Lester, Steven J.

Background and AimDividing patients with heart failure (HF) based solely on ejection fraction (EF) may over simplify the hemodynamic states of these patients. We describe a novel echo‐derived hemodynamic HF model based on flow (stroke volume index [SVI]) and left atrial pressure (E:E′) correlates.MethodsA retrospective analysis of patients admitted with HF with both reduced (HFrEF) and preserved EF (HFpEF). Patients were subdivided into four hemodynamic groups based on echocardiographic SVI (< or ≥35 mL/m2) and E/E′ (≥ or <15). Group A: normal flow and normal filling pressure, Group B: normal flow but high filling pressure, Group C: low flow and low filling pressure, and Group D: low flow and high filling pressure.ResultsA total of 176 patients were enrolled, 123 patients had HFrEF and 53 patients had HFpEF. Baseline characteristics were not statistically significant in both groups. In HFrEF, most patients were in group D compared to a heterogeneous distribution in HFpEF (P<.0001). In HFrEF, there was a trend toward an increase in B‐type natriuretic peptide levels with a decrease in SVI and increase in E/E′ (P=.05) but not in HFpEF. There was no difference in death, major adverse cardiac events, but a higher readmissions rate in the HFpEF group at 30 days and 18 months.ConclusionsHemodynamic subgroups differ between HFrEF and HFpEF. There is no difference in major adverse cardiovascular events between both groups with increased readmissions in HPpEF patients. Larger studies may help assess the impact of echo‐derived hemodynamic state on clinical outcome.

Lysozyme amyloidosis (ALys) is a rare form of hereditary amyloidosis that typically manifests with renal impairment, gastrointestinal (GI) symptoms, and sicca syndrome, whereas cardiac involvement is exceedingly rare and neuropathy has not been reported. Here, we describe a 40-year-old man with renal impairment, cardiac and GI symptoms, and peripheral neuropathy. Renal biopsy specimen analysis revealed amyloidosis with extensive involvement of glomeruli, vessels, and medulla. Amyloid was also detected in the GI tract. Echocardiographic and electrocardiographic findings were consistent with cardiac involvement. Proteomic analysis of Congo red–positive renal and GI amyloid deposits detected abundant lysozyme C protein. DNA sequencing of the lysozyme gene in the patient and his mother detected a heterozygous c.305T>C alteration in exon 3, which causes a leucine to serine substitution at codon 102 (Human Genome Variation Society nomenclature: p.Leu102Ser; legacy designation: L84S). We also detected the mutant peptide in the proband’s renal and GI amyloid deposits. PolyPhen analysis predicted that the mutation damages the encoded protein. Molecular dynamics simulations suggested that the pathogenesis of ALys p.Leu102Ser is mediated by shifting the position of the central β -hairpin coordinated with an antiparallel motion of the C-terminal helix, which may alter the native-state structural ensemble of the molecule, leading to aggregation-prone intermediates.

BackgroundThe development of a left ventricular (LV) apical pouch in patients with apical hypertrophic cardiomyopathy (aHCM) has been thought to be the transition point that can become an apical aneurysm, which is linked to higher risk of adverse events. In our study, we sought to compare the ability of transthoracic echocardiography (echo) and cardiac magnetic resonance imaging (cMRI) to accurately identify the presence of an apical pouch or aneurysm in patients with aHCM.MethodsWe retrospectively reviewed the charts of all consecutive patients that had features of aHCM on imaging. Data from cMRI and echo examinations were abstracted, and the ability of these diagnostic modalities to identify the presence of a LV apical pouch and aneurysm was analyzed.ResultsOf 31 patients with aHCM, 17 (54.8%) had an apical pouch and 2 were found to have apical aneurysm (6.5%) on cMRI. Echo with and without perflutren contrast was able to accurately identify both aneurysms, but only 47.1% (8/17) of apical pouches seen by cMRI. Two patients had apical thrombus that was identified by cMRI, but not by echo.ConclusionOur findings indicate that cMRI is superior to echo in identifying apical pouches in patients with aHCM. Our results also suggest that in patients undergoing echo, the use of perflutren contrast for LV opacification increases the diagnostic yield. Further study is necessary to delineate whether earlier identification of an apical pouch will be of clinical benefit for patients with aHCM by altering clinical management and avoiding adverse cardiovascular events.

The authors describe transient apical thickening mimicking apical hypertrophic cardiomyopathy following apical ballooning syndrome. These findings are observed on multimodality cardiac imaging and appear to constitute a novel entity. Possible pathophysiologic mechanisms are explored.

In patients with aortic stenosis (AS) and eccentric transaortic flow, greater pressure loss occurs as the jet collides with the aortic wall together with delayed and diminished pressure recovery. This leads to the elevated transaortic valve pressure gradients noted on both Doppler and cardiac catheterization. Such situations may present a diagnostic dilemma where traditional measures of stenosis severity indicate severe AS, while imaging modalities of the aortic valve geometric aortic valve area (GOA) suggest less than severe stenosis. In this study, we present a series of cases exemplifying this clinical dilemma and demonstrate how color M‐mode, 2D and 3D transthoracic (TTE) and transesophageal (TEE) echocardiography, cardiac computed tomography angiography (CTA), and magnetic resonance imaging (MRI), may be used to resolve such discrepancies.

ObjectivesThe clinical severity of aortic stenosis (AS) is based largely on symptoms. However, AS severity is primarily determined by estimating the aortic valve area (AVA) and pressure gradients (ΔP). Conditions may arise in which there is a mismatch in severity between AVA and ΔP determinations secondary to errors in measurement and/or assumption, alterations of flow, or variations in the magnitude of pressure recovery. The cause of discrepancy between area and gradient determinations must be deciphered so as to best counsel patients on the most ideal treatment strategy. (J Interven Cardiol 2013;26:183–194)