AUTHOR=Chaloupecký Václav , Jičínská Denisa , Tomek Viktor , Materna Ondřej , Gebauer Roman , Poruban Rudolf , Antonová Petra , Adla Theodor , Štefánek Matěj , Illinger Vojtěch , Kotaška Karel , Janoušek Jan 

TITLE=Impact of liver fibrosis and nodules formation on hemodynamics in young adults after total cavopulmonary connection. A magnetic resonance study

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=9

YEAR=2022

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2022.986653

DOI=10.3389/fcvm.2022.986653

ISSN=2297-055X

ABSTRACT=<sec><title>Background</title><p>The aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC).</p></sec><sec><title>Materials and methods</title><p>Adult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test.</p></sec><sec><title>Results</title><p>The cohort consisted of 39 patients (46% female) with a median age at study 26 (IQR 23–28) years and interval from TCPC 21 (IQR 20–23) years. There was no correlation between ELF test and any MRI variables, but procollagen III amino-terminal peptide (PIIINP), a single component of ELF test, correlated significantly with ventricular end-diastolic volume (<italic>r</italic> = 0.33; <italic>p</italic> = 0.042) and inferior vena cava flow (<italic>r</italic> = 0.47; <italic>p</italic> = 0.003). Fifteen (38%) patients with liver nodules had compared to other 24 patients higher end-diastolic volume (ml/m<sup>2</sup>) 102.8 ± 20.0 vs. 88.2 ± 17.7; <italic>p</italic> = 0.023, respectively. PIIINP correlated significantly with inferior vena cava flow (<italic>r</italic> = 0.56; <italic>p</italic> = 0.030) and with end-diastolic volume (<italic>r</italic> = 0.53; <italic>p</italic> = 0.043), but only in patients with liver nodules.</p></sec><sec><title>Conclusion</title><p>Gradual progression of liver fibrosis, particularly hepatic arterialization caused by liver nodules formation, increases inferior vena cava flow and subsequent ventricular volume overload may further compromise single ventricle functional reserve in adult patients after TCPC.</p></sec>