April 2014
Volume 55, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2014
Cardiac Conduction in Leber’s Hereditary Optic Neuropathy
Author Affiliations & Notes
  • Starleen Elizabeth Frousiakis
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Rustum Karanjia
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Amitha K Ganti
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Andrew Pouw
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Milton Moraes
    Centro Universitario do Espirito Santo, Colatina, Brazil
    Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil
  • Solange Rios Salomao
    Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil
  • Rubens Belfort, Jr.
    Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil
  • Peter A Quiros
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Filipe Chicani
    Department of Ophthalmology, Federal University of Sao Paulo, Sao Paulo, Brazil
  • Alfredo A Sadun
    Ophthalmology, University of Southern California, Los Angeles, CA
  • Footnotes
    Commercial Relationships Starleen Frousiakis, None; Rustum Karanjia, None; Amitha Ganti, None; Andrew Pouw, None; Milton Moraes, None; Solange Salomao, None; Rubens Belfort, Jr., None; Peter Quiros, None; Filipe Chicani, None; Alfredo Sadun, None
  • Footnotes
    Support None
Investigative Ophthalmology & Visual Science April 2014, Vol.55, 6205. doi:
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      Starleen Elizabeth Frousiakis, Rustum Karanjia, Amitha K Ganti, Andrew Pouw, Milton Moraes, Solange Rios Salomao, Rubens Belfort, Jr., Peter A Quiros, Filipe Chicani, Alfredo A Sadun; Cardiac Conduction in Leber’s Hereditary Optic Neuropathy. Invest. Ophthalmol. Vis. Sci. 2014;55(13):6205.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose: To evaluate cardiac conduction in affected patients and carriers of Leber’s hereditary optic neuropathy (LHON), 11778 mutation, using electrocardiogram (ECG) from a large Brazilian cohort.

Methods: Patient populations were categorized by disease status: affected patients (n1=17) and carriers of the 11778 mutation (n2=43). Each population underwent ECG testing, performed by a cardiologist, with measurement of PR interval and QTc duration. Control measurements were obtained from a de-identified database and healthy volunteers (n3=33). An idealized curve of published data was also obtained from a previously published Italian cohort (Cameli, M. et al. Alcohol Clin Exp Res. 2009 Dec;33(12):2141-6). A Shapiro-Wilks test was used to compare the distributions of PR and QTc to a normal distribution. An unpaired student’s t-test was performed to determine if there was a difference between the groups.

Results: There was a significant decrease in the PR interval in the affected and carriers populations compared to controls. Mean PR intervals and standard deviations in control, affected and carrier populations were 151.2±19.0, 140.6±34.4 and 129.8±38.1 milliseconds, respectively. The Shapiro-Wilks test demonstrated that the carrier and affected PR intervals were non-normally distributed when compared to controls. Carrier distribution of PR interval demonstrated a positive skew (p<0.001), with a mode of 120 milliseconds; the lower limit of normal. Similarly, distribution of affected PR intervals demonstrated a positive skew (p<0.001). There was no significant difference detected in the QTc intervals in affected or carrier patients, relative to the control population. The difference between carrier and affected patients was also insignificant.

Conclusions: This study determined that affected and carrier populations of the LHON 11778 mutation had a non-normal distribution of PR interval. These findings suggest that there may be subclinical cardiac conduction changes in patients and carriers of LHON.

Keywords: 531 ganglion cells • 600 mitochondria • 613 neuro-ophthalmology: optic nerve  
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