TY - JOUR
T1 - Angiopoietin-2 predicts morbidity in adults with Fontan physiology
AU - Shirali, Aditya S.
AU - Lluri, Gentian
AU - Guihard, Pierre J.
AU - Conrad, Miles B.
AU - Kim, Helen
AU - Pawlikowska, Ludmila
AU - Boström, Kristina I.
AU - Iruela-Arispe, M. Luisa
AU - Aboulhosn, Jamil A.
N1 - Funding Information:
We would like to thank Rachel Bolanos, Gregory Petersen, Soraya Sadeghi, and Dr. Jana Tarabay for their efforts in enrolling patients in this study, and the study coordinators at UCSF HHT Center of Excellence and UCSF Center for Cerebrovascular Research, Melissa Dickey and Diana Guo, for providing plasma samples from HHT patients with PAVMs. This work was supported by the Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research at UCLA Clinical Fellowship Training Program (AS), by the Streisand Endowed Chair (JAA) in Clinical Cardiology at UCLA, and NIH grants HL81397 and HL112839 (KIB) at UCLA, and by R01 NS034949 (HK) and U54 NS065705 (Michael Lawton) at UCSF.
Publisher Copyright:
© 2019, The Author(s).
PY - 2019/12/1
Y1 - 2019/12/1
N2 - Morbidity in patients with single-ventricle Fontan circulation is common and includes arrhythmias, edema, and pulmonary arteriovenous malformations (PAVM) among others. We sought to identify biomarkers that may predict such complications. Twenty-five patients with Fontan physiology and 12 control patients with atrial septal defects (ASD) that underwent cardiac catheterization were included. Plasma was collected from the hepatic vein and superior vena cava and underwent protein profiling for a panel of 20 analytes involved in angiogenesis and endothelial dysfunction. Ten (40%) of Fontan patients had evidence of PAVM, eighteen (72%) had a history of arrhythmia, and five (20%) were actively in arrhythmia or had a recent arrhythmia. Angiopoietin-2 (Ang-2) was higher in Fontan patients (8,875.4 ± 3,336.9 pg/mL) versus the ASD group (1,663.6 ± 587.3 pg/mL, p < 0.0001). Ang-2 was higher in Fontan patients with active or recent arrhythmia (11,396.0 ± 3,457.7 vs 8,118.2 ± 2,795.1 pg/mL, p < 0.05). A threshold of 8,500 pg/mL gives Ang-2 a negative predictive value of 100% and positive predictive value of 42% in diagnosing recent arrhythmia. Ang-2 is elevated among adults with Fontan physiology. Ang-2 level is associated with active or recent arrhythmia, but was not found to be associated with PAVM.
AB - Morbidity in patients with single-ventricle Fontan circulation is common and includes arrhythmias, edema, and pulmonary arteriovenous malformations (PAVM) among others. We sought to identify biomarkers that may predict such complications. Twenty-five patients with Fontan physiology and 12 control patients with atrial septal defects (ASD) that underwent cardiac catheterization were included. Plasma was collected from the hepatic vein and superior vena cava and underwent protein profiling for a panel of 20 analytes involved in angiogenesis and endothelial dysfunction. Ten (40%) of Fontan patients had evidence of PAVM, eighteen (72%) had a history of arrhythmia, and five (20%) were actively in arrhythmia or had a recent arrhythmia. Angiopoietin-2 (Ang-2) was higher in Fontan patients (8,875.4 ± 3,336.9 pg/mL) versus the ASD group (1,663.6 ± 587.3 pg/mL, p < 0.0001). Ang-2 was higher in Fontan patients with active or recent arrhythmia (11,396.0 ± 3,457.7 vs 8,118.2 ± 2,795.1 pg/mL, p < 0.05). A threshold of 8,500 pg/mL gives Ang-2 a negative predictive value of 100% and positive predictive value of 42% in diagnosing recent arrhythmia. Ang-2 is elevated among adults with Fontan physiology. Ang-2 level is associated with active or recent arrhythmia, but was not found to be associated with PAVM.
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U2 - 10.1038/s41598-019-54776-w
DO - 10.1038/s41598-019-54776-w
M3 - Article
C2 - 31797976
AN - SCOPUS:85075994190
SN - 2045-2322
VL - 9
JO - Scientific Reports
JF - Scientific Reports
IS - 1
M1 - 18328
ER -