Compound mutations in long QT syndrome assessed by a computer model

Eleonora Grandi, J. L. Puglisi, Donald M Bers, S. Severi

Research output: Chapter in Book/Report/Conference proceedingConference contribution

1 Scopus citations


Long QT syndrome (LQTS) is an electrical disorder that predisposes affected individuals to sudden death from cardiac arrhythmias. Recently, it has been shown that compound mutations in LQTS are more common than expected and cause a severe phenotype. We used a mathematical model of rabbit ventricular myocyte (LabHEART) to investigate the simultaneous effects of three compound mutations reported in LQTS patients. Our results show that the mutations prolong the action potential (AP), being the impact of compound mutations stronger than the additive effects of single ones. The user-friendly characteristic of LabHEART allows combining easily different levels of current alterations to evaluate their outcome. This feature makes it an invaluable tool for researchers who want to explore the effects of channel mutations on the AP waveform.

Original languageEnglish (US)
Title of host publicationComputers in Cardiology
Number of pages4
StatePublished - 2006
Externally publishedYes
Event2006 Computers in Cardiology, CIC - Valencia, Spain
Duration: Sep 17 2006Sep 20 2006


Other2006 Computers in Cardiology, CIC

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Software


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