title: Heart rate turbulence and deceleration capacity for risk prediction of serious arrhythmic events in Marfan syndrome.
abstract: OBJECTIVE
  Marfan syndrome (MFS) is associated with a substantial risk for ventricular arrhythmia and sudden cardiac death (SCD). We used heart rate turbulence (HRT) and deceleration capacity (DC), to evaluate the risk stratification for these patients.
  
  
  METHODS
  We enrolled 102 patients [45 male (44.1 %), age 40.5 ± 14.6 years] with MFS. Blood samples were obtained to determine N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. Transthoracic echocardiography studies were conducted to evaluate heart function parameters and a 24-h holter ECG was performed. An analysis of two HRT parameters, turbulence onset (TO) and turbulence slope (TS), and DC was performed. Therefore, optimal cut-off values were calculated. Primary endpoint was the combination of SCD, ventricular arrhythmia and arrhythmogenic syncope. Secondary endpoint was total mortality.
  
  
  RESULTS
  During a follow-up of 1145 ± 491 days, 12 (11.7 %) patients reached the primary and 8 (7.8 %) patients the secondary endpoint. Patients reaching the primary were significantly older, had a higher burden of premature ventricular complexes and NT-proBNP levels and lower values of LVEF, DC and HRT TS. Multivariate analysis identified NT-proBNP (HR 1.25, 95 % CI 1.01-1.56, p = .04) and the abnormal HRT (abnormal TS and/or TO (HR 7.04, 95 % CI 1.07-46.27, p = .04) as independent risk predictor of arrhythmogenic events.
  
  
  CONCLUSIONS
  Patients with Marfan syndrome are at risk for severe ventricular arrhythmias and SCD. Abnormal HRT parameters and NT-proBNP values are independent risk factors for arrhythmogenic events and SCD. The assessment of these tools may help predicting SCD patients with MFS.
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany. b.schaeffer@uke.de.
authors.forename: Benjamin N
authors.initials: BN
authors.lastname: Schaeffer
authors.affiliation: Department of Cardiology, University Heart Center, University Hospital Hamburg, Hamburg, Germany.
authors.forename: Meike
authors.initials: M
authors.lastname: Rybczynski
authors.affiliation: Department of Cardiology, University Heart Center, University Hospital Hamburg, Hamburg, Germany.
authors.forename: Sara
authors.initials: S
authors.lastname: Sheikhzadeh
authors.affiliation: Department of Cardiology, University Heart Center, University Hospital Hamburg, Hamburg, Germany.
authors.forename: Ruken Ö
authors.initials: RÖ
authors.lastname: Akbulak
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Moser
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Mario
authors.initials: M
authors.lastname: Jularic
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Doreen
authors.initials: D
authors.lastname: Schreiber
authors.affiliation: Department of Medical Biometry and Epidemiology, University Hospital Hamburg, Hamburg, Germany.
authors.forename: Anne
authors.initials: A
authors.lastname: Daubmann
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Willems
authors.affiliation: Department of Cardiology, University Heart Center, University Hospital Hamburg, Hamburg, Germany.
authors.forename: Yskert
authors.initials: Y
authors.lastname: von Kodolitsch
authors.affiliation: Department of Cardiology - Electrophysiology, University Heart Center, University Hospital Hamburg, Martinistr. 52, 20246, Hamburg, Germany.
authors.forename: Boris A
authors.initials: BA
authors.lastname: Hoffmann
mesh_terms: D000328:Adult; D000367:Age Factors; D001145:Arrhythmias, Cardiac / Q000453:epidemiology* / Q000209:etiology; D016757:Death, Sudden, Cardiac / Q000453:epidemiology*; D003654:Deceleration; D004452:Echocardiography / Q000379:methods; D015716:Electrocardiography, Ambulatory / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate / Q000502:physiology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D008875:Middle Aged; D015999:Multivariate Analysis; D020097:Natriuretic Peptide, Brain / Q000378:metabolism; D010446:Peptide Fragments / Q000378:metabolism; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D018879:Ventricular Premature Complexes / Q000453:epidemiology / Q000209:etiology
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: Heart rate turbulence; Marfan syndrome; Risk stratification; Sudden cardiac death