scholarly journals Imaging systolic and diastolic heart murmurs in hypertrophic cardiomyopathy

2014 ◽  
Vol 1 (1) ◽  
pp. 1
Author(s):  
Andrea Barison ◽  
Giancarlo Todiere ◽  
Pier Giorgio Masci ◽  
Giovanni D Aquaro

Muscular obstruction in hypertrophic cardiomyopathy can occur not only in systole, but also in diastole. Here we report a case of hypertrophic cardiomyopathy in a middle-aged lady: echocardiography and cardiovascular magnetic resonance confirmed the diagnosis of hypertrophic cardiomyopathy with significant systolic and diastolic midventricular obstruction, corresponding to the murmurs audible in mid-systole and early-diastole respectively. Moreover, the mid-systolic murmur was clearly distinguishable from the mitral regurgitation murmur, which was audible throughout systole (pan-systolic) and likely related to systolic anterior motion of the mitral chords; similarly, the early-diastolic heart murmur due to diastolic obstruction was  better audible at the apex and clearly distinguishable from common diastolic murmurs due to aortic regurgitation. Echocardiography (with Doppler recordings) and magnetic resonance (with turbulence flows at steady state free precession imaging) allowed a detailed visualization of each audible murmur (systolic obstruction, mitral regurgitation, diastolic obstruction). In particular, while systolic obstruction has been extensively studied in hypertrophic cardiomyopathy, only very few reports about diastolic obstruction exist, describing either a normodirected midventricular diastolic obstruction (forward flow) similar to this case or a paradoxical midventricular diastolic obstruction (backward flow) in patients with an apical aneurysm emptying during early diastole into the left ventricular base through a narrow midventricular channel. Whether diastolic obstruction implies worse prognosis or requires specific treatment, deserves further research.

2020 ◽  
Vol 21 (12) ◽  
pp. 1341-1350 ◽  
Author(s):  
Kai Yang ◽  
Yan-Yan Song ◽  
Xiu-Yu Chen ◽  
Jia-Xin Wang ◽  
Lu Li ◽  
...  

Abstract Aims Hypertrophic cardiomyopathy (HCM) with left ventricular apical aneurysm (LVAA) is associated with an increased risk of adverse cardiovascular events. However, the clinical significance of LVAA in apical HCM (ApHCM) has not been reported. This study aimed to investigate the prevalence, cardiac magnetic resonance (CMR) characteristics, and prognosis of LVAA in ApHCM patients. Methods and results A total of 1332 consecutive ApHCM patients confirmed by CMR in our hospital were retrospectively analysed. LVAAs were identified in 31 patients of all ApHCM patients (2.3%, 31/1332). Besides, 31 age- and gender-matched ApHCM patients without LVAA were used for comparison. Of the 31 aneurysm patients (mean age, 53.8 ± 15.1 years old), 28 (90.3%) had clinical symptoms, and 3 (9.7%) had a family history of HCM. The rate of missed diagnosis of echocardiography for detecting LVAA was 64.5% (20/31), most (90%, 18/20) of unidentified LVAAs by echocardiography were small aneurysms (<20 mm). Compared with ApHCM patients without LVAA, the proportion of systolic mid-cavity obstruction and late gadolinium enhancement (LGE) presence, and the LGE extent in ApHCM patients with LVAA were significantly higher (all P<0.05). The Kaplan–Meier curves showed that the event-free survival rate in ApHCM patients with LVAA was significantly lower than that in ApHCM patients without LVAA (log rank, P = 0.010). Conclusion ApHCM with LVAA is a very rare condition, which is often missed by echocardiography and could be reliably detected with CMR and is associated with a higher risk of adverse cardiovascular events compared with ApHCM without LVAA.


2019 ◽  
Vol 03 (03) ◽  
Author(s):  
Parthena Theodoridou ◽  
Despoina Masmanidou ◽  
Panagiotis Kousidis ◽  
Panagiotis Roumelis ◽  
Anastasios Tsarouchas ◽  
...  

Author(s):  
Zsofia Dohy ◽  
Liliana Szabo ◽  
Attila Toth ◽  
Csilla Czimbalmos ◽  
Rebeka Horvath ◽  
...  

AbstractThe prognosis of patients with hypertrophic cardiomyopathy (HCM) varies greatly. Cardiac magnetic resonance (CMR) is the gold standard method for assessing left ventricular (LV) mass and volumes. Myocardial fibrosis can be noninvasively detected using CMR. Moreover, feature-tracking (FT) strain analysis provides information about LV deformation. We aimed to investigate the prognostic significance of standard CMR parameters, myocardial fibrosis, and LV strain parameters in HCM patients. We investigated 187 HCM patients who underwent CMR with late gadolinium enhancement and were followed up. LV mass (LVM) was evaluated with the exclusion and inclusion of the trabeculae and papillary muscles (TPM). Global LV strain parameters and mechanical dispersion (MD) were calculated. Myocardial fibrosis was quantified. The combined endpoint of our study was all-cause mortality, heart transplantation, malignant ventricular arrhythmias and appropriate implantable cardioverter defibrillator (ICD) therapy. The arrhythmia endpoint was malignant ventricular arrhythmias and appropriate ICD therapy. The LVM index (LVMi) was an independent CMR predictor of the combined endpoint independent of the quantification method (p < 0.01). The univariate predictors of the combined endpoint were LVMi, global longitudinal (GLS) and radial strain and longitudinal MD (MDL). The univariate predictors of arrhythmia events included LVMi and myocardial fibrosis. More pronounced LV hypertrophy was associated with impaired GLS and increased MDL. More extensive myocardial fibrosis correlated with impaired GLS (p < 0.001). LVMi was an independent CMR predictor of major events, and myocardial fibrosis predicted arrhythmia events in HCM patients. FT strain analysis provided additional information for risk stratification in HCM patients.


2020 ◽  
Vol 19 (2) ◽  
pp. 18-23
Author(s):  
I. E. Obramenko

Introduction. About 0.2 % of the adult population all over the world suffers from hypertrophic cardiomyopathy. Early and timely diagnosis of the apical form of hypertrophic cardiomyopathy remains an urgent medical problem, since the disease has a wide variability of clinical manifestations and often occurs asymptomatic or with symptoms of other heart diseases. Magnetic resonance imaging is an informative method of radiation diagnosis of hypertrophic cardiomyopathy. The aim is improving of radiology diagnostics in applying to the apical form of hypertrophic cardiomyopathy. Materials and methods. 98 patients with apical hypertrophic cardiomyopathy aged 19 to 76 years were еxamined. There were 48 men and 50 women. All subjects were examined by a cardiologist, all patients underwent electrocardiography and echocardiography, 45 patients underwent magnetic resonance imaging (MRI) of the heart. Results. In our study 13 patients had MRI determined the isolated form of apical form of hypertrophic cardiomyopathy, 32 had combined one. 42.2% of the patients with symmetrical hypertrophy of all apical segments had sawtoothed configuration of the LV revealed by MRI. The symptom of left ventricular cavity obliteration was determined in 19 patients. The symptom of LV cavity sequestration was determined in 5 subjects. 5 patients had an aneurysm on the top of the left ventricle, 1 – on the top of the right ventricle. Signs of left ventricular outflow tract obstruction were visualized in 2 patients, intraventricular obstruction at the level of the middle segments of the left ventricle was determined in 5 cases. Akinesis and hypokinesis were detected in areas of fibrous changes (n=21) or in areas of cardiosclerosis (n=2). In 17.8 % of subjects identified non-compacted myocardium, in 3 cases it was combined with apical form of hypertrophic cardiomyopathy. With contrast enhancement in 29 patients, foci (n=22) or zones (n=7) of pathological accumulation of contrast agent were determined, which indicated the replacement of myocardium with fibrous tissue.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Elizabeth W. Thompson ◽  
Srikant Kamesh Iyer ◽  
Michael P. Solomon ◽  
Zhaohuan Li ◽  
Qiang Zhang ◽  
...  

Abstract Background Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular wall thickness, cardiomyocyte hypertrophy, and fibrosis. Adverse cardiac risk characterization has been performed using late gadolinium enhancement (LGE), native T1, and extracellular volume (ECV). Relaxation time constants are affected by background field inhomogeneity. T1ρ utilizes a spin-lock pulse to decrease the effect of unwanted relaxation. The objective of this study was to study T1ρ as compared to T1, ECV, and LGE in HCM patients. Methods HCM patients were recruited as part of the Novel Markers of Prognosis in Hypertrophic Cardiomyopathy study, and healthy controls were matched for comparison. In addition to cardiac functional imaging, subjects underwent T1 and T1ρ cardiovascular magnetic resonance imaging at short-axis positions at 1.5T. Subjects received gadolinium and underwent LGE imaging 15–20 min after injection covering the entire heart. Corresponding basal and mid short axis LGE slices were selected for comparison with T1 and T1ρ. Full-width half-maximum thresholding was used to determine the percent enhancement area in each LGE-positive slice by LGE, T1, and T1ρ. Two clinicians independently reviewed LGE images for presence or absence of enhancement. If in agreement, the image was labeled positive (LGE + +) or negative (LGE −−); otherwise, the image was labeled equivocal (LGE + −). Results In 40 HCM patients and 10 controls, T1 percent enhancement area (Spearman’s rho = 0.61, p < 1e-5) and T1ρ percent enhancement area (Spearman’s rho = 0.48, p < 0.001e-3) correlated with LGE percent enhancement area. T1 and T1ρ percent enhancement areas were also correlated (Spearman’s rho = 0.28, p = 0.047). For both T1 and T1ρ, HCM patients demonstrated significantly longer relaxation times compared to controls in each LGE category (p < 0.001 for all). HCM patients also showed significantly higher ECV compared to controls in each LGE category (p < 0.01 for all), and LGE −− slices had lower ECV than LGE + + (p = 0.01). Conclusions Hyperenhancement areas as measured by T1ρ and LGE are moderately correlated. T1, T1ρ, and ECV were elevated in HCM patients compared to controls, irrespective of the presence of LGE. These findings warrant additional studies to investigate the prognostic utility of T1ρ imaging in the evaluation of HCM patients.


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