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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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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.
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.
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.
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.
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