Cardiac manifestation is evident in chorea-acanthocytosis but different from McLeod syndrome.

INTRODUCTION: We aimed to study the various cardiac manifestations of the two core neuroacanthocytosis (NA) syndromes, namely chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS). So far, cardiac involvement has been described as specific feature only for MLS. METHODS: We studied six patients with ChAc (mean age 44.5 years, five men, one woman) and six patients with MLS (mean age 57.1 years, all men). Cardiac evaluation included echocardiography and/or cardiac magnetic resonance imaging (cardiac MRI), 24-h ECG-recording and examination of cardiac biomarkers. RESULTS: Cardiac involvement of ChAc was found in four of six patients. Two patients showed mildly reduced left ventricular ejection fraction (LVEF), two other patients mild to moderate left ventricular (LV) dilatation. Neither an increase in ventricular ectopic beats nor ventricular tachycardia were evident in ChAc. Four of five MLS patients showed left ventricle dilatation and reduced left ventricular ejection fraction (LVEF). Two of these, in addition, had critical ventricular tachycardia. High sensitive troponin T was elevated in all patients, for whom data were available (n = 10). In contrast, elevation of high sensitive troponin I was found in one of six ChAc and one of two MLS patients. CONCLUSION: For the first time, we reveal cardiac involvement in a cohort of six ChAc patients, while the risk to develop heart failure seems lower than in MLS. Our study confirms the malignant nature of MLS in terms of ventricular arrhythmias and progression to advanced heart failure. Herein, we define disease-specific recommendations for cardiac assessment in both conditions.

Adrenal glands enhancement in computed tomography as predictor of short-and intermediate term mortality in critically ill patients.

PURPOSE: To investigate whether adrenal gland radiodensities alone or compared to the inferior vena cava (IVC) can correctly predict hospital mortality in patients in intensive care. METHODS: One hundred thirteen intensive care patients (76 males, age: 67.2 ± 14.0 years) with an acute clinical deterioration were included in this retrospective analysis. For the venous and the arterial phase CT attenuation (Hounsfield units) of adrenal glands and IVC was ROI-based evaluated by two radiologists separately. ROC analysis, combined with the Matthews Correlation Coefficient (MCC) as a classifier, was used to assess whether one of the parameters is suitable for predicting short and medium-term mortality and, if so, which parameter is most appropriate. Interrater agreement was assessed using the intraclass correlation coefficient. RESULTS: Twenty-one patients (18.6%) died within three days in the ICU. Measurements of the adrenal glands in the portal venous phase yielded the highest discriminative power (=AUC) to distinguish between deceased and survivors. A threshold ratio of >95.5 predicted 72-hour mortality with a sensitivity of 76.19% and a specificity of 92.39% (AUC = 0.84; p < 0.0001). The positive likelihood ratio was 10.1; the positive predictive value was 69%. The predictive power for 24-hour mortality was slightly lower. Venous adrenal-to-IVC ratios and arterial measurements as a whole were substantially less suitable. All intraclass correlation coefficients indicated a high interrater agreement. CONCLUSIONS: In the portal venous phase, hyperattenuating of the adrenal glands on contrast-enhanced CT can predict short and intermediate ICU mortality quite well and may serve as a reproducible prognostic marker for individual patient outcomes.

Vascular changes of the superior mesenteric artery (SMA): A new component of the hypovolemic shock complex (HSC).

PURPOSE: To assess the type and frequency of vascular changes in the superior mesenteric artery (SMA) associated with the hypovolemic shock complex (HSC). METHODS: Twenty-six patients (14 males, 70.6 ±â€¯11.2 years) meeting the criteria for hypovolemic shock complex in computed tomography were examined for the presence of angiographic signs of non-occlusive mesenteric ischemia (NOMI) in the SMA: the string of sausages sign and spasms of the arcades of mesenteric arteries on coronal maximum intensity projection images (MIP). Interrater agreement was assessed using weighted kappa (κ). RESULTS: Vascular changes of the SMA were visible in almost all of the patients with HSC with a frequency of 88.5 %-96.2 %. Intraclass correlation coefficients indicated a substantial to almost perfect interrater agreement. CONCLUSIONS: Using computed tomography, it is possible to reliably and reproducibly detect vascular changes in SMA known from angiography in the context of hypoperfusion. The pathological vascular changes also occur more frequently than other classic signs of a CT hypoperfusion complex. Since the qualitative assessment of the SMA requires only a small amount of time, it is suitable as a further criterion for the presence of the CT hypoperfusion complex.

Reduction of atrial fibrillation burden by pulmonary vein isolation leads to a decrease of CD11b expression on inflammatory cells.

Aims: It is hypothesized that inflammation could promote structural and electrical remodelling processes in atrial fibrillation (AF). Atrial infiltration of monocytes and granulocytes has been shown to be dependent on CD11b expression. The aim of this study was to investigate whether treatment of AF by pulmonary vein isolation (PVI) may lead to reduced inflammation, as indicated by a decrease of CD11b expression on monocytes and granulocytes. Methods and results: Flow-cytometric quantification analysis and determination of systemic inflammatory markers of peripheral blood were performed in 75 patients undergoing PVI 1 day before and 6 months after PVI. The extent of activation of monocytes and granulocytes was measured by quantifying the cell adhesion molecule CD11b. The mean expression of CD11b on monocytes (20.9 ± 2.5 vs. 10.2 ± 1.4; P < 0.001) and granulocytes (13.9 ± 1.6 vs. 6.8 ± 0.5; P < 0.001), as well as the relative count of CD11b-positive monocytes (P < 0.05) and CD11b-positive granulocytes (P < 0.01) were significantly reduced when comparing the identical patients before and 6 months after PVI. Systemic inflammatory parameters showed only a declining tendency after 6 months. Patients with unsuccessful PVI and ongoing AF on the day of follow-up showed no decrease in CD11b expression. Conclusions: A significant reduction of CD11b expression on monocytes and granulocytes, as a sign of reduced cellular inflammation, was achieved by treatment of AF using PVI. These data strongly support that AF is not only a consequence of but also a cause for inflammatory processes, which, in turn, may contribute to atrial remodelling.

Three-dimensional transoesophageal echocardiography is crucial for valid assessment of mitral valve leaflet morphology in severe mitral regurgitation prior to interventional repair.

BACKGROUND: Interventional mitral valve (MV) repair of severe symptomatic mitral regurgitation (MR) is a therapeutic option in high-risk surgical or inoperable patients. Assessment of the MV remains a crucial part of pre-interventional screening. Three-dimensional transoesophageal echocardiography (3D-TOE) may compensate for well-known pitfalls that occur in 2D-TOE. PURPOSE: We investigated whether the functional length of the central segments of the posterior and anterior MV leaflets (PML-P2 and AML-A2) is more reliably determined by 3D-TOE full volume datasets (3D-MPR) or orthogonal biplane-imaging (Xplane) when compared to 2D-TOE. METHODS AND RESULTS: Between February 2014 and August 2015, 265 consecutive patients with moderate to severe symptomatic MR were screened. Seventy patients were judged suitable for interventional MV repair by the in-house Heart-Team. Eventually, 59 patients remained for data analysis. Inter-observer variability was lowest in 3D-MPR followed by Xplane (r = 0.92 and 0.90, p < .001 for both) and highest in Mplane (r = 0.82, p < .001). Mean functional PML-P2 lengths were similar in Xplane (12.6 ± 1.7 mm) and 3D-MPR (12.1 ± 2.0 mm), however, significantly different in 2D-TOE (10.0 ± 2.1 mm, p < .001). 2D-TOE underestimated PML-P2 length with a bias of -2.5 mm compared to Xplane and -1.95 mm compared to 3D-MPR. In contrast, functional AML-A2 length was determined similar across all methods. CONCLUSIONS: Our results demonstrate the superiority of 3D-TOE over 2D-TOE for accurate MV assessment in MR, especially for the determination of the functional PML length. Erroneous MV leaflet assessment may result in inadequate therapy restriction if the MV is deemed not suitable for interventional repair.