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  • Cardiologic examination and detailed analysis of

    2019-06-28

    Cardiologic examination and detailed analysis of 12-lead rest ECG were performed by an experienced cardiologist (E.C.) and reevaluated by another independent cardiologist (S.R.). According to Groh et al., severe ECG abnormality included at least one of the following: rhythm other than sinus; PR interval of 240 ms or more; QRS duration of 120 ms or more; second-degree or third-degree AV block. For comparison between two groups, chi square test, Fisher test and Student t Vicriviroc Malate molecular test were used as appropriate. Spearman coefficient was used for correlations. Statistical significance was two-sided with α sets at 0.05 for statistical significance and 0.01 for high statistical significance.
    Results The main sociodemographic and clinical features of this Vicriviroc Malate molecular study\'s DM1 patients and HCs are presented in Table 1. These two groups were well matched for gender and age (p > 0.05). Cardiologic and ECG findings are reported in Table 2. Patients with DM1 had lower blood pressure and more common severe ECG abnormalities compared to HCs (p < 0.01). Heart valve echocardiographic findings are given in Table 3. MV prolapse was found in 23.0% of DM1 patients versus 8.5% of HCs (p < 0.05), while maximal mitral velocity was lower in DM1 patients (p < 0.01), but this was not of clinical significance. On the other hand, mild aortic regurgitation and tricuspid valve fibrosis were even more common in HCs (p < 0.05 and p < 0.01, respectively). EF between 45% and 55% was observed in 1.9% of patients, and EF < 45% in 5.6% of patients, while all HCs had EF > 55% (p < 0.05) (Table 4). Decreased global contractility of LV was present in 6.3% of DM1 patients compared to 0% of HCs (p < 0.05), while segmental wall motion abnormalities was present in 9.7% of patients and 0% HCs (p < 0.01). Increased end-systolic diameter of LV was observed in 6.5% of DM1 patients, and this parameter was normal in all HCs (p < 0.05). Echocardiographic impairments were correlated with all sociodemographic and clinical parameters listed in Table 1. The following associations were observed: patients with impaired global and regional contractility of the LV were older (51.6 ± 8.7 vs. 41.5 ± 10.8 years, and 49.1 ± 5.7 vs. 41.8 ± 11.1 years, p < 0.05, respectively). CTG repeat length did not correlate with cardiologic findings. Since childhood-onset and late-onset groups were small, comparisons were made only between juvenile and adult groups, and we did not observe significant differences in cardiac findings.
    Discussion In our study, MV prolapse was found in 23% of DM1 patients vs. 8% of HCs. In accordance with this, frequency of MV prolapse was reported to be from 13% to 40% in different cohorts of DM1 patients. According to the American Heart Association, antibiotics prophylaxis is not advised in routine use, even before having a dental procedure. On the other side, a majority of clinicians point out that antibiotic prophylaxis should be considered in patients with mitral valve prolapse until concrete clinical evidence is provided to dispute against the use of this strategy. This may be particularly applicable in DM1 patients, who commonly have cardiac conduction defects and arrhythmias. We found more common mild aortic regurgitation in HCs compared to DM1 patients, which might be explained by the fact that hypertension is more common in general population than in DM1 patients. This is probably due to the impairment of the smooth muscles of blood vessels in DM1. Furthermore, hypotension and lower prevalence of the metabolic syndrome among DM1 patients may explain the relatively low percentage of tricuspid fibrosis in DM1 patients. LV systolic dysfunction was observed in 6% of our patients. In line with this, 6% of subjects had global and 10% had regional hypocontractility of the LV wall, while 6% had increased end-systolic diameter of the LV. Although systolic dysfunction was reported in significantly higher percentage in some series of DM1 patients, meta-analysis showed an overall prevalence of 7.2%, which is similar to our results and significantly higher than in patients with arterial hypertension or in general population, where prevalences are 2.8% and 2.3%, respectively. All of our DM1 patients with EF of LV below 45% had at least mild symptoms of heart failure. Clinical signs of HF were not obvious, which might be explained by the limited level of activity in DM1 patients. Previous study reported that DM1 patients with HF were at four-times higher risk of all-cause death, and at six-times higher risk of cardiac death. Although there is no a single clinical trial that showed benefit from the treatment of HF in DM1, it seems logical that DM1 patients should benefit from the medical therapy. Since beta blockers are reported to be poorly tolerated in DM1 due to fatigue and cardiac conduction abnormalities, angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor antagonists seem to be good choices. ACE inhibitors are shown to diminish heart fibrosis and hypertrophy in mice with hypertrophic cardiomyopathy.