Differentiation between Fabry disease and hypertrophic cardiomyopathy with cardiac T1 mapping.

PURPOSE: To evaluate the potential of non-contrast myocardial T1 mapping on cardiovascular magnetic resonance examination (CMR) in differentiating patients with Fabry disease (FD) from those with hypertrophic cardiomyopathy (HCM) and healthy control subjects. MATERIALS AND METHODS: Seventeen patients with FD (8 men, 9 women; mean age, 48 ±18 [SD] years; [range: 19-73 years]; 53% with left ventricular hypertrophy [LVH]) were matched with 36 patients with hypertrophic cardiomyopathy (HCM) (22 men, 14 women; mean age, 57±16 [SD] years; [range: 22-85 years]) and 70 healthy control subjects (34 men, 36 women; mean age, 38 ±15 [SD] years; [range: 18-65 years]). Cardiac T1 mapping was performed using the modified Look-Locker inversion (MOLLI®) sequence on a 1.5-T magnet. T1 values were calculated, on midventricular section, for septal left ventricular segments (S8-S9) and all mid-ventricular ones (global T1 values; S7-S12). Statistical analysis included unpaired Mann-Whitney test, receiver operating characteristic curve and likelihood ratios. RESULTS: Septal native T1 values were significantly decreased in patients with FD (889±61 [SD] ms; range: 784-980ms) compared to those with HCM (995±48 [SD] ms; range: 935-1125ms) (P<0.001) and versus healthy controls (965±29 [SD] ms; range: 910-1028ms) (P<0.001). Global native T1 values were also significantly decreased in patients with FD (891±49 [SD] ms; range 794-970ms) compared to those with HCM (995±34 [SD] ms; range: 952-1086ms) (P<0.001) and versus healthy controls (966±27 [SD] ms; range: 920-1042ms) (P<0.001). A septal left ventricular native T1 cutoff value of 940ms could distinguish FD from HCM with 88% sensitivity (95% CI: 73-100%) and 92% specificity (95% CI: 83-100%). Positive likelihood ratio was 11, negative likelihood ratio was 0.12. Compared to controls, the same threshold could distinguish FD with 88% sensitivity (95% CI: 73-100%) and 86% specificity (95% CI: 78-94%). Positive likelihood ratio was 6.3, negative likelihood ratio was 0.14. T1 value was abnormal in 4 of 8 (50%) of FD patients who did not have LVH. CONCLUSION: Native T1 values are significantly lower in patients with FD by comparison with those with HCM and healthy volunteers.

[Acute carpal tunnel syndrome caused by a thrombosis of a persistent median artery: 2 case reports]. / Canal carpien aigu : penser à la thrombose de l'artère persistante du nerf médian !

Carpal tunnel syndrome is a common peripheral neuropathy, usually idiopathic or post-traumatic due to the compression of the median nerve. Numbness and paresthesias in the median nerve distribution are the most common symptoms associated with this condition. Persistent median artery is a rare anatomic variation, thrombosis of this additional artery can be responsible for an acute carpal tunnel syndrome, and patients frequently complain about coldness and acute hand swelling. These unusual features must lead clinicians to think of a vascular cause. The diagnosis can be easily confirmed by using ultrasound doppler, but CT-scan and MRI are sometimes helpful. We describe 2 cases of acute carpal tunnel syndrome due to thrombosed persistent median artery, including a case of thromboangiitis obliterans. These thrombosis might also be due to traumatic causes. No guidelines are currently available to help physicians for the management of carpal tunnel syndrome from thrombosed persistent median artery. Antiplatelet therapy, statin, anticoagulant might be helpful, and surgery has sometimes be reported as effective.

Multidetector computed tomography sizing of aortic annulus prior to transcatheter aortic valve replacement (TAVR): Variability and impact of observer experience.

PURPOSE: To evaluate intra- and inter-observer variability of multidetector computed tomography (MDCT) sizing of the aortic annulus before transcatheter aortic valve replacement (TAVR) and the effect of observer experience, aortic valve calcification and image quality. METHODS: MDCT examinations of 52 consecutive patients with tricuspid aortic valve (30 women, 22 men) with a mean age of 83±7 (SD) years (range: 64-93 years) were evaluated retrospectively. The maximum and minimum diameters, area and circumference of the aortic annulus were measured twice at diastole and systole with a standardized approach by three independent observers with different levels of experience (expert [observer 1]; resident with intensive 6 months practice [observer 2]; trained resident with starting experience [observer 3]). Observers were requested to recommend the valve prosthesis size. Calcification volume of the aortic valve and signal to noise ratio were evaluated. RESULTS: Intra- and inter-observer reproducibility was excellent for all aortic annulus dimensions, with an intraclass correlation coefficient ranging respectively from 0.84 to 0.98 and from 0.82 to 0.97. Agreement for selection of prosthesis size was almost perfect between the two most experienced observers (k=0.82) and substantial with the inexperienced observer (k=0.67). Aortic valve calcification did not influence intra-observer reproducibility. Image quality influenced reproducibility of the inexperienced observer. CONCLUSION: Intra- and inter-observer variability of aortic annulus sizing by MDCT is low. Nevertheless, the less experienced observer showed lower reliability suggesting a learning curve.