Djoeke Wolff1, Joost P van Melle2, Tineke P Willems3, Beatrijs Bartelds1,4, Mark-Jan Ploegstra1, Hans Hillege5, Tjark Ebels6, Rolf M F Berger1. 1. Center for Congenital Heart Diseases, Department of Pediatric Cardiology/Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. 2. Center for Congenital Heart Diseases, Department of Cardiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. 3. Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. 4. Department of Pediatric Cardiology, Erasmus Medical Center, Rotterdam, the Netherlands. 5. Department of Epidemiology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands. 6. Center for Congenital Heart Diseases, Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.
Abstract
OBJECTIVE: N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients. METHODS AND RESULTS: Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012-2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61-264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjusted R2 = 0.615). CONCLUSIONS: N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function.
OBJECTIVE:N-terminal pro-brain natriuretic peptide has an established role in the diagnosis and prognosis of heart failure. In Fontan patients, this peptide is often increased, but its diagnostic value in this particular non-physiologic, univentricular circulation is unclear. We investigated whether N-terminal pro-brain natriuretic peptide represents ventricular function or other key variables in Fontan patients. METHODS AND RESULTS: Ninety-five consecutive Fontan patients ≥10 years old who attended the outpatient clinic of the Center for Congenital Heart Diseases in 2012-2013 were included. Time since Fontan completion was 16 ± 9 years. Median N-terminal pro-brain natriuretic peptide was 114 (61-264) ng/l and was higher than gender-and age-dependent normal values in 54% of the patients. Peptide Z-scores were higher in patients in NYHA class III/IV compared to those in class I/II, but did not correlate with ventricular function assessed by MRI and echocardiography, nor with peak exercise capacity. Instead, peptide Z-scores significantly correlated with follow-up duration after Fontan completion (p < 0.001), right ventricular morphology (p = 0.004), indexed ventricular mass (p = 0.001), and inferior caval vein diameter (p < 0.001) (adjusted R2 = 0.615). CONCLUSIONS:N-terminal pro-brain natriuretic peptide levels in Fontan patients correlate with functional class, but do not necessarily indicate ventricular dysfunction. Increased peptide levels were associated with a longer existence of the Fontan circulation, morphologic ventricular characteristics, and signs of increased systemic venous congestion. Since the latter are known to be key determinants of the performance of the Fontan circulation, these findings suggest increase in N-terminal pro-brain natriuretic peptide levels to indicate attrition of the Fontan circulation, independent of ventricular function.
Authors: Peter Kramer; Anastasia Schleiger; Marie Schafstedde; Friederike Danne; Johannes Nordmeyer; Felix Berger; Stanislav Ovroutski Journal: Front Cardiovasc Med Date: 2022-03-10