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Background  Marfan syndrome is associated with ventricular arrhythmia but risk factors including  FBN1  mutation characteristics require elucidation.    Methods and Results  We performed an observational cohort study of 80 consecutive adults (30 men, 50 women aged 42±15 years) with Marfan syndrome caused by  FBN1  mutations. We assessed ventricular arrhythmia on baseline ambulatory electrocardiography as >10 premature ventricular complexes per hour (>10 PVC/h), as ventricular couplets (Couplet), or as non-sustained ventricular tachycardia (nsVT), and during 31±18 months of follow-up as ventricular tachycardia (VT) events (VTE) such as sudden cardiac death (SCD), and sustained ventricular tachycardia (sVT). We identified >10 PVC/h in 28 (35%), Couplet/nsVT in 32 (40%), and VTE in 6 patients (8%), including 3 with SCD (4%). PVC>10/h, Couplet/nsVT, and VTE exhibited increased N-terminal pro–brain natriuretic peptide serum levels( P <.001). All arrhythmias related to increased NT-proBNP ( P <.001), where PVC>10/h and Couplet/nsVT also related to increased indexed end-systolic LV diameters ( P  = .024 and  P  = .020), to moderate mitral valve regurgitation ( P  = .018 and  P  = .003), and to prolonged QTc intervals ( P  = .001 and  P  = .006), respectively. Moreover, VTE related to mutations in exons 24–32 ( P  = .021). Kaplan–Meier analysis corroborated an association of VTE with increased NT-proBNP ( P <.001) and with mutations in exons 24–32 ( P <.001).    Conclusions  Marfan syndrome with causative  FBN1  mutations is associated with an increased risk for arrhythmia, and affected persons may require life-long monitoring. Ventricular arrhythmia on electrocardiography, signs of myocardial dysfunction and mutations in exons 24–32 may be risk factors of VTE.

Observational Cohort Study of Ventricular Arrhythmia in Adults with Marfan Syndrome Caused by FBN1 Mutations