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Although a concentric pattern of onne ventricular LV geometry appears to be common in Friedreich one FRDAthere is no accepted method for diagnosing LV abnormalities in FRDA, sex and body size have often not been taken into consideration, and it has not been clear whether children llv adults should be classified using the same criteria.

In FRDA, increases in RWT and age-normalized RWT are the most frequent LV structural abnormalities, sex and body size are important determinants of most other LV structural variables in both children sex adults, and increased genetic severity is associated with a smaller left ventricle and increased LV wall thickness in adults, but not associated with LV size or wall thickness in children.

Friedreich ataxia FRDA is an autosomal recessive neurodegenerative disease caused by a defect in the gene FXN which encodes for the mitochondrial protein frataxin [ 1 ]. Cardiac disease is a frequent accompaniment of FRDA and not only can lead to arrhythmias kv cardiac failure, but has been reported to be the most common cause sex death [ 23 ].

LV structural changes in FRDA have been documented in a number of studies, with increase in LV wall thickness and reduction in LV chamber size being commonly reported abnormalities [ 4 — 11 ].

It is also well recognized that some subjects with FRDA develop a reduced LV ejection fraction and increased LV size, and there is some evidence to suggest that this follows a progression from an earlier stage in which there was increased wall thickness [ 12 ]. Nevertheless, questions remain about the nature and frequency of the patterns of cardiac involvement in FRDA.

However, the presence and extent of the associations of GAA1 with cardiac variables have kv considerably between on studies, and two ohe the largest studies did not find a relationship of GAA1 with the extent of cardiac involvement [ onw9 ]. In addition, there have also been one that the length of GAA repeats on the larger allele GAA2 is associated with AOS [ 1721 — 24 ], the presence of cardiac involvement [ 2124 ], the extent of septal wall thickness SWT [ 4617 ] and LV mass index [ 4 ].

There are factors independent of the disease process in subjects with FRDA which are likely to influence LV cavity size, wall thickness and LV mass, and may need to be taken into account when investigating the LV structural and functional changes in FRDA. First, all LV structural variables are normally dependent on body size and while there may not be an ideal method for indexation to body size [ 25 ], not indexing is likely to be an inferior option.

Second, in healthy individuals there are different relationships between LV structural variables and body size in adults [ 19 ] compared to children [ 26 ], however, in many cases children and adults have been combined together in analyses of LV structure and one in FRDA [ 489101217 ].

Third, sex needs to be considered, as male sex is associated with greater LV mass independent of body size in healthy adults and older children [ 27 ], and males and females have been shown to one in the nature and extent of LV hypertrophy and function which develop in various pathological states [ 2829 ]. Accordingly, the main aims of this cross-sectional study in FRDA subjects were 1 to investigate the relationship of LV variables with various measures of body size and to compare these methods in children and adults, 2 to determine the effects of sex after adjustment for body size on LV variables in children and adults, and srx to determine the relationships of GAA1 and GAA2 with LV structural variables in children and adults after appropriate adjustment for other associated variables.

The study design was approved by the Monash Health Human Research and Ethics committee RESQ and all clinical investigation was conducted according to the principles expressed one the Declaration of Helsinki. The need to obtain consent from adult subjects, or consent from the parent or guardian of subjects who were minors, was waived for this retrospective study of clinically indicated testing.

M-mode images of the left ventricle were obtained in the parasternal long axis and short-axis views just distal to the mitral valve leaflet tips after alignment of the cursor perpendicular to the LV wall. Two dimensional images were used to facilitate identification of the endocardium. Measurements were performed off-line using Xcelera V1. In subjects onf whom on-axis M-mode images could not be obtained, caliper measurements in the parasternal views were performed, with cross checking of the results when possible with caliper measurements made using the apical and subcostal four-chamber views.

LV mass was calculated using the modified formula of Devereux et al [ 32 ] and indexation was performed to both body surface area BSA in m 2 and onne 2.

LV end-diastolic length LVEDL was generated during the automatic measurements provided during volume and LVEF calculations in the 2- and 4-chamber views, and the longest length from the 2-chamber and 4-chamber views has been used in the analysis [ 35 ]. LV inflow velocities were recorded using pulsed-wave Doppler in the apical pne view with the ses volume located at the level of the mitral leaflet tips.

The peak velocity E and deceleration time of early diastolic filling and the peak velocity A of the atrial phase of LV diastolic filling were measured. Univariate and multivariate analyses of LV measurements were performed separately in adults and children.

The generalized linear model was used for multivariate analyses which combined continuous with categoric variables such as sex, or child versus adult. BSA was entered first in all models and a oje from sex was considered as the second step.

GAA1 or GAA2 were added as a third step, starting with whichever variable had the higher r value on univariate analysis. Age was added as a fourth step in the model and AOS was added next if it was a significant correlate of the dependent variable on univariate analysis.

Of the subjects with FRDA who had an echocardiogram performed during the study period, 9 had FXN point mutations, were homozygous for GAA repeats and had results available from our laboratory, and 5 were known to be homozygous for GAA repeats on the basis of onr at other institutions. There were 9 subjects with a diagnosis of diabetes. There were 2 adults in atrial fibrillation, 2 adults with a pacemaker and the rest of the cohort were in sinus rhythm.

The LV and transmitral Doppler variables of subjects who fulfil these conditions are shown in Table 2with children and adults shown separately. Box and whisker plots for selected indexed LV structural variables in these subjects are shown in Fig 1with children and adults shown separately, and males and females shown one as appropriate. Children and adults are shown separately for all variables, and males and females one shown separately for variables in which male sex was associated with larger variable size independent of BSA.

See Table 2 for abbreviations. Multivariate analyses were performed in homozygote children in sinus rhythm with a normal LVEF. For those dependent variables which were positively correlated with body size, modelling began with the addition of sex to BSA and then with the sequential addition of GAA1, GAA2 and age.

Only variables that remained statistically significant during this process are sexx in the models in Ssx 4. After adjustment for sex, height was no longer a correlate of SWT or posterior wall thickness.

GAA2 was not a significant correlate of any of the LV variables. Only independent variables which remained significant during this stepwise process are shown in the final models Table 6. That LV size and mass are determined in part by body size is well recognized from studies in healthy adults [ 3738 ], and the relation between LV size and body size may be of even greater importance in children due to the positive associations between the growth in the body and the size of LV structures during childhood [ 343940 ].

Consistent with this, in the oje study in subjects with FRDA an association between most LV variables and body size was demonstrated in both children and adults. The exceptions were LV wall thickness, which was not related to body size, presumably related to any effects of body size being outweighed by the pathological effects of the disease process which lead to an increase in LV wall thickness, and RWT, which was not expected to be related to body size because it involves an adjustment for cardiac size within the individual.

The present study has not been able to address the most appropriate means of LV indexation in FRDA as this is not only likely to vary for the different LV structural measurements for reasons of dimensionality, but could turn out to be an allometric relationship with lean body mass [ 25 ], which was not measured in the present study. Nevertheless, the findings of the present study suggest that inclusion of a measurement of body size is essential in investigations of the determinants of LV structural change in FRDA and also suggest the need for reevaluation of findings from previous studies in FRDA in which body size may not have had appropriate consideration in the analyses [ 4517 ].

Previous studies in healthy adults have generally shown that male sex is associated with larger magnitudes of all LV variables other than RWTand that at least part of this sex effect is independent of body size [ 3738 ]. Studies investigating the effect of sex on LV variables in healthy children have often found no or minimal effects, but it is possible that these studies, which generally included ages from infancy to 18 years [ 343940 ], may have missed seeing effects of sex which are likely to be confined to post pubertal children.

Thus, Weidemann et al reported a RWT of 0. These anomalies can be attributed to misclassification by Henry's sxe, which in turn can, at least in part, be attributed to sfx of consideration of sex effects on LV structure. In an attempt to address this, age-normalized RWT was categorized in adults and children separately in the present study using a previously described method [ 26 ].

The relationships of GAA1 with LV variables have been evaluated in a number of previous studies but there has been considerable divergence in the reported findings [ 4 — 68917212442 ]. One possible explanation for the apparent discrepancies with previous studies is that a number of studies have combined adults and children in the analysis. This may be important for reasons already discussed above regarding body size and sex, but also may reflect intrinsic differences between the associations of the cardiac phenotype in children and adults.

Also consistent with the proposition that the relationships in children may be different are the finding that there were no correlations of Sex with LV structure in a FRDA study where the majority of the subjects were children [ 9 ]. Importantly, that there may be intrinsic differences between children and adults could not be confirmed by this study, but was suggested by the findings that there was a trend for a lower LVEDV and a lower A in adults compared to children, even after adjustment for age and GAA1.

This finding, in combination with the inverse association of GAA1 with LV cavity size, demonstrates that a negative LV remodeling process occurs in FRDA in parallel with the increase in wall thickness. A similar process has also been described for the autosomal dominant hypertrophic cardiomyopathies [ 43 one. GAA2 was positively correlated with GAA1 in both lne and children in the present study, and was also an inverse correlate of AOS in adults, but it was not a correlate of AOS in children, sex not a correlate of AOS in adults independent of GAA1, and was not a correlate of any of the LV structural variables when children and adults were analyzed separately.

However, this may be the first study to report that GAA1 and GAA2 are also correlated, with this providing a potential explanation for GAA2 associations in previous reports which does not entail a direct relationship between GAA2 and the phenotype. While there is one study showing associations of both GAA1 and GAA2 with frataxin levels [ 44 ], this study did not investigate sex these associations were independent.

Indeed, there is evidence from a previous study that age is inversely associated with LV mass index in FRDA [ 6 oone, suggesting either that higher LV mass index leads to early death, that increasing duration of the disease process leads to decreases in LV mass, or a combination of these mechanisms. There is also evidence from a small longitudinal study that progressive LV thinning occurs [ 12 ].

Whatever the process is, it was evident in the adult subjects with preservation of LVEF in the present study, where age was found to be an independent inverse correlate of LV mass index. Only a small group of subjects in the present study had a reduced LVEF at the time of their initial echocardiogram at our institution and further exploration of the nature, timing and causes of a reduced LVEF in FRDA will require larger numbers and longer-term follow up. Comparison of LV echocardiographic findings in adult subjects with FRDA with age and sex-matched control subjects has been reported previously in subgroups of the current cohort who were part of studies which investigated LV and right ventricular long-axis function [ 711 ].

In the present study, which examined and compared adults and children with FRDA, there was no onr group included, and decisions about the presence of increases in RWT and LV mass index were determined by using published normal ranges, with sex for age group and sex as appropriate. The present study has substantially added to our previous findings in FRDA by demonstrating noe in age-normalized RWT, comparing different methods for LV mass indexation, showing that there are differences between children and adults and demonstrating that male sex is associated with increased LV size and sex index in both children and adults.

There are a number of limitations of this cross-sectional study. Early mortality will alter the makeup of the population of subjects with FRDA available to be included in a cohort study and this could distort our understanding of the LV structural changes in FRDA if a particular pattern of LV myocardial involvement was more likely to lead to premature death. Similarly the analysis of a group with normal LVEF has necessarily excluded a proportion of subjects who had previously progressed over time to have a reduced LVEF, and this could also confound analyses which include age or symptom duration.

Unfortunately this limitation can only be fully addressed by long-term serial studies, preferably in which echocardiographic data was available prior to symptom onset.

We have used an arbitrary definition for children as being up to the age of 18 years, but the definition of child versus adult on varied in population studies of LV structure. The lack of positive findings with respect to associations of GAA1 in children compared to adults could sex a false negative due to the smaller size of the group of children.

However, there were no trends from the analysis in children to suggest that greater numbers were likely to have made a difference to the findings. Furthermore, as discussed above, there was evidence that some variables may be different in adults and children independently of other determinants.

Information about smoking, alcohol intake, drug use, exercise and menopausal status was not collected and any LV effects of these factors could therefore not be evaluated in this study.

In conclusion, RWT and age-normalized RWT are the most sensitive echocardiographic measures for identifying LV involvement in FRDA and they have the advantage of being independent of sex and body one in both children and adults.

GAA1 is an independent determinant of both increased LV wall thickness and reduced LV sex in kv, but not in children, and the existence of a difference between children and adults could be a contributing factor to previous discrepant findings with respect to the relationship of GAA1 with LV structure in FRDA.

GAA2 is not an independent correlate of LV structural variables in either children or adults and previous positive findings with respect to GAA2 may just reflect the presence of a currently unexplained association between GAA2 and GAA1.

Age is an independent predictor of smaller LV size and mass in adults, possibly reflecting in part xex nature of cardiac disease progression in FRDA, however, sex longitudinal data will be required to further clarify this relationship, and also the determinants and time course of a reduced LVEF.

National Center for Biotechnology InformationU. PLoS One. Published online Nov Roger E. Louise A. Martin B. Andreas Brodehl, Editor. Author information Article notes Copyright and License information Disclaimer. Competing Interests: The authors have declared that no competing interests exist. Received May 16; Accepted Oct ov This is an open access article distributed under the terms of the Creative Commons Attribution Licensewhich permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Abstract Introduction Although a concentric pattern of left ventricular LV geometry appears to be common in Friedreich ataxia FRDAthere is no accepted method for diagnosing Onw abnormalities in FRDA, sex and body size obe often not been taken into consideration, and it has not been clear whether children and adults should be classified using the same criteria.

Conclusion In FRDA, increases in RWT and age-normalized RWT are the most frequent LV structural abnormalities, sex and body size are important determinants of most one LV structural variables in both children and adults, and increased genetic severity is associated with a onf left ventricle and increased LV wall thickness in adults, but not associated with LV size or wall thickness in children.

Introduction Friedreich ataxia FRDA is an autosomal recessive ,v disease caused by a defect in the gene FXN which encodes for the mitochondrial protein frataxin [ 1 ]. Methods Study group The study design was approved by the Monash Health Human Research and One committee RESQ and all clinical investigation was conducted according to the principles expressed in the Declaration of Helsinki.

Results Of the subjects with FRDA who had an echocardiogram performed during the study period, 9 had FXN point mutations, were homozygous for GAA repeats and had results available from our laboratory, and 5 were known to be homozygous for GAA repeats on the basis of testing at other institutions.

Table 1 Demographic, anthropometric and disease specific characteristics in adults and children sxe Friedreich ataxia.

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