Ventricular arrhythmia in 80 patients.

<p> Five patients had >1 ventricular event.</p

Baseline characteristics of 80 patients with Marfan syndrome and <i>FBN1</i> mutation.

<p>± standard deviation or numbers (percentage). Mean </p

According to the revised Ghent nosology [17] we identified disease causality for 80 <i>FBN1</i> mutations, as missense mutations affecting/creating cysteine residues in 25 (30%), nonsense mutations in 15 (19%), inframe and out of frame deletion/insertions in 14 (18%), missense mutations affecting conserved residues of the EGF consensus sequence in 9 (11%), other missense mutations in 9 (11%), splice site mutations in 6 (8%), and missense mutations creating cysteine residues in a EGF consensus sequence in 2 (3%) [17].

<p>According to the revised Ghent nosology <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0081281#pone.0081281-Loeys1" target="_blank">[17]</a> we identified disease causality for 80 <i>FBN1</i> mutations, as missense mutations affecting/creating cysteine residues in 25 (30%), nonsense mutations in 15 (19%), inframe and out of frame deletion/insertions in 14 (18%), missense mutations affecting conserved residues of the EGF consensus sequence in 9 (11%), other missense mutations in 9 (11%), splice site mutations in 6 (8%), and missense mutations creating cysteine residues in a EGF consensus sequence in 2 (3%) <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0081281#pone.0081281-Loeys1" target="_blank">[17]</a>.</p

Baseline characteristics according to arrhythmia.

<p> ACE-I identifies angiotensin converting enzyme inhibitors; ARB, angiotensin-receptor blockers; HDL cholesterol, high-density lipoprotein cholesterol; LDL, low-density lipoprotein cholesterol, and nsVT, non-sustained ventricular tachycardia.</p><p>–Whitney test for continuous data and the Fisher's exact test for nominal and categorical data. Mann</p><p> Three patients received two or three different classes of drugs.</p

<i>FBN1</i> mutation characteristics according to arrhythmia.

<p>–Whitney test for continuous data and the Fisher's exact test for nominal and categorical data. Mann</p

ROC curve analysis identifies NT-proBNP serum levels >618 pg/ml as a threshold for increased risk of VTE.

<p>The area under the curve is .919 (95% confidence interval .850 to.988; <i>P</i><.001; upper panel). For better identification of cut-offs separating high and low risk, we separately display sensitivity and specificity (lower panel).</p

Kaplan–Meier curves indicate an increased cumulative risk for VTE depending on presence of nsVT (upper panel), of NT-proBNP serum levels >618 pg/ml (middle panel), and of <i>FBN1</i> gene mutation in exons 24–32 (lower panel).

<p>Kaplan–Meier curves indicate an increased cumulative risk for VTE depending on presence of nsVT (upper panel), of NT-proBNP serum levels >618 pg/ml (middle panel), and of <i>FBN1</i> gene mutation in exons 24–32 (lower panel).</p