Cardiac involvement in Wilson disease: Review of the literature and description of three cases of sudden death.

Wilson disease (WD) is a rare genetic condition that results from a build-up of copper in the body. It requires life-long treatment and is mainly characterized by hepatic and neurological features. Copper accumulation has been reported to be related to the occurrence of heart disease, although little is known regarding this association. We have conducted a systematic review of the literature to document the association between WD and cardiac involvement. Thirty-two articles were retained. We also described three cases of sudden death. Cardiac manifestations in WD include cardiomyopathy (mainly left ventricular (LV) remodeling, hypertrophy, and LV diastolic dysfunction, and less frequently LV systolic dysfunction), increased levels of troponin, and/or brain natriuretic peptide, electrocardiogram (ECG) abnormalities, and rhythm or conduction abnormalities, which can be life-threatening. Dysautonomia has also been reported. The mechanism of cardiac damage in WD has not been elucidated. It may be the result of copper accumulation in the heart, and/or it could be due to a toxic effect of copper, resulting in the release of free oxygen radicals. Patients with signs and/or symptoms of cardiac involvement or who have cardiovascular risk factors should be examined by a cardiologist in addition to being assessed by their interdisciplinary treating team. Furthermore, ECG, cardiac biomarkers, echocardiography, and 24-hours or more of Holter monitoring at the diagnosis and/or during the follow-up of patients with WD need to be evaluated. Cardiac magnetic resonance imaging, although not always available, could also be a useful diagnostic tool, allowing assessment of the risk of ventricular arrhythmias and further guidance of the cardiac workup.

Echocardiography in Confirmed and Highly Suspected Symptomatic COVID-19 Patients and Its Impact on Treatment Change.

BACKGROUND: COVID-19 interacts at multiple levels with the cardiovascular system. The prognosis of COVID-19 infection is known to be worse for patients with underlying cardiovascular diseases. Furthermore, the virus is responsible for many cardiovascular complications. Myocardial injury may affect up to 20% of the critically ill patients. However, echocardiography's impact on the management of patients affected by COVID-19 remains unknown. OBJECTIVES: To explore echocardiography's impact on the management of COVID-19 patients. METHODS: This study was conducted from March 24th to April 14th, 2020, in a single center at Adolphe de Rothschild Foundation Hospital, Paris, France. All consecutive inpatients with laboratory and/or CT COVID-19 diagnosis were included in this study. Patients' characteristics (clinical, biological, and imaging) and treatment change induced by echocardiography were collected and analyzed. Patients with and without treatment change induced by echocardiography were compared. RESULTS: A total of 56 echocardiographies in 42 patients with highly suspected or confirmed COVID-19 were included in the final analyses. The median age was 66 (IQR 60.5-74). Echocardiography induced a treatment change in 9 cases (16%). The analyzed clinical data were not associated with any treatment change induced by echocardiography. D-dimer and Troponin levels were the only biological predictors of the induced treatment change. On echocardiography, higher systolic pulmonary arterial pressure and documented cardiac thrombi were associated with treatment changes in these patients. CONCLUSIONS: Echocardiography may be useful for the management of selected COVID-19 patients, especially those with elevated D-Dimer and Troponin levels, in up to 16% of patients.

Left Ventricular End Diastolic Volume and Ejection Fraction Calculation: Correlation between Three Echocardiographic Methods.

BACKGROUND: Left ventricular ejection fraction (LVEF) and end diastolic volume (EDV) are measured using Simpson's biplane (SB), 3-dimensional method (3DE), and speckle tracking (STE). Comparisons between methods in routine practice are limited. Our purpose was to compare and to determine the correlations between these three methods in clinical setting. METHODS: LVEF and EDV were measured by three methods in 474 consecutive patients and compared using multiple Bland-Altman (BA) plots. The correlations (R) between methods were calculated. RESULTS: Median (IQR) LVEF_SB, LVEF_STE, and LVEF_3DE were 63.0% (60-69)%, 61% (57-65)%, and 62% (57-68)%. Median (IQR) EDV_SB, EDV_STE, and EDV_3DE were 85 ml (71-106) ml, 82 ml (69-100) ml, and 73 ml (59-89) ml. R between LVEF_SB and LVEF_3DE was 0.65 when echogenicity was good and 0.43 when poor. R for EDV_SB and EDV_3DE was 0.75 when echogenicity was good and 0.45 when poor. On BA analysis, biases were acceptable (<3.5% for LVEF) but limits of agreement (LOA) were large: 95% of the differences were between -15.4% and +18.8% for LVEF as evaluated by SB in comparison with 3DE, with a bias of 1.7%. In the comparison EDV_SB and EDV_3DE, the bias was 14 ml and the LOA were between -24 ml and +53 ml. On linear regressions, LVEF_3DE = 17.92 + 0.69 LVEF_SB and EDV_3DE = 18.94 + 0.63 EDV_SB. CONCLUSIONS: The three methods were feasible and led to acceptable bias but large LOA. Although these methods are not interchangeable, our results allow 3DE value prediction from SB, the most commonly used method.

Relationship between cognitive impairment and echocardiographic parameters: a review.

With >24 million people affected worldwide, dementia is one of the main public health challenges modern medicine has to face. The path leading to dementia is often long, with a wide spectrum of clinical presentations, and preceded by a long preclinical phase. Previous studies have demonstrated that clinical strokes and covert vascular lesions of the brain contribute to the risk for developing dementia. Although it is not yet known whether preventing such lesions reduces the risk for dementia, it is likely that starting preventive measures early in the course of the disease may be beneficial. Echocardiography is a widely available, relatively inexpensive, noninvasive imaging modality whereby morphologically or hemodynamically derived parameters may be integrated easily into a risk assessment model for dementia. The aim of this review is to analyze the information that has accumulated over the past two decades on the prognostic value of echocardiographic factors in cognitive impairment. The associations between cognitive impairment and echocardiographic parameters, including left ventricular systolic and diastolic indices, left atrial morphologic parameters, cardiac output, left ventricular mass, and aortic root diameter, have previously been reported. In the light of these studies, it appears that echocardiography may help further improve currently used risk assessment models by allowing detection of subclinical cardiac abnormalities associated with future cognitive impairment. However, many limitations, including methodologic heterogeneity and the observational designs of these studies, restrict the scope of these results. Further prospective studies are required before integrating echocardiography into a preventive strategy.