Up to 5% of patients will develop A-CHF 15 years after treatment, and patients treated with a cumulative dose of anthracyclines higher than 300 mg/m(2) are at highest risk for A-CHF. This is thus a considerable and serious problem among these young patients. The findings reinforce the need for strategies for early detection of patients at risk for A-CHF and for the evaluation of other chemotherapeutic possibilities or cardioprotective agents in relation to the survival.
Kawasaki disease is a systemic vasculitis of unknown etiology, with clinical observations suggesting a substantial genetic contribution to disease susceptibility. We conducted a genome-wide association study and replication analysis in 2,173 individuals with Kawasaki disease and 9,383 controls from five independent sample collections. Two loci exceeded the formal threshold for genome-wide significance. The first locus is a functional polymorphism in the IgG receptor gene FCGR2A (encoding an H131R substitution) (rs1801274; P = 7.35 × 10(-11), odds ratio (OR) = 1.32), with the A allele (coding for histadine) conferring elevated disease risk. The second locus is at 19q13, (P = 2.51 × 10(-9), OR = 1.42 for the rs2233152 SNP near MIA and RAB4B; P = 1.68 × 10(-12), OR = 1.52 for rs28493229 in ITPKC), which confirms previous findings(1). The involvement of the FCGR2A locus may have implications for understanding immune activation in Kawasaki disease pathogenesis and the mechanism of response to intravenous immunoglobulin, the only proven therapy for this disease.
Purpose: To validate magnetic resonance imaging (MRI) assessment of aortic pulse wave velocity (PWV MRI ) with PWV determined from invasive intra-aortic pressure measurements (PWV INV ) and to test the reproducibility of the measurement by MRI.
Materials and Methods:PWV MRI was compared with PWV INV in 18 nonconsecutive patients scheduled for catheterization for suspected coronary artery disease. Reproducibility of PWV MRI was tested in 10 healthy volunteers who underwent repeated measurement of PWV MRI at a single occasion. Velocity-encoded MRI was performed on all participants to assess PWV MRI in the total aorta (Ao total ), the proximal aorta (Ao prox ), and the distal aorta (Ao dist ). Conclusion: MRI assessment of aortic pulse wave velocity shows good agreement with invasive pressure measurements and can be determined with high reproducibility.
Results
Background-Pulmonary regurgitation (PR) late after total correction for tetralogy of Fallot may lead to progressive right ventricular (RV) dilatation and an increased incidence of severe arrhythmias and sudden death. Timing of pulmonary valve replacement (PVR) is subject to discussion, because the effect of PVR on RV function in adults is unclear. In this study, MRI was used to assess the effect of PVR on RV function and PR. Clinical improvement was established by means of the NYHA classification. Methods and Results-Twenty-six adult patients were included. Cardiac MRI was performed at a median of 5.1Ϯ3.4 months before and 7.4Ϯ2.4 months after PVR. Mean preoperative PR was 46Ϯ10% (range, 25% to 64%). After PVR, 20 of 26 patients (77%) showed no residual PR, 5 patients showed mild residual PR, and 1 patient showed moderate PR. RV end-diastolic volume (RV-EDV) decreased from 305Ϯ87 to 210Ϯ62 mL (PϽ0.001), and RV end-systolic volume (RV-ESV) decreased from 181Ϯ67 to 121Ϯ58 mL (PϽ0.001). No significant change was found in RV-EF (42% versus 42%
Presence of symptoms and documented AFL/AF are the best predictors of SD in TGA patients. Patients with these findings should be further evaluated for risk of SD.
NMR velocity mapping is an accurate method for the noninvasive, volumetric quantification of pulmonary regurgitation after surgical correction of tetralogy of Fallot.
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