Long QT syndrome: an emerging role for inflammation and immunity
The Long QT Syndrome (LQTS), classified as congenital or acquired, is a multi-factorial disorder of myocardial repolarization predisposing to life-threatening ventricular arrhythmias, particularly torsades de pointes. In the latest years inflammation and immunity have been increasingly recognized as...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2015-05-01
|
| Series: | Frontiers in Cardiovascular Medicine |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/Journal/10.3389/fcvm.2015.00026/full |
| _version_ | 1811294471730495488 |
|---|---|
| author | Pietro Enea eLazzerini Pier Leopoldo eCapecchi Franco eLaghi-Pasini |
| author_facet | Pietro Enea eLazzerini Pier Leopoldo eCapecchi Franco eLaghi-Pasini |
| author_sort | Pietro Enea eLazzerini |
| collection | DOAJ |
| description | The Long QT Syndrome (LQTS), classified as congenital or acquired, is a multi-factorial disorder of myocardial repolarization predisposing to life-threatening ventricular arrhythmias, particularly torsades de pointes. In the latest years inflammation and immunity have been increasingly recognized as novel factors crucially involved in modulating ventricular repolarization. In the present paper we critically review the available information on this topic, also analyzing putative mechanisms and potential interplays with the other etiologic factors, either acquired and inherited.Accumulating data indicate inflammatory activation as a potential cause of acquired LQTS. The putative underlying mechanisms are complex but essentially cytokine-mediated, including both direct actions on cardiomyocyte ion channels expression and function, and indirect effects resulting from an increased central nervous system sympathetic drive on the heart. Autoimmunity represents another recently arising cause of acquired LQTS. Indeed, increasing evidence demonstrates that autoantibodies may affect myocardial electric properties by directly cross-reacting with the cardiomyocyte and interfering with specific ion currents as a result of molecular mimicry mechanisms. Intriguingly, recent data suggest that inflammation and immunity may be also involved in modulating the clinical expression of congenital forms of LQTS, possibly triggering or enhancing electrical instability in patients who already are genetically predisposed to arrhythmias. In this view, targeting immuno-inflammatory pathways may in the future represent an attractive therapeutic approach in a number of LQTS patients, thus opening new exciting avenues in antiarrhythmic therapy. |
| first_indexed | 2024-04-13T05:18:03Z |
| format | Article |
| id | doaj.art-0489e79160164298bf084900bba041d7 |
| institution | Directory Open Access Journal |
| issn | 2297-055X |
| language | English |
| last_indexed | 2024-04-13T05:18:03Z |
| publishDate | 2015-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cardiovascular Medicine |
| spelling | doaj.art-0489e79160164298bf084900bba041d72022-12-22T03:00:50ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2015-05-01210.3389/fcvm.2015.00026137646Long QT syndrome: an emerging role for inflammation and immunityPietro Enea eLazzerini0Pier Leopoldo eCapecchi1Franco eLaghi-Pasini2University of Siena, SienaUniversity of Siena, SienaUniversity of Siena, SienaThe Long QT Syndrome (LQTS), classified as congenital or acquired, is a multi-factorial disorder of myocardial repolarization predisposing to life-threatening ventricular arrhythmias, particularly torsades de pointes. In the latest years inflammation and immunity have been increasingly recognized as novel factors crucially involved in modulating ventricular repolarization. In the present paper we critically review the available information on this topic, also analyzing putative mechanisms and potential interplays with the other etiologic factors, either acquired and inherited.Accumulating data indicate inflammatory activation as a potential cause of acquired LQTS. The putative underlying mechanisms are complex but essentially cytokine-mediated, including both direct actions on cardiomyocyte ion channels expression and function, and indirect effects resulting from an increased central nervous system sympathetic drive on the heart. Autoimmunity represents another recently arising cause of acquired LQTS. Indeed, increasing evidence demonstrates that autoantibodies may affect myocardial electric properties by directly cross-reacting with the cardiomyocyte and interfering with specific ion currents as a result of molecular mimicry mechanisms. Intriguingly, recent data suggest that inflammation and immunity may be also involved in modulating the clinical expression of congenital forms of LQTS, possibly triggering or enhancing electrical instability in patients who already are genetically predisposed to arrhythmias. In this view, targeting immuno-inflammatory pathways may in the future represent an attractive therapeutic approach in a number of LQTS patients, thus opening new exciting avenues in antiarrhythmic therapy.http://journal.frontiersin.org/Journal/10.3389/fcvm.2015.00026/fullAutoantibodiesCytokinesImmunityInflammationLong QT SyndromeAnti-Ro/SSA |
| spellingShingle | Pietro Enea eLazzerini Pier Leopoldo eCapecchi Franco eLaghi-Pasini Long QT syndrome: an emerging role for inflammation and immunity Frontiers in Cardiovascular Medicine Autoantibodies Cytokines Immunity Inflammation Long QT Syndrome Anti-Ro/SSA |
| title | Long QT syndrome: an emerging role for inflammation and immunity |
| title_full | Long QT syndrome: an emerging role for inflammation and immunity |
| title_fullStr | Long QT syndrome: an emerging role for inflammation and immunity |
| title_full_unstemmed | Long QT syndrome: an emerging role for inflammation and immunity |
| title_short | Long QT syndrome: an emerging role for inflammation and immunity |
| title_sort | long qt syndrome an emerging role for inflammation and immunity |
| topic | Autoantibodies Cytokines Immunity Inflammation Long QT Syndrome Anti-Ro/SSA |
| url | http://journal.frontiersin.org/Journal/10.3389/fcvm.2015.00026/full |
| work_keys_str_mv | AT pietroeneaelazzerini longqtsyndromeanemergingroleforinflammationandimmunity AT pierleopoldoecapecchi longqtsyndromeanemergingroleforinflammationandimmunity AT francoelaghipasini longqtsyndromeanemergingroleforinflammationandimmunity |