Prospective risk stratification of sudden cardiac death in Marfan's syndrome
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Prospective risk stratification of sudden cardiac death in Marfan's syndrome. / Hoffmann, Boris A; Rybczynski, Meike; Rostock, Thomas; Servatius, Helge; Drewitz, Imke; Steven, Daniel; Aydin, Ali; Sheikhzadeh, Sara; Darko, Vivien; von Kodolitsch, Yskert; Willems, Stephan.
in: INT J CARDIOL, Jahrgang 167, Nr. 6, 10.09.2013, S. 2539-2545.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Prospective risk stratification of sudden cardiac death in Marfan's syndrome
AU - Hoffmann, Boris A
AU - Rybczynski, Meike
AU - Rostock, Thomas
AU - Servatius, Helge
AU - Drewitz, Imke
AU - Steven, Daniel
AU - Aydin, Ali
AU - Sheikhzadeh, Sara
AU - Darko, Vivien
AU - von Kodolitsch, Yskert
AU - Willems, Stephan
N1 - Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.
PY - 2013/9/10
Y1 - 2013/9/10
N2 - 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.
AB - 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.
KW - Adult
KW - Death, Sudden, Cardiac/epidemiology
KW - Female
KW - Follow-Up Studies
KW - Humans
KW - Male
KW - Marfan Syndrome/diagnostic imaging
KW - Middle Aged
KW - Prospective Studies
KW - Risk Assessment
KW - Ultrasonography
KW - Young Adult
U2 - 10.1016/j.ijcard.2012.06.036
DO - 10.1016/j.ijcard.2012.06.036
M3 - SCORING: Journal article
C2 - 22738784
VL - 167
SP - 2539
EP - 2545
JO - INT J CARDIOL
JF - INT J CARDIOL
SN - 0167-5273
IS - 6
ER -