Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy
Exercise stress echocardiography (ESE) is a valuable diagnostic and prognostic tool in adults with hypertrophic cardiomyopathy (HCM). Inducible and resting left ventricular outflow tract gradients are important predictors of heart failure. However, there are minimal data on the utility of this modality in children.
AbstractHypertrophic cardiomyopathy (HCM) is a prevalent cardiomyopathy in children, with variable etiologies, phenotypes, and associated syndromic genetic disorders (GD). The spectrum of evaluation in this heterogeneous population has not been well described. We aimed to describe mortality and medical management in the pediatric HCM population, and compare HCM pediatric patients with GD to those without GD. Children (
CONCLUSION: The results of our study show that similar to many centers, the etiology of HCM is often uncertain. In the absence of etiology, treatment aimed at the cause is either impossible or, at best, empirical. PMID: 32419413 [PubMed - in process]
Conclusions BBs differ within the class in so many ways that it is not only logical but also practical for real-life clinical practice that experts in the field make a list of the compelling indications for individual BBs. Time has come to incorporate our knowledge about BBs into a new approach of presenting these useful and very individual drugs. PMID: 32418526 [PubMed - as supplied by publisher]
Abstract BACKGROUND: Although elevated B-type natriuretic peptide (BNP) levels predict outcome in patients with hypertrophic cardiomyopathy (HCM), the association between BNP levels and outcome in patients with the apical phenotype of HCM remains unclear. We evaluated the impact of elevated BNP levels on outcome in a cohort of apical HCM patients. METHODS: Among 432 HCM patients, 144 with an apical phenotype were examined. Plasma BNP levels were measured at the time of the initial evaluation. RESULTS: The median (interquartile range) BNP level at initial evaluation in these patients was 188.5 (72.0-334.4...
This study aims to develop a mapping algorithm between the MLHFQ and the Assessment of Quality of Life (AQoL) 8D utility instrument in patients with dilated cardiomyopathy (DCM).MethodsMLHFQ and AQoL-8D data were collected on 61 Australian adults with idiopathic DCM or other non-hypertrophic cardiomyopathies. Three statistical methods were used as follows: ordinary least squares (OLS) regression, the robust MM estimator, and the generalised linear models (GLM). Each included a range of explanatory variables. Model performance was assessed using key goodness-of-fit measures, the mean absolute error (MAE), and the root-mean-...
Conclusion HCM is a relatively benign cardiomyopathy in Greece, similarly to other countries. Apical hypertrophy pattern is more common in Greece than in the other European countries, whereas the presence of apical aneurysm is the most important risk factor for arrhythmic events on top of the established risk factors for sudden death.
Conclusions: Our findings provide compelling evidence that ferritin plays a major role in protecting against cardiac ferroptosis and subsequent heart failure, thereby providing a possible new therapeutic target for patients at risk of developing cardiomyopathy. PMID: 32349646 [PubMed - as supplied by publisher]
ConclusionsAdolescent RHR is associated with future risk of HF, regardless of associated aetiological condition. The association was strongest for HF associated with CM, driven by the association with dilated CM. These findings indicate a causal pathway between elevated RHR and myocardial dysfunction that warrants further investigation.
A unique clinical circumstance involving middle-aged male identical twins with obstructive hypertrophic cardiomyopathy (HC) is reported. The concordance of morphologic (i.e., phenotype) findings and clinical course between the 2 patients is remarkable, including timing of the onset and progression of heart failure due to left ventricular outflow tract obstruction, frequency of paroxysmal atrial fibrillation and beneficial response to surgical myectomy and Cox-Maze IV procedure (performed 14 days apart).
AbstractPurpose of ReviewThis review summarizes the evidence concerning the imaging techniques and features to (a) diagnose and (b) risk stratify patients with, or at risk of, inherited cardiomyopathies. The inherited cardiomyopathies we have focused on are dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM) and arrhythmogenic cardiomyopathies (arrhythmogenic right ventricular cardiomyopathy (AVRC), arrhythmogenic left ventricular cardiomyopathy (ALVC) and noncompaction cardiomyopathy (NCCM)).Recent FindingsEchocardiogram and cardiac magnetic resonance imaging (CMR) still form the foundations of the diagnosis a...