MRI discovers heart damage among male triathletes
Using late gadolinium-enhanced MRI scans, German researchers found myocardial...Read more on AuntMinnie.comRelated Reading: Some cardiac MRI findings predict future heart events Stress cardiac MR matches 320-row CCTA results Study shows feasibility of cardiac PET/MRI -- with caveats Cardiac MR tops ECG in finding hidden myocardial infarctions Late MRI enhancement predicts death risk in hypertrophic cardiomyopathy
AbstractCoronary microvascular dysfunction and, its functional consequence, myocardial ischemia are common pathologic features in patients with hypertrophic cardiomyopathy (HCM). Both have been commonly invoked as potential triggers of and/or contributors to the underlying pathophysiological processes leading to heart failure, and malignant ventricular arrhythmias. Positron emission tomography (PET) with myocardial blood flow quantification provides a unique opportunity to evaluate the integrity and function of the coronary microcirculation in HCM. The purpose of the present review is to summarize all the pertinent literat...
Hypertrophic cardiomyopathy (HCM) is thought to be a leading cause of sudden cardiac death (SCD) in athletes, and while SCD is the most dramatic and feared of all HCM presentations, its exact incidence remains unclear. Current expert opinion and consensus panels that formulated exercise recommendations in HCM to reduce the risk of sudden death by avoiding competitive sport are based on scant, observational, often circumstantial, and sometimes conflicting evidence. These recommendations rely on multiple cross-referencing of few original papers from a limited number of research groups.
Conclusions -LGE was present in 46% of children and adolescents with overt HCM, in contrast to ~60% typically reported in adult HCM. In the subset of patients with serial imaging, statistically significant increases in LGE, left ventricular mass and left atrial size were detected over 2.5 years, indicating disease progression over time. Further prospective studies are required to confirm these findings and to better understand the clinical implications of LGE in pediatric HCM. PMID: 29622585 [PubMed - as supplied by publisher]
Abstract Left ventricular outflow tract obstruction is observed in 70% of patients with hypertrophic cardiomyopathy, which occurs in about 1 of every 500 adults in the general population. It has been widely believed that the motion of the mitral valve, in particular, its systolic anterior motion (SAM), attributes significantly to such obstruction. For a better understanding of the mitral valve motion, a 3D patient-specific fluid-structure interaction model of the left ventricle from a patient with hypertrophic obstructive cardiomyopathy based on computed tomography (CT) scan images was proposed in this study. Disp...
Hypertrophic cardiomyopathy (HC) patients are at increased risk for sudden cardiac death (SCD). Abnormalities in myocardial blood flow (MBF) detected by positron emission tomography (PET) are common in HC, but a PET marker that identifies patients at risk for SCD is lacking. We hypothesized that disparities in regional myocardial perfusion detected by PET would identify HC patients at risk for ventricular arrhythmias. In order to test this hypothesis, we quantified global and regional MBF by 13NH3-PET at rest/stress, and developed a heterogeneity index to assess MBF heterogeneity, in 133 symptomatic HC patients.
Patients with hypertrophic cardiomyopathy (HC) are at increased risk of sudden cardiac death. Abnormalities in myocardial blood flow (MBF) detected by positron emission tomography (PET) are common in HC, but a PET marker that identifies patients at risk of sudden cardiac death is lacking. We hypothesized that disparities in regional myocardial perfusion detected by PET would identify patients with HC at risk of ventricular arrhythmias. To test this hypothesis, we quantified global and regional MBFs by 13NH3-PET at rest and at stress, and developed a heterogeneity index to assess MBF heterogeneity in 133 symptomatic patients with HC.
ConclusionsAnatomic characteristics specific to HCM hearts contribute to lower correlations between MBF/MFR values obtained by PMod and QPET, compared with non-HCM patients. These differences indicate that PMod and QPET cannot be used interchangeably for MBF/MFR analyses in HCM patients.
We present two boys with Noonan syndrome and the identical de novo RAF1 missense variant c.1082G>C/p.(Gly361Ala) affecting the CR3, but located outside the kinase activation segment. The p.(Gly361Ala) mutation has been identified as a RAF1 allele conferring resistance to RAF inhibitors. This amino acid change favors a RAF1 conformation that allows for enhanced RAF dimerization and increased intrinsic kinase activity. Both patients with Noonan syndrome showed typical craniofacial dysmorphism, macrocephaly, and short stature. One individual developed HCM and was diagnosed with a disseminated oligodendroglial‐like leptom...