Cohort Characteristics, Persons with Myocardial Infarction and follow-up by age, sex, and family history.

<p>Characteristics on 128,384 persons with myocardial infarction in a cohort of 4,445,255 persons born in 1930 or later and aged 20 years or more. The cohort was followed for 104,135,666 person-years from 1977 to 2012 in Denmark. Abbreviations: MI, Myocardial Infarction</p><p>^a Cohort members could contribute more than 1 type relative to the analyses; numbers add up to more than 128,384 (100%).</p><p>^b<b>First-degree</b>: parents, children and siblings.</p><p>^c<b>Second-degree</b>: grandparents, grandchildren, half-siblings, uncles, aunts, nieces and nephews.</p><p>Cohort Characteristics, Persons with Myocardial Infarction and follow-up by age, sex, and family history.</p

Incidence Rate Ratios of Myocardial Infarction by Number and Degree of Kinship.

<p>Incidence rate ratios with 95% confidence intervals for myocardial infarction in all persons aged 20 years or more with one, two or three or more first- or second-degree relatives with myocardial infarction compared to persons with similar a number of relatives without myocardial infarction, follow-up from 1977 to 2012. Abbreviations: MI, Myocardial Infarction; CI, confidence interval</p><p>^a Incidence rate ratios are adjusted for age, sex and calendar period</p><p>^{b & c} For definitions of first- and second-degree relatives, see <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0125896#pone.0125896.t001" target="_blank">Table 1</a>.</p><p>Incidence Rate Ratios of Myocardial Infarction by Number and Degree of Kinship.</p

Associations Between Complex Family Histories Of Myocardial Infarction And Myocardial Infarction Risk in Persons Aged 36-55y.

<p>Incidence rate ratios with 95% confidence intervals for myocardial infarction in persons aged 35 to 55 years with combinations of none, one or two or more first- and second-degree relatives with myocardial infarction, follow-up from 1977 to 2012. Abbreviations: MI, Myocardial Infarction; CI, confidence interval</p><p>^a Reference incidence rate are rates in those cohort members with a myocardial infarction and identifiable relatives of both first- and second-degree without myocardial infarction. Incidence rate ratios are adjusted for age, sex and calendar period.</p><p>^{b & c} For definitions of first- and second-degree relatives, see <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0125896#pone.0125896.t001" target="_blank">Table 1</a>.</p><p>Associations Between Complex Family Histories Of Myocardial Infarction And Myocardial Infarction Risk in Persons Aged 36-55y.</p

The four graphs in the figure illustrate incidence rate ratios (IRRs) with 95% confidence intervals (CIs) for myocardial infarction in persons with one or more first-degree relatives with myocardial infarction.

<p>The analyses were stratified by age of first-degree relative at time of myocardial infarction. The reference group for each IRR consists of persons with relatives in the given age group but without a history of MI among those relatives. Age of the relative at time of myocardial infarction is shown in decades on the x-axis, while the IRRs for myocardial infarction are shown on the y-axis. The upper graph shows the risk for all persons in the cohort ≥20 years of age at some point during the follow-up period. The three lower graphs shows the results from analyses restricted to follow-up time while cohort members were 20–35 years of age, 36–55 years of age and >55 years of age. In the analysis of persons aged 20–35 years, there were no first-degree relatives aged 80–89 years with a myocardial infarction.</p

Echocardiographic and clinical findings in patients with Fabry disease during long-term enzyme replacement therapy: a nationwide Danish cohort study

<p><i>Objectives</i>: In patients with Fabry disease (FD), left ventricular hypertrophy and arrhythmias are frequently observed and cardiac involvement is the leading cause of death. Long-term efficacy of enzyme replacement therapy (ERT) on cardiac involvement is unclear. We assessed and compared long-term progression of cardiac involvement according to ERT and non-ERT. <i>Methods</i>: We retrospectively assessed and compared long-term progression of cardiac involvement in adult patients with FD in the nationwide Danish cohort. We followed clinical signs, symptoms and findings by echocardiography, electrocardiography and Holter-monitoring. <i>Results</i>: We included 66 patients; 47 patients (27 women) received ERT (ERT group) and 19 patients (15 women) did not (non-ERT group). The groups were followed for a median of 8 [0–12] years and 6 [0–13] years, respectively. Comparison between ERT and non-ERT receiving patients by left ventricular mass (echocardiographic assessment) and Sokolow-Lyon voltage- and Cornell product criteria (electrocardiographic assessment) revealed no significant differences. In the ERT group, we observed no change in left ventricular mass but a decrease in Sokolow-Lyon voltage- and Cornell product criteria from baseline to follow-up; 30 mm [15–53] vs. 25 mm [3–44], <i>p</i> < 0.005 and 1710 mm·ms [480–3740] vs. 1520 mm·ms [550–5740], <i>p</i> < .05, respectively. There were no changes within the non-ERT group. During follow-up, cardiac symptoms and use of cardiovascular procedures and -medication increased significantly in the ERT group, whereas no differences were observed within the non-ERT group. <i>Discussion</i>: We raise concerns regarding the efficacy and benefit of ERT on cardiac involvement in Fabry disease and stress the need for further research.</p

The algorithm used to select for potentially pathogenic mtDNA variants.

<p>The algorithm used to select for potentially pathogenic mtDNA variants.</p

Private variants with a GenBank frequency < 0.1%.

<p>Private variants with a GenBank frequency < 0.1%.</p

Demographic and clinical characteristics of HCM probands.

<p>*Mean (SD).</p><p>¹ In index patients > 18 years of age. BP, blood pressure; LA, left atrial diameter; MaxLVD, maximal left ventricular wall thickness: MaxIVS, maximal interventricular wall thickness.</p><p>Demographic and clinical characteristics of HCM probands.</p

The gene distribution of the 446 identified variants in the mtDNA genomes.

<p>The gene distribution of the 446 identified variants in the mtDNA genomes.</p

Variants previously associated with HCM.

<p>Variants previously associated with HCM.</p