Objectives The aim of this study was to compare the reproducibility of 7 late gadolinium enhancement (LGE) quantification techniques across 3 conditions in which LGE is known to be important: acute ...myocardial infarction (AMI), chronic myocardial infarction (CMI), and hypertrophic cardiomyopathy (HCM). Background LGE by cardiac magnetic resonance is the gold-standard technique for assessing myocardial scar. No consensus exists on the best method for its quantification, and research in this area is scant. Techniques include manual quantification, thresholding by 2, 3, 4, 5, or 6 SDs above remote myocardium, and the full width at half maximum (FWHM) technique. To date, LGE has been linked to outcome in 3 conditions: AMI, CMI, and HCM. Methods Sixty patients with 3 LGE etiologies (AMI, n = 20; CMI, n = 20; HCM, n = 20) were scanned for LGE. LGE volume was quantified using the 7 techniques. Mean LGE volume, interobserver and intraobserver reproducibility, and impact on sample size were assessed. Results LGE volume varied significantly with the quantification method used. There was no statistically significant difference between LGE volume by the FWHM, manual, and 6-SD or 5-SD techniques. The 2-SD technique generated LGE volumes up to 2 times higher than the FWHM, 6-SD, and manual techniques. The reproducibility of all techniques was worse in HCM than AMI or CMI. The FWHM technique was the most reproducible in all 3 conditions compared with any other method (p < 0.001). Use of the FWHM technique for LGE quantification in paired analysis would lead to at least a 60% reduction in required sample size compared with any other method. Conclusions Regardless of the disease under study, the FWHM technique for LGE quantification gives LGE volume mean results similar to manual quantification and is statistically the most reproducible, reducing required sample sizes by up to one-half.
Assessment and Significance of Left Ventricular Mass by Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy Iacopo Olivotto, Martin S. Maron, Camillo Autore, John R. Lesser, Luigi Rega, ...Giancarlo Casolo, Marcello De Santis, Giovanni Quarta, Stefano Nistri, Franco Cecchi, Carol J. Salton, James E. Udelson, Warren J. Manning, Barry J. Maron, Left ventricular (LV) mass was assessed by cardiovascular magnetic resonance in 264 patients with hypertrophic cardiomyopathy (HCM), and compared with LV mass in 606 healthy control subjects. The LV mass index in HCM patients significantly exceeded that of control subjects, but was within normal range in 56 patients (21%), and only mildly increased in 18 (16%). Left ventricular mass was weakly related to maximal LV thickness (r2 = 0.38; p < 0.001). During follow-up, markedly increased LV mass index proved a more sensitive predictor of outcome than extreme maximal wall thickness. In distinction to prior perceptions, increased LV mass is not required to establish the clinical diagnosis of HCM.
Abstract Background: Data from the literature have suggested that bortezomib is the only effective agent in the treatment of plasma cell leukemia (PCL), a type of plasma cell dyscrasia characterized ...by poor prognosis despite conventional chemotherapy including autologous and allogeneic transplantation. Objective: This case series examined the antineoplastic activity of the bortezomib-based regimens in a small cohort of patients with PCL. Case summaries: We describe a retrospective review of the 3 cases of PCL diagnosed at Antonio Perrino Hospital, Brindisi, Italy, between July 2004 and October 2006 (2 women and 1 man, all white, ages 71, 64, and 42 years; 2 with primary PCL and 1 with secondary PCL). These patients were treated with bortezomib variously combined with other drugs outside of clinical trials. Patients 1 and 2 received bortezomib-based regimens (bortezomib 1.3 mg/m2 IV once daily on days 1, 4, 8, and 11; dexamethasone 20 mg IV once daily on days 1–4 and 8–11; oral cyclophosphamide 50 mg once daily on days 1–21, every 28 days) after 2 previous chemotherapeutic treatments. Patient 3 received a bortezomib-based regimen (bortezomib 1.3 mg/m2 IV once daily on days 1, 4, 8, and 11; doxorubicin 9 mg/m2 IV once daily on days 1–4; and dexamethasone 40 mg IV once daily on days 1–4, 8–11, and 15–18 during cycle 1 and days 1–4 during subsequent cycles) after one previous chemotherapeutic regimen. In all 3 patients, circulating plasma cells persisted. Patients 1 and 2 were not considered candidates for autologous peripheral blood stem cell transplantation (PBSCT) because of their nonresponse to the bortezomib-based regimens and severe deterioration of their clinical conditions (kidney and liver failure) due to disease progression. The overall survivals after administration of the bortezomib- based regimens were 4 months in patient 1 and 1 month in patient 2. After further treatment according to the modified protocol for patients with acute lymphatic leukemia (cyclophosphamide 800 mg/m2 IV on day 1 and 200 mg/m2 IV on days 2–5; vincristine 1.5 mg/m2 IV once daily on days 1, 8, and 15; doxorubicin 40 mg/m2 IV on day 1; methotrexate 1200 mg/m2 /h IV + 240 mg/m2 /h IV for 23 hours modified CODOX-M protocol) due to the unavailability of a human leukocyte antigen-identical donor, patient 3 received high-dose (200 mg/m2 ) melphalan with autologous PBSCT, obtaining partial remission lasting 9 months. The patient died 5 months later because of disease progression. Conclusion: These 3 patients with primary or secondary PCL who received a bortezomib-based regimen as rescue medication did not respond to treatment.
The authors report the case of a patient with hypertrophic cardiomyopathy who developed progressive and severe left ventricular wall thinning, as assessed by two-dimensional echocardiography, despite ...a preserved supranormal ejection fraction and an absence of cardiac symptoms. Extensive fibrosis was identified on cardiovascular magnetic resonance.
Summary
Neridronate is a third generation bisphosphonate with established efficacy in metabolic bone disease. In this randomized, open‐label study, 118 adults with β‐thalassaemia and bone mineral ...density (BMD) Z scores ≤−2·0 were randomized 1:1–500 mg calcium with 400 international unis (iu) vitamin D daily or 500 mg calcium with 400 iu vitamin D daily plus neridronate 100 mg intravenously every 90 d. Significant increases in BMD at the lumbar spine and total hip were noted in the neridronate group at 6 and 12 months from baseline (P < 0·001), and values were significantly higher than the control group at both time intervals. Neridronate also significantly decreased serum bone alkaline phosphatase and C‐telopeptide of collagen type 1 levels from as early as 3 months (P = 0·04 and P < 0·001, respectively), reaching significantly lower values at 12 months compared with the control group (P < 0·05). Reductions in back pain and analgesic use were also evident, starting 3 months from commencing treatment. Treatment was well tolerated by all patients. In this largest randomized trial in thalassaemia‐induced osteoporosis to date, neridronate was safe and effective in reducing bone resorption and increasing BMD. The associated reduction in back pain and improved quality of life will encourage adherence to therapy. (Clinicaltrials.gov identifier NCT01140321.)
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