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).
Aims: Dobutamine and adenosine stress cardiac magnetic resonance imaging (CMR) are relatively contraindicated in patients with moderate to severe AS. We aimed to determine the safety of dobutamine and adenosine stress CMR in patients with moderate to severe AS.Methods: In this retrospective study patients with AS who underwent either dobutamine or adenosine stress CMR between January 2009 and December 2021 for exclusion of obstructive CAD were enrolled. We recorded clinical data, CMR and echocardiography ndings and side effects (major complications, minor complications and complaints).Patients with AS were compared to matched individuals without AS.Results: 187 patients with AS were identi ed and compared to age-, gender-and BMI-matched 187 patients without AS. No major complications were reported in the study nor the control group. The reported frequency of complaints and minor complications were similar between the study and the control groups.Nineteen patients with AS experienced side effects during dobutamine stress CMR compared to eighteen patients without AS (p=0.855). One patient with AS and two patients without AS undergoing adenosine stress CMR experienced side effects (p=0.562). Four examinations were aborted because of severe chest pain, paroxysmal atrial brillation and third-degree atrioventricular block. Age, prior coronary artery bypass grafting, prior stroke and inducible ischaemia were associated with a higher incidence of complications.Conclusion: Moderate to severe AS was not associated with side effects of complications during CMR stress test. The data suggest CMR stress testing to be safe in patients with AS.
Introduction Wilson's disease (WD) is a rare autosomal recessive copper disorder with limited excretion of excess copper into the bile. Primary symptoms are hepatic or neurological. However, the clinical range of WD is wide and can result in cardiac symptoms as well. Previous studies revealed a higher incidence of heart failure in WD patients compared to the rest of the population. Purpose Cardiac magnetic resonance imaging (CMR) is used to identify the typical features of several systemic disorders with excessive myocardial deposition of substrates. The aim of this study was to perform a cardiac tissue characterization in WD patients by using CMR and to identify subgroups of WD patients with reduced ejection fraction (EF). Methods Patients with known WD using Ferenci-Score were included in this prospective study. WD patients were referred to 1.5 Tesla CMR. The following CMR protocol was performed; Cine-images, T1-, T2- and T2*-Mapping, fast-SENC strain and late gadolinium enhancement (LGE). Fast-SENC strain measurements were compared with values from healthy individuals scanned at the center. Results 43 patients (age 38.7±12.8 years, 20 female, BMI 23.80 (17.4–33.1)) with WD could be identified and were evaluated with CMR. CMR revealed normal left ventricular (LV) EF (62.4±5.4%) and right ventricular (RV) EF (64.4±7.1%) overall. However, three patients (7%), who suffered primarily from neurological symptoms, were found to have mildly reduced LV-EF (46.5%, 51%, and 53.5%). Strain analysis revealed significantly reduced LV global circumferential strain (GCS) overall compared to healthy individuals (WD (%): −19.2 2.7; control (%): −20.71±1.5, p<0.05). Patients with primarily hepatic symptoms (WD-h) did not show reduced strain measurements compared to the control group. Patients suffering from primarily neurological symptoms (WD-n) showed significantly reduced LV GCS compared to healthy individuals (WD-n (%): −18.3±3.1; control (%): −20.7±1.5, p<0.05) and RV GCS (WD-n (%): −17.5±3.0; control (%): −19.2±1.8, p<0.05). Also, LV GCS in WD-n was significantly reduced compared to WD-h (WD-n (%): −18.3±3.1; WD-h (%): −20.0±2.0). Furthermore, there were no significant differences between the two subgroups, besides a significant thicker lateral wall in patients with WD-n (WD-n (mm): 7 (5–9); WD-h (mm): 6 (5–8), p<0.05). T1-, T2- and T2*-Mapping did not show any pathological pattern and were overall in the normal range (T1: 1020±30ms; T2: 52.9±3.0ms; T2*: 38.4±5.6ms). Epicardial LGE was present in 1 patient. Conclusion Cardiac tissue characterization was performed in WD patients using CMR. Reduced EF, LV and RV GCS have been detected in patients with primarily neurological symptoms. Cardiovascular autonomic dysfunction in this subgroup could be a reason for the reduced biventricular strain. It is unknown if reduced circumferential strain influences the prognosis of WD patients, which should be investigated in further studies.
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