Siegrun Mebus1, Nicole Nagdyman1, Johanna Kügel1, Reinhart Zachoval2, Siegmund Lorenz Braun3, Guido Haverkämper4, Bernd Opgen-Rhein4, Felix Berger5, Sophia Horster2, Jörg Schoetzau1, Claudia Pujol Salvador1, Ulrike Bauer6, John Hess1, Peter Ewert1, Harald Kaemmerer7. 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. Institute of Laboratory Medicine, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. 4. Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Centre Berlin and Charité Universitätsmedizin Berlin, Berlin, Germany. 5. Department of Congenital Heart Defects and Pediatric Cardiology, German Heart Centre Berlin and Charité Universitätsmedizin Berlin, Berlin, Germany; DZHK (German Cardiovascular Research Centre), partner site Berlin, Berlin, Germany. 6. Competence Network for Congenital Heart Defects, Berlin, Germany. 7. Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Technical University of Munich (TUM), Munich, Germany. Electronic address: kaemmerer@dhm.mhn.de.
Abstract
BACKGROUND: Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. METHODS AND RESULTS: 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23-62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and cirrhosis and 1/10 patients. CONCLUSIONS: Adult Eisenmenger patients are at increased risk of hepatic impairment. Non-invasive screening could be helpful in detecting liver alterations. In our small series, however, TE could not detect fibrosis or cirrhosis in any affected patient, while ARFI was very reliable. Patients should be transferred to centres, where a multidisciplinary expert knowledge is available and a close collaboration between cardiologists and hepatologists exists.
BACKGROUND:Eisenmenger syndrome as a severe form of cyanotic congenital heart disease results in a complex multisystemic disorder. Due to increased systemic venous pressure and the inability to ensure systemic perfusion and metabolic requirements, the liver may develop congestion, fibrosis or cirrhosis. This study aimed to assess hepatic abnormalities in Eisenmenger patients non-invasively. METHODS AND RESULTS: 10 adults with Eisenmenger syndrome (six female; median age 44.2years; range 23-62years) were enrolled and hepatic involvement was assessed - using clinical assessment, laboratory analysis, hepatic fibrotic markers, abdominal sonography and liver stiffness measurements (transient elastography (TE) and acoustic radiation force impulse imaging (ARFI)). Using imaging and laboratory analysis, 60% (6/10) of the Eisenmenger patients had signs of liver fibrosis (5/10) or cirrhosis (1/10). While TE, however, showed no relevant liver abnormalities in any Eisenmenger patient, ARFI detected liver fibrosis in 5/10 and cirrhosis and 1/10 patients. CONCLUSIONS: Adult Eisenmenger patients are at increased risk of hepatic impairment. Non-invasive screening could be helpful in detecting liver alterations. In our small series, however, TE could not detect fibrosis or cirrhosis in any affected patient, while ARFI was very reliable. Patients should be transferred to centres, where a multidisciplinary expert knowledge is available and a close collaboration between cardiologists and hepatologists exists.
Authors: Florian P Reiter; Nino J Hadjamu; Nicole Nagdyman; Reinhart Zachoval; Julia Mayerle; Enrico N De Toni; Harald Kaemmerer; Gerald Denk Journal: Cardiovasc Diagn Ther Date: 2021-04