BNP and haematological parameters are markers of severity of Ebstein's anomaly: correlation with CMR and cardiopulmonary exercise testing.
OBJECTIVE
Ebstein's anomaly (EA) involves a displaced and dysplastic tricuspid valve resulting in an atrialized portion of the right ventricle and an enlargement of the functional right ventricle and right atrium. Biomarkers targeting heart failure such as brain natriuretic peptide (BNP) or haematological parameters [haemoglobin (Hb) and haematocrit (Hct)] are upregulated in states of pulmonary hypoperfusion. We hypothesized that decreased pulmonary perfusion dependent on the stage of right heart failure is a possible mechanism in EA, and that it can be correlated with cardiac magnetic resonance (CMR) parameters. The aim of this study was to investigate the relationship between BNP and haematological parameters with functional parameters from CMR and exercise testing in patients with EA.


RESULTS
Twenty-five patients with non-corrected EA were studied prospectively (mean age 26 ± 14 years). BNP level was increased (74 ± 127 ng/L), and in 16% markedly above the heart failure cut-off level of 100 ng/L. Hb and Hct were increased above normal levels in 20 and 24% of patients, respectively. BNP and Hct/Hb correlated with CMR [total right/left (R/L)-Volume-Index, right atrium-end-diastolic volume index (EDVi), functional right ventricle (fRV)-EDVi, fRV-ejection fraction (EF), tricuspid regurgitation, pulmonary artery flow, and left ventricular EF] and exercise testing [workload/kg, oxygen uptake (VO2), ventilatory response to carbon dioxide production (VE/VCO2), oxygen (O2) pulse, and heart rate reserve]. The higher BNP and haematological parameters, the higher was the disease severity and the more limited was the physical exercise capacity.


CONCLUSIONS
In this EA cohort, BNP levels and haematological parameters correlated well with functional data from CMR and exercise testing. The total R/L-Volume-Index and BNP, and to some extent hematological parameters, may be useful as prognostic markers in patients with EA.
Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Heart Center, UMG, German Center for Cardiovascular Research, partner site Goettingen, DZHK, Robert-Koch-Str. 40, Göttingen 37075, Germany.
Olga
O
Hösch
Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Heart Center, UMG, German Center for Cardiovascular Research, partner site Goettingen, DZHK, Robert-Koch-Str. 40, Göttingen 37075, Germany.
Thuy-Trang
TT
Ngyuen
Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Heart Center, UMG, German Center for Cardiovascular Research, partner site Goettingen, DZHK, Robert-Koch-Str. 40, Göttingen 37075, Germany.
Peter
P
Lauerer
Department of Cardiology and Pneumology, University Medical Center, Georg-August-University Göttingen, Göttingen, Germany DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany.
Andreas
A
Schuster
University of Nebraska Medical Center/Children's Hospital and Medical Center, Omaha, NE, USA.
Shelby
S
Kutty
Institution for Diagnostic and Interventional Radiology, University Medical Center, Georg-August-University Göttingen, Heart Center, Göttingen, Germany.
Wieland
W
Staab
DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany.
Christina
C
Unterberg-Buchwald
DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany Institution for Diagnostic and Interventional Radiology, University Medical Center, Georg-August-University Göttingen, Heart Center, Göttingen, Germany.
Jan M
JM
Sohns
Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Heart Center, UMG, German Center for Cardiovascular Research, partner site Goettingen, DZHK, Robert-Koch-Str. 40, Göttingen 37075, Germany DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany.
Thomas
T
Paul
DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany Institution for Diagnostic and Interventional Radiology, University Medical Center, Georg-August-University Göttingen, Heart Center, Göttingen, Germany.
Joachim
J
Lotz
Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Heart Center, UMG, German Center for Cardiovascular Research, partner site Goettingen, DZHK, Robert-Koch-Str. 40, Göttingen 37075, Germany DZHK, German Center for Heart Research, partner site Göttingen, Göttingen, Germany michael.steinmetz@med.uni-goettingen.de.
Michael
M
Steinmetz
D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood*; D002648:Child; D004437:Ebstein Anomaly / Q000503:physiopathology*; D005080:Exercise Test; D005260:Female; D006334:Heart Function Tests; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D011446:Prospective Studies; D012720:Severity of Illness Index
D015415:Biomarkers; D020097:Natriuretic Peptide, Brain
Brain natriuretic peptide; Cardiopulmonary exercise testing; Cardiovascular magnetic resonance imaging; Ebstein's anomaly