter Ewert1,8, Harald Kaemmerer1. 1. Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. 2. Department of Gastroenterology and Hepatology, Ludwig-Maximilians-University Munich, Munich, Germany. 3. Department of Interdisciplinary Ultrasound Center, Ludwig-Maximilians-University Munich, Munich, Germany. 4. Institute of Laboratory Medicine, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. 5. Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Centre Berlin and Charité Universitätsmedizin Berlin, Berlin, Germany. 6. DZHK (German Cardiovascular Research Centre), partner site Berlin, Berlin, Germany. 7. Competence Network for Congenital Heart Defects, Berlin, Germany. 8. DZHK (German Cardiovascular Research Centre), Munich Heart Alliance, Munich, Germany.
Abstract
BACKGROUND: 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. METHODS: 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)]. RESULTS: 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%). CONCLUSIONS: 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. 2019 Cardiovascular Diagnosis and Therapy. All rights reserved.
BACKGROUND: 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. METHODS: 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)]. RESULTS: 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%). CONCLUSIONS: 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. 2019 Cardiovascular Diagnosis and Therapy. All rights reserved.
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