BACKGROUND: Marfan syndrome (MFS) is a variable, autosomal-dominant disorder of 
the connective tissue. In MFS serious ventricular arrhythmias and sudden cardiac 
death (SCD) can occur. The aim of this prospective study was to reveal 
underlying risk factors and to prospectively investigate the association between 
MFS and SCD in a long-term follow-up.
METHODS: 77 patients with MFS were included. At baseline serum N-terminal 
pro-brain natriuretic peptide (NT-proBNP), transthoracic echocardiogram, 12-lead 
resting ECG, signal-averaged ECG (SAECG) and a 24-h Holter ECG with time- and 
frequency domain analyses were performed. The primary composite endpoint was 
defined as SCD, ventricular tachycardia (VT), ventricular fibrillation (VF) or 
arrhythmogenic syncope.
RESULTS: The median follow-up (FU) time was 868 days. Among all risk 
stratification parameters, NT-proBNP remained the exclusive predictor (hazard 
ratio [HR]: 2.34, 95% confidence interval [CI]: 1.1 to 4.62, p=0.01) for the 
composite endpoint. With an optimal cut-off point at 214.3 pg/ml NT-proBNP 
predicted the composite primary endpoint accurately (AUC 0.936, p=0.00046, 
sensitivity 100%, specificity 79.0%). During FU, seven patients of Group 2 
(NT-proBNP ≥ 214.3 pg/ml) reached the composite endpoint and 2 of these patients 
died due to SCD. In five patients, sustained VT was documented. All patients 
with a NT-proBNP<214.3 pg/ml (Group 1) experienced no events. Group 2 patients 
had a significantly higher risk of experiencing the composite endpoint 
(logrank-test, p<0.001).
CONCLUSIONS: In contrast to non-invasive electrocardiographic parameter, 
NT-proBNP independently predicts adverse arrhythmogenic events in patients with 
MFS.