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.

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).

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.

A problem facing doctors treating adults with congenitally malformed hearts is that a significant number of these patients are lost for follow-up. The purpose of our study is to describe the medical history and clinical findings in a group of such adults that was lost for follow-up.

The Danish press ran a front-page story about adults with congenitally malformed hearts who were lost for follow-up. These patients were strongly advised to contact a center for congenital cardiac disease, and we examined all responding patients within four-weeks.

We carried out a structured interview, a clinical examination, echocardiography, and measured levels of N-terminal pro brain natriuretic peptide.

The number of responders was 147. Based on the diagnosis and the findings, further follow-up was scheduled for 52 (35.4%), either because of significant residual lesions, found in 32, or the risk of late complications, judged to be present in 20. Symptoms were present in 36.5% of patients scheduled for follow-up. The presence of a heart murmur was highly predictive of the need for further follow-up but the sensitivity was too low to recommend the use of auscultation as a screening test.

A large proportion of adults with congenitally malformed hearts who are lost for follow-up require regular assessment according to a modern standard. Symptoms, signs, and measurement of natriuretic peptide cannot replace full cardiological assessment. It is a challenge for centres treating adults with congenital heart disease to find the lost group of patient with significant cardiac malformations.

Our purpose was to evaluate the effect of a treatment over six months with bisoprolol on the surrogate parameters of N-Terminal-pro brain natriuretic peptide, subsequently to be described as brain natriuretic peptide, peak uptake of oxygen, and ventricular function assessed by magnetic resonance imaging in grown ups and adults who had undergone surgical correction of tetralogy of Fallot.

We designed a prospective, randomized, double-blind, placebo controlled trial. We enrolled 33 patients, aged 30.9 plus or minus 9.5 years in either class 1 or 2 of the grading of the New York Heart Association class with both levels of brain natriuretic peptide greater than 100 pg/ml and a reduced peak uptake of oxygen less than 25 ml/kg/min. During treatment with Bisoprolol, the levels of brain natriuretic peptide increased significantly from 206 plus or minus 95 to 341 plus or minus 250 pg/ml (p< 0.05), and those of atrial natriuretic peptide from 4117 plus or minus 1837 to 5340 plus or minus 2102 fmol/ml (p = 0.0005). These measures remained unchanged in the group of patients receiving the placebo. Peak uptake of oxygen did not differ significantly in either group, nor did treatment have any significant effect on right and left ventricular volumes and ejection fractions as determined by magnetic resonance imaging. The clinical state as judged within the grading system of the New York Heart Association was also unchanged by beta-blockade.

Beta blockade with Bisoprolol seems to have no beneficial effect on asymptomatic or mildly symptomatic patients with right ventricular dysfunction secondary to repaired tetralogy of Fallot with residual pulmonary regurgitation and/or stenosis.

Measurement of the serum B-type natriuretic peptide (BNP) level and more recently its precursor, N-terminal proBNP (NT-proBNP), has been advocated to facilitate the diagnosis of heart failure in the emergency department (ED). We sought to determine the potential impact of adding NT-proBNP testing to the routine evaluation of emergency patients with acute dyspnea.

This prospective cohort study enrolled a convenience sample of acutely dyspneic patients at a tertiary care ED. We excluded trauma patients and those under 30 years of age. Patients underwent standard evaluation, including radiography when indicated. At the point of final diagnosis and blinded to the NT-proBNP result, physicians documented the likelihood that heart failure accounted for the patient's acute dyspnea on a 7-point Likert scale, the data from which was subsequently collapsed to 3 categories for analysis purposes. The primary outcome was the agreement between clinical impression and the NT-proBNP assay classified using manufacturer-recommended, age-specific cut-offs. Newly proposed cut-offs from a recent study were also evaluated.

One hundred and twenty-nine patients making 139 ED visits were enrolled (median age 76 years; 59% admitted). The serum NT-proBNP assay was positive in 119 (86%, 95% confidence interval [CI] 80%–91%) cases, including 75% (43/57, 95% CI 62%–86%) of the cases that the treating physician felt were not caused by heart failure, and 86% (25/29, 95% CI 68%–96%) where the treating physician was unsure. The median NT-proBNP concentration was higher in patients clinically believed to have heart failure rather than pneumonia or chronic obstructive pulmonary disease; however, the ranges of these values overlapped extensively (median 4361 pg/mL; interquartile range [IQR] 2386–10877 v. 1651 pg/mL; IQR 370–4745, respectively).

There is high discordance between the clinical impression of treating physicians and NT-proBNP concentrations, notably in patients who are believed not to have heart failure. Although the reference standard of ED diagnosis is imperfect, the broad overlap in NT-proBNP concentrations suggests poor specificity in this target patient population. The introduction of routine ED NT-proBNP testing using the current cut-offs would be expected to result in substantial indirect costs from further diagnostic testing. It remains unclear whether the introduction of this diagnostic test would have a positive impact on clinically relevant patient outcomes.