Autonomic Dysfunction in Wilson's Disease: A Comprehensive Evaluation during a 3-Year Follow Up

Li, Kai; Lindauer, Charlotte; Haase, Rocco; Rüdiger, Heinz; Reichmann, Heinz; Reuner, Ulrike; Ziemssen, Tjalf

doi:10.3389/fphys.2017.00778

Cited by 14 publications

(14 citation statements)

References 41 publications

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“…15 Cardiovascular autonomic dysfunction due to involvement of both parasympathetic and sympathetic systems was previously reported in adult Wilson's disease patients with neurological presentation. 16,17 In the current study, the standard electrocardiographic assessment was normal in both groups, and QT intervals and minimum and maximum P-wave values were not different. However, QT and P-wave dispersion values were significantly higher in the Wilson's group.…”

Section: Discussionsupporting

confidence: 43%

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

et al. 2019

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Background:This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.Method:Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.Results:The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.Conclusion:Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

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Section: Discussionsupporting

confidence: 43%

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

et al. 2019

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“…A simple assessment of HRV in metronomic respiration allows for the calculation of the strongly age-dependent quotient from the longest heart rate intervals during exhalation to the shortest intervals during inhalation (I:E Ratio) (42–45). For metronomic breathing at six breaths per minute, this age-dependent quotient should be >1.2.…”

Section: The Most Important Cardiovascular Autonomic Function Testsmentioning

confidence: 99%

“…The pressor stimuli applied here all lead to a stimulation of the sympathetic afference independent of the baroreceptor afference (45, 59). All pressor tests lead to an increase in blood pressure and heart rate.…”

Section: The Most Important Cardiovascular Autonomic Function Testsmentioning

confidence: 99%

The Investigation of the Cardiovascular and Sudomotor Autonomic Nervous System—A Review

2019

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The autonomic nervous system as operating system of the human organism permeats all organ systems with its pathways permeating that it is involved with virtually all diseases. Anatomically a central part, an afferent part and sympathetic and parasympathetic efferent system can be distinguished. Among the different functional subsystems of the autonomic nervous system, the cardiovascular autonomic nervous system is most frequently examined with easily recordable cardiovascular biosignals as heart rate and blood pressure. Although less widely established, sudomotor tests pose a useful supplement to cardiovascular autonomic assessment as impaired neurogenic sweating belongs to the earliest clinical signs of various autonomic neuropathies as well as neurodegenerative disorders and significantly reduces quality of life. Clinically at first, the autonomic nervous system is assessed with a detailed history of clinical autonomic function and a general clinical examination. As a lof of confounding factors can influence autonomic testing, subjects should be adequately prepared in a standardized way. Autonomic testing is usually performed in that way that the response of the autonomic nervous system to a well-defined challenge is recorded. As no single cardiovascular autonomic test is sufficiently reliable, it is recommended to use a combination of different approaches, an autonomic test battery including test to measure parasympathetic and sympathetic cardiovascular function (deep breathing test, Valsalva maneuver, tilt, or pressor test). More specialized tests include carotid sinus massage, assessment of baroreceptor reflex function, pharmacological tests or cardiac, and regional hemodynamic measurements. Techniques to measure functional integrity of sudomotor nerves include the quantitative sudomotor axon reflex sweat test, analysis of the sympathetic skin response as well as the thermoregulatory sweat test. In addition to these rather established techniques more recent developments have been introduced to reduce technical demands and interindividual variability such as the quantitative direct and indirect axon reflex testing or sudoscan. However, diagnostic accuracy of these tests remains to be determined. We reviewed the current literature on currently available autonomic cardiovascular and sudomotor tests with a focus on their physiological and technical mechanisms as well as their diagnostic value in the scientific and clinical setting.

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“…Elevated T2 signal of the myocardium quantifies myocardial edema [ 26 ], indicating that some patients in their study might have suffered from an acute exacerbation compared to ours [ 49 ]. Additionally, the colleagues and also others reported LV hypertrophy in WD patients [ 10 – 12 , 49 ]. However, LV hypertrophy was not found in our cohort, even though septum and lateral wall of WD patients was increased, in particular in WD-neuro + patients.…”

Section: Discussionmentioning

confidence: 90%

“…Neuronal damage in other neurological conditions such as epilepsy and stroke has been shown to impact cardiac function via a brain–heart interaction [ 56 , 57 ]. Autonomic dysfunction is also more common in WD-neuro + patients [ 12 ]. Increased autonomic dysfunction in patients with neurological disorders of the brain can lead to cardiac fibrosis via catecholaminergic toxicity [ 56 , 58 ].…”

Section: Discussionmentioning

confidence: 99%

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study

Salatzki

Mohr

Heins

et al. 2021

Journal of Cardiovascular Magnetic Resonance

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Background Systemic effects of altered serum copper processing in Wilson Disease (WD) might induce myocardial copper deposition and consequently myocardial dysfunction and structural remodeling. This study sought to investigate the prevalence, manifestation and predictors of myocardial tissue abnormalities in WD patients. Methods We prospectively enrolled WD patients and an age-matched group of healthy individuals. We applied cardiovascular magnetic resonance (CMR) to analyze myocardial function, strain, and tissue characteristics. A subgroup analysis of WD patients with predominant neurological (WD-neuro+) or hepatic manifestation only (WD-neuro−) was performed. Results Seventy-six patients (37 years (27–49), 47% women) with known WD and 76 age-matched healthy control subjects were studied. The prevalence of atrial fibrillation in WD patients was 5% and the prevalence of symptomatic heart failure was 2.6%. Compared to healthy controls, patients with WD had a reduced left ventricular global circumferential strain (LV-GCS), and also showed abnormalities consistent with global and regional myocardial fibrosis. WD-neuro+ patients presented with more severe structural remodeling and functional impairment when compared to WD-neuro− patients. Conclusions In a large cohort, WD was not linked to a distinct cardiac phenotype except CMR indexes of myocardial fibrosis. More research is warranted to assess the prognostic implications of these findings. Trial registration: This trial is registered at the local institutional ethics committee (S-188/2018).

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Autonomic Dysfunction in Wilson's Disease: A Comprehensive Evaluation during a 3-Year Follow Up

Cited by 14 publications

References 41 publications

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

Assessment of cardiac function and electrocardiographic findings in patients with Wilson’s disease

The Investigation of the Cardiovascular and Sudomotor Autonomic Nervous System—A Review

The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study

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