Familial amyloidotic polyneuropathy with muscle, vitreous, leptomeningeal, and cardiac involvement: Phenotypic, pathological, and MRI description.
Ann Indian Acad Neurol. 2010 Apr;13(2):142-4
Authors: Prashantha DK, Taly AB, Sinha S, Yasha TC, Gayathri N, Kovur JM, Vijayan J
Familial amyloidotic polyneuropathy (FAN type 1) is a rare systemic disease that causes severe and disabling peripheral neuropathy. We describe the phenotypic, radiological, and pathological characteristics of a patient with familial amyloid polyneuropathy type 1 who had evidence of motor-sensory-autonomic neuropathy, ocular vitreous deposits, diffuse leptomeningeal involvement, and hypertrophic cardiomyopathy. Muscle involvement, an infrequently reported feature, was also observed. Early recognition of the disease has significant therapeutic implications.
20814501
Original post
Negative predictive value of normal adenosine-stress cardiac MRI in the assessment of coronary artery disease and correlation with semiquantitative perfusion analysis.
J Magn Reson Imaging. 2010 Sep;32(3):615-21
Authors: Pilz G, Eierle S, Heer T, Klos M, Ali E, Scheck R, Wild M, Bernhardt P, Hoefling B
PURPOSE:: To prospectively determine the negative predictive value of normal adenosine stress cardiac MR (CMR) in routine patients referred for evaluation of coronary artery disease (CAD), predominantly with intermediate to high pretest risk. MATERIALS AND METHODS:: Consecutive patients referred for coronary angiography were examined in a 1.5 Tesla whole-body scanner before catheterization. A total of 158 patients with normal CMR on qualitative assessment were included, and semiquantitative perfusion analysis was performed. Significant CAD was regarded as luminal narrowing of >/=70% in coronary angiography. RESULTS:: In the 158 study patients, negative predictive value of normal adenosine-stress CMR for significant CAD was 96.2% (for stenosis >/=90%: 98.1%). True-negative and false-negative patients were comparable regarding clinical presentation, risk factors, and CMR findings. Semiquantitative perfusion analysis gave significantly prolonged arrival time index and peak time index in the false-negative group. Using cutoff values >1.8 for arrival time index or >1.2 for peak time index, the CMR negative predictive value increased to 98.7% (for stenosis >/=90%: to 100%). CONCLUSION:: The very high negative predictive value for CAD supports CMR-based decision making for the indication to coronary angiography. Semiquantitative perfusion analysis seems promising to identify the small group of CAD patients not detectable by qualitative CMR assessment. J. Magn. Reson. Imaging 2010;32:615-621. (c) 2010 Wiley-Liss, Inc.
20815059
See the original post here
Double-Trouble Doppler: Reduction in Aortic Flow due to Combined Left Ventricular Outflow Tract Obstruction and Severe Aortic Stenosis, Complete Diagnosis by Transthoracic Doppler Echocardiography.
J Am Soc Echocardiogr. 2010 Aug 30;
Authors: Kansal MM, Mookadam F, Tajik AJ
In patients with hypertrophic obstructive cardiomyopathy and dynamic left ventricular outflow tract obstructions, an additional fixed obstruction may uncommonly coexist. In these situations, flow through the aortic valve is usually delayed but typically still throughout the entire ejection period. We describe a case of marked reduction in aortic flow during mid and late systole, diagnosed by Doppler echocardiography, caused by combined hypertrophic obstructive cardiomyopathy and severe calcific bicuspid aortic stenosis.
20810244
View original post here
Differences of Myocardial Systolic Deformation and Correlates of Diastolic Function in Competitive Rowers and Young Hypertensives: A Speckle-Tracking Echocardiography Study.
J Am Soc Echocardiogr. 2010 Aug 30;
Authors: Galderisi M, Lomoriello VS, Santoro A, Esposito R, Olibet M, Raia R, Dario Di Minno MN, Guerra G, Mele D, Lombardi G
BACKGROUND: The aim of this study was to compare speckle-tracking echocardiography-derived left ventricular (LV) systolic mechanics and their relationships with LV diastolic properties in young patients with hypertension and in young competitive athletes in relation to their respective alterations of LV structure. METHODS: Nineteen sedentary controls, 22 top-level rowers, and 18 young newly diagnosed, never-treated patients with hypertension, all male, underwent Doppler echocardiography including pulsed tissue Doppler of the mitral annulus and speckle-tracking echocardiography. Peak longitudinal strain was calculated in apical long-axis, four-chamber, and two-chamber views, and values of the three views were averaged (global longitudinal strain [GLS]). Regional circumferential and radial strain were calculated at the LV basal, middle, and apical levels, and values were averaged (global circumferential strain and global radial strain). LV torsion was determined as the net difference in the mean rotation between the apical and basal levels. RESULTS: The three groups were comparable for age, whereas body mass index and blood pressure were higher in patients with hypertension, and heart rate was lower in rowers. LV mass index was higher in rowers and in patients with hypertension than in controls, without differences in relative wall thickness, ejection fraction, and midwall shortening. Left atrial volume index was greater in rowers than in controls and patients with hypertension. Annular systolic velocity (s’) (P < .001) and early diastolic velocity (e') (P < .0001) were lower and the E/e' ratio was higher (P < .0001) in patients with hypertension. GLS was lower in patients with hypertension (-17.5 +/- 2.8%) than in rowers (-22.2 +/- 2.7%) and in controls (-21.1 +/- 2.0%) (P < .0001). Global circumferential strain, global radial strain, and torsion were similar among the three groups. In the pooled population, GLS was an independent contributor to E/e' ratio (P < .0001) after adjusting for age, heart rate, meridional end-systolic stress, LV mass index and left atrial volume index. By receiver operating characteristic curve analyses, both GLS and E/e' ratio appeared to be accurate in discriminating patients with hypertension from healthy controls, with the E/e' ratio being more sensitive (77.8%) and GLS more specific (89.5%). CONCLUSIONS: The hearts of young patients with hypertension are characterized by reduced GLS, whereas global circumferential strain, global radial strain, and torsion are similar to those of athletes' hearts. The extent of GLS is strongly associated with LV diastolic function, independently of afterload changes and the degree of LV hypertrophy.
20810245
Read the original
Quantitative Assessment of Mitral Regurgitation: Comparison Between Three-dimensional Transesophageal Echocardiography and Magnetic Resonance Imaging.
Circ Cardiovasc Imaging. 2010 Sep 1;
Authors: Shanks M, Siebelink HM, Delgado V, van de Veire NR, Ng AC, Sieders A, Schuijf JD, Lamb HJ, Ajmone Marsan N, Westenberg JJ, Kroft L, de Roos A, Bax JJ
BACKGROUND: -Quantification of mitral regurgitation severity with 2-dimensional (2D) imaging techniques remains challenging. The present study compared the accuracy of 2D transesophageal echocardiography (TEE) and 3-dimensional (3D) TEE for quantification of mitral regurgitation, using magnetic resonance imaging (MRI) as reference method. METHODS AND RESULTS: -2D and 3D TEE and cardiac MRI were performed in 30 patients with mitral regurgitation. Mitral effective regurgitant orifice area (EROA) and regurgitant volume (Rvol) were estimated with 2D and 3D TEE. With 3D TEE, EROA was calculated using planimetry of the color Doppler flow from “en face” views and Rvol was derived by multiplying the EROA by the velocity time integral of the regurgitant jet. Finally, using MRI mitral Rvol was quantified by subtracting the aortic flow volume from left ventricular stroke volume. Compared to 3D TEE, 2D TEE underestimated the EROA by a mean of 0.13 cm(2). In addition, 2D TEE underestimated the Rvol by 21.6% when compared to 3D TEE and by 21.3% when compared to MRI. In contrast, 3D TEE underestimated the Rvol by only 1.2% when compared to MRI. Finally, one third of the patients in grade 1 and >/=50% of the patients in grade 2 and 3, as assessed with 2D TEE, would have been upgraded to a more severe grade based on the 3D TEE and MRI measurements. CONCLUSIONS: -Quantification of mitral EROA and Rvol with 3D TEE is feasible and accurate as compared to MRI and results in less underestimation of the Rvol as compared to 2D TEE.
20810848
Read the rest here
