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| Item Type: | Article |
|---|---|
| Title: | Risk factors for malignant ventricular arrhythmias in lamin A/C mutation carriers a European cohort study |
| Creators: |
van Rijsingen, I.A.W., Arbustini, E., Elliott, P.M., Mogensen, J., Hermans-van Ast, J.F., van der Kooi, A.J., van Tintelen, J.P., van den Berg, M.P., Pilotto, A., Pasotti, M., Jenkins, S., Rowland, C., Aslam, U., Wilde, A.A.M., Perrot, A.  ORCID: https://orcid.org/0000-0002-8800-342X, Pankuweit, S., Zwinderman, A.H., Charron, P. and Pinto, Y.M.
|
| Abstract: | OBJECTIVES: The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers. BACKGROUND: LMNA mutations cause a variety of clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many LMNA mutation carriers have a poor prognosis, because of a high frequency of MVA and progression to end-stage heart failure. However, it is unclear how to identify mutation carriers that are at risk for MVA. METHODS: In this multicenter cohort of 269 LMNA mutation carriers, we evaluated risk factors for MVA, defined as sudden cardiac death, resuscitation, and appropriate implantable cardioverter-defibrillator (ICD) treatment. RESULTS: In a median follow-up period of 43 months (interquartile range: 17 to 101 months), 48 (18%) persons experienced a first episode of MVA: 11 persons received successful cardiopulmonary resuscitation, 25 received appropriate ICD treatment, and 12 persons died suddenly. Independent risk factors for MVA were nonsustained ventricular tachycardia, left ventricular ejection fraction <45% at the first clinical contact, male sex, and non-missense mutations (ins-del/truncating or mutations affecting splicing). MVA occurred only in persons with at least 2 of these risk factors. There was a cumulative risk for MVA per additional risk factor. CONCLUSIONS: Carriers of LMNA mutations with a high risk of MVA can be identified using these risk factors. This facilitates selection of LMNA mutation carriers who are most likely to benefit from an ICD. |
| Keywords: | Cardiomyopathy, Implantable Cardioverter-Defibrillator, Lamin A/C, Risk Factors, Sudden Cardiac Death |
| Source: | Journal of the American College of Cardiology |
| ISSN: | 0735-1097 |
| Publisher: | Elsevier |
| Volume: | 59 |
| Number: | 5 |
| Page Range: | 493-500 |
| Date: | 31 January 2012 |
| Official Publication: | https://doi.org/10.1016/j.jacc.2011.08.078 |
| PubMed: | View item in PubMed |
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