Layer-specific strain and the degree of left ventricular thickness in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Tsugu ◽  
Y Nagatomo ◽  
R Dulgheru ◽  
S Marchetta ◽  
A Postolache ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Myocardial Dysfunction ◽  
Interventricular Septum ◽  
Area Under The Curve ◽  
Cellular Level ◽  
Left Ventricular ◽  
Myocardial Tissue ◽  
Characteristic Analysis ◽  
Specific Strain

Abstract Background Left ventricular (LV) wall thickness is an important parameter for the diagnosis of hypertrophic cardiomyopathy (HCM) and is also associated with long-term clinical outcome in HCM patients. However, conventional tools have failed to analyze the mechanisms of structural and functional abnormalities that occur at the cellular level in hypertrophied myocardial tissue. Recently, technological progression of 2D-speckle tracking echocardiography (2D-STE) has enabled the estimation of layer-specific strain (LSS), such as epicardial, mid-myocardial, and endocardial longitudinal strain, respectively. LSS may have the potential to elucidate the detailed mechanisms of myocardial dysfunction. Purpose The aim of this study was (i) to clarify the detailed mechanisms of structural and functional abnormalities of myocardial tissue in HCM using LSS (ii) to investigate the diagnostic accuracy of LSS for HCM. Methods Forty-one patients with HCM and preserved LV ejection fraction (LVEF) (66% male, 52±18 years, LVEF 62.9±3.7%) and 41 controls matched for age and sex (66% male, 52±20 years, LVEF 63.5±8.2%) underwent 2D-STE (GE-Healthcare, Vivid-E9). Quantitative strain values of epicardial, mid-myocardial, and endocardial layers were measured. Results LV wall thickness including interventricular septum thickness (HCM vs. Controls; 18.9±5.0 vs. 9.1±1.8, p<0.001), posterior wall thickness (11.5±2.5 vs. 8.8±1.9, p<0.001), and maximum wall thickness (20.1±4.3 vs. 9.4±0.4, p<0.001) were significantly lower in HCM than in Controls. Absolute values of LSS for all layers were lower in HCM than in Controls (HCM vs. Controls; epicardial; −13.1±3.3 vs. −19.5±1.6, p<0.001; mid-myocardial; −15.8±3.3 vs. −21.4±1.7, p<0.001; endocardial; −18.9±3.9 vs. −23.6±1.9, p<0.001). End/Epi ratio was higher in HCM than in Controls (HCM vs. Controls; 1.5±0.2 vs. 1.2±0.0, p<0.001). Next, we investigated the echocardiographic parameters that correlated with LV maximal wall thickness (MWT). End/Epi ratio was an independent predictor of LV MWT (β=0.96, p<0.001). Receiver operating characteristic analysis revealed that a higher End/Epi ratio (≥1.3) was the strongest predictor of diagnostic criteria for HCM (LV wall thickness ≥15 mm) (area under the curve 0.99, p<0.001, sensitivity 98%, specificity 97%). Conclusions In HCM patients with preserved LVEF, (i) LSS was lower and End/Epi ratio was higher than in controls. (ii) End/Epi ratio (≥1.3) was the strongest predictor of abnormal wall thickness of HCM. The mechanism of higher End/Epi ratio in HCM might be attributable to the more common myofibrillar disarray in mid- and epicardial layers. Variations of LSS represented by End/Epi ratio might have the potential to accurately detect HCM and to elucidate the pathophysiology of impaired LV wall motion at cellular level in HCM. Funding Acknowledgement Type of funding source: None

scholarly journals Novel Morphological Features on CMR for the Prediction of Pathogenic Sarcomere Gene Variants in Subjects Without Hypertrophic Cardiomyopathy

2021 ◽  
Vol 8 ◽  
Author(s):  
Nikki van der Velde ◽  
Roy Huurman ◽  
H. Carlijne Hassing ◽  
Ricardo P. J. Budde ◽  
Marjon A. van Slegtenhorst ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Area Under The Curve ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Morphological Features ◽  
Gene Variants ◽  
Healthy Controls ◽  
Score System

Background: Carriers of pathogenic DNA variants (G+) causing hypertrophic cardiomyopathy (HCM) can be identified by genetic testing. Several abnormalities have been brought forth as pre-clinical expressions of HCM, some of which can be identified by cardiovascular magnetic resonance (CMR). In this study, we assessed morphological differences between G+/left ventricular hypertrophy-negative (LVH-) subjects and healthy controls and examined whether CMR-derived variables are useful for the prediction of sarcomere gene variants.Methods: We studied 57 G+ subjects with a maximal wall thickness (MWT) < 13 mm, and compared them to 40 healthy controls matched for age and sex on a group level. Subjects underwent CMR including morphological, volumetric and function assessment. Logistic regression analysis was performed for the determination of predictive CMR characteristics, by which a scoring system for G+ status was constructed.Results: G+/LVH- subjects were subject to alterations in the myocardial architecture, resulting in a thinner posterior wall thickness (PWT), higher interventricular septal wall/PWT ratio and MWT/PWT ratio. Prominent hook-shaped configurations of the anterobasal segment were only observed in this group. A model consisting of the anterobasal hook, multiple myocardial crypts, right ventricular/left ventricular ratio, MWT/PWT ratio, and MWT/left ventricular mass ratio predicted G+ status with an area under the curve of 0.92 [0.87–0.97]. A score of ≥3 was present only in G+ subjects, identifying 56% of the G+/LVH- population.Conclusion: A score system incorporating CMR-derived variables correctly identified 56% of G+ subjects. Our results provide further insights into the wide phenotypic spectrum of G+/LVH- subjects and demonstrate the utility of several novel morphological features. If genetic testing for some reason cannot be performed, CMR and our purposed score system can be used to detect possible G+ carriers and to aid planning of the control intervals.

scholarly journals The dynamics of the blood content of N-terminal fragment of brain natriuretic peptide (NT-proBNP) in hypertrophic cardiomyopathy in children

2019 ◽  
Vol 19 (2) ◽  
pp. 75-80
Author(s):  
Vasilisa S. Ermolenko ◽  
I. V. Silnova ◽  
E. N. Basargina
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Brain Natriuretic Peptide ◽  
Pressure Gradient ◽  
Natriuretic Peptide ◽  
Wall Thickness ◽  
Myocardial Hypertrophy ◽  
Interventricular Septum ◽  
Left Ventricular ◽  
Blood Concentrations

N-terminal pro-brain natriuretic peptide (NT-proBNP) is the unbiased marker of congestive heart failure. Levels of this marker have not been studied in children with hypertrophic cardiomyopathy (HCM). There are presented data of the comparison of blood concentrations of NT-proBNP in hereditary HCM and other diseases associated with myocardial hypertrophy in children. There was examined 41 patient (age: from 3 to 90 months) with myocardial hypertrophy The patients were divided into 3 groups. There was evaluated the interrelationship between NT-proBNP concentrations and the severity of heart failure and echocardiographic parameters. Results. It was established that in the first group of patients with hereditary HCM (41,4%) there was revealed the significant positive relationship between NT-proBNP concentrations and values of the bloodflow velocity and pressure gradient in the LV outflow track, interventricular septum thickness and LVposterior wall thickness (LVPWT), in cases from the second group (34.1%) with syndromes of malformations and metabolic diseases - with RV anterior wall thickness and LVposterior wall thickness, bloodflow velocity and pressure gradient in the RV outflow track, in the third group with myocardial remodeling against background of arterial hypertension the positive correlation was revealed with LVPWT, whereas negative one - with the fraction of left ventricular output. Maximal blood level of NT-proBNP in blood was detected in pronounced hemodynamic disturbances in patients from the second and third group Conclusion. NT-proBNP level assessment is necessary for differentiated diagnostics of HCM and associated diseases.

scholarly journals 436 A peri-myocarditis unmasks an underlying hypertrophic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Francesca Rubino ◽  
Sofia Capocci ◽  
Leonardo Portolan ◽  
Alessia Gambaro ◽  
Michele Pighi ◽  
...  
Keyword(s):  
Chest Pain ◽  
Hypertrophic Cardiomyopathy ◽  
Arterial Hypertension ◽  
Medical Therapy ◽  
Wall Thickness ◽  
Interventricular Septum ◽  
Aortic Disease ◽  
Left Ventricular ◽  
Provisional Diagnosis ◽  
Acute Aortic Disease

Abstract Methods and results A 41-year-old black man complaining of severe oppressive chest pain radiated to the back presented to our accident & emergency department (A&E). His symptoms started few days before his hospital admission. Past medical history was remarkable for arterial hypertension in medical therapy and microdrepanocytosis. In A&E, the patient’s physical examination and vital signs were normal, he was normotensive, apyretic with normal oxygen saturation. The ECG showed ST elevation in anterior and lateral leads. Because of his history of arterial hypertension, and the severe chest pain irradiated to the back, an angio-CT was indicated at first. The CT ruled out acute aortic disease. Excluded the acute aortic disease, the patient underwent an urgent coronary angiography. No coronary stenosis was found. Therefore, the patient was admitted in cardiac intensive care unit. The blood test showed an elevation of high sensitive cardiac troponin T (cTnT peak 3093 ng/L) and inflammatory index (leukocytosis 13.65 109/l and protein C reactive peak 347 mg/L). An in-depth anamnestic collection revealed fever with respiratory symptoms about 2 weeks before. The echocardiography demonstrated left ventricular (LV) dysfunction with increased ventricular wall thickness and mild pericardial effusion. No LV outflow tract obstruction was found. A provisional diagnosis of peri-myocarditis was made. During hospitalization, anti-remodelling cardiac therapy was introduced and up titrated. To confirm the provisional diagnosis, a Gadolinium cardiac Magnetic Resonance (CMR) was performed, and it revealed myocardial oedema on basal anterior interventricular septum and multiple areas of late gadolinium enhancement with subepicardial pattern. Moreover, severe LV hypertrophy was confirmed (interventricular septum 19 mm, inferior wall 17 mm). This pattern was consistent to the diagnosis of peri-myocarditis on a hypertrophic cardiomyopathy (HCM). Main infectious causes of peri-myocarditis were investigated, but the results were inconclusive. Unfortunately, genetic test results are still not available. Patient was discharged with recovered LV systolic function and free of symptoms on optimal medical therapy; no ventricular arrhythmias was detected during hospitalization. HCM risk sudden cardiac death (SCD) was lower than 4%. Conclusions Peri-myocarditis can mimic symptoms of an acute coronary syndrome. Furthermore, the inflammation of cardiac muscle and the subsequent interstitial oedema may cause an increase in LV wall thickness. In this setting, the diagnosis of an underlying cardiomyopathy is challenging. This interesting and unusual case highlights the relevance of an accurate diagnostic work up to deliver good clinical practice. In particular, Gadolinium CMR is of paramount importance in this setting.

scholarly journals Aetiology-discriminative multimodality imaging of hypertrophic cardiomyopathy: deformation patterns relate to synchrotron-based assessment of microstructural tissue remodelling

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
F Loncaric ◽  
A Garcia-Alvarez ◽  
P Garcia-Canadilla ◽  
L Sanchiz ◽  
H Dejea ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Wall Thickness ◽  
Multimodality Imaging ◽  
Global Longitudinal Strain ◽  
Microstructural Analysis ◽  
Left Ventricular ◽  
Doppler Imaging ◽  
Deformation Analysis ◽  
Septal Myectomy ◽  
Regional Deformation

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – EU funding. Main funding source(s): Horizon 2020 European Commission Project H2020-MSCA-ITN-2016 (764738) and the Clinical Research in Cardiology grant from the Spanish Cardiac Society. Background The aetiology of left ventricular hypertrophy (LVH) is a relevant clinical challenge with consequences for patient management. Phenotypes resulting from hypertensive remodelling and sarcomere mutation often overlap. Synchrotron X-ray phase-contrast imaging (X-PCI) is a technique that can provide 3-dimensional detailed information on myocardial micro-structure non-destructively. The aim is to relate macrostructural/functional, non-invasive, imaging phenotypes of hypertrophic cardiomyopathy (HCM) to the underlying myocardial microstructure assessed with X-PCI. Methods Myocardial tissue samples were obtained from three patients (P1-3) with obstructive myocardial hypertrophy undergoing septal myectomy. Medical history and the 5-year HCM risk scores were evaluated. The patients were imaged with magnetic resonance imaging and echocardiography prior to procedure. Myocardial structure was assessed with wall thickness, late gadolinium enhancement (LGE), whereas function with speckle-tracking deformation (STE) and tissue Doppler imaging (TDI). Myectomy tissue was imaged with X-PCI in the TOMCAT beamline, using a multiscale propagation-based protocol combining a low-resolution (LR) and a high-resolution (HR) setup (5.8 and 0.7 um pixel size, respectively). Results The clinical and imaging data are shown in Fig 1. On initial assessment, wall thickness, LGE distribution, global longitudinal strain and septal TDI demonstrated a similar macrostructural and functional phenotype of P1 and P2, whereas P3 stood out with more severe hypertrophy, scarring and dysfunction. Additional regional deformation analysis with STE revealed reduced deformation in the basal and mid septum in P1, paired with a hypertensive pattern of post-systolic shortening (PSS) (yellow arrows). In comparison, in P2 and P3, deformation was more heterogeneous regionally, with regions of almost complete absence of deformation (orange arrows). Upon further exploration with TDI, areas with abnormal deformation were identified on the transition from basal to mid septum in both P2 and P3, whereas deformation was normal, but reduced in P1, and paired with PSS. LR X-PCI defined regions of interest to scan with HR (yellow frame), where HR revealed extensive interstitial fibrosis (orange arrow) with normal myocyte size and organisation in P1, compatible with severe hypertensive remodelling. However, in P2 and P3, patches of fibrosis (yellow arrow) paired with enlarged myocytes organized in visible disarray, considerably more prominent in P3, were both compatible with sarcomere-mutation HCM. Conclusion The results demonstrate multiscale phenotyping of HCM - relating micro- and macrostructural findings to function, and integrating multimodality data. In-depth regional deformation analysis, validated by synchrotron-based microstructural analysis, showed potential to identify distinct imaging phenotypes in HCM, distinguishing between overlapping presentations in different aetiologies. Abstract Figure 1

scholarly journals Prediction of Extubation Readiness Using Transthoracic Ultrasound in Preterm Infants

2020 ◽  
Author(s):  
Reem M. Soliman ◽  
Yasser Elsayed ◽  
Reem N. Said ◽  
Abdulaziz M. Abdulbaqi ◽  
Rania H. Hashem ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Preterm Infants ◽  
Pulmonary Artery Pressure ◽  
Neonatal Intensive Care ◽  
Ductus Arteriosus ◽  
Interventricular Septum ◽  
Area Under The Curve ◽  
Left Ventricular ◽  
Predictive Values ◽  
Artery Pressure

ABSTRACTObjectiveTo test the hypothesis that a lung ultrasound severity score (LUSsc) and assessment of left ventricular eccentricity index of the interventricular septum (LVEI) by focused heart ultrasound can predict extubation success in mechanically ventilated preterm infants with respiratory distress syndrome (RDS).DesignProspective observational study of premature infants <34 weeks’ of gestation age supported with mechanical ventilation due to RDS. LUSsc and LVEI were performed on postnatal days 3 and 7 by an investigator who was masked to infants’ ventilator parameters and clinical conditions. RDS was classified based on LUSsc into mild (score 0–9) and moderate-severe (score 10–18). A receiver operator curve was constructed to assess the ability to predict extubation success. Pearson’s correlation was performed between LVEI and pulmonary artery pressure (PAP).SettingLevel III neonatal intensive care unit, Cairo, Egypt.ResultsA total of 104 studies were performed to 66 infants; of them 39 had mild RDS (LUSsc 0–9) and 65 had moderate-severe RDS (score ≥10). LUSsc predicted extubation success with a sensitivity and a specificity of 91% and 69%; the positive and negative predictive values were 61% and 94%, respectively. Area under the curve (AUC) was 0.83 (CI: 0.75-0.91). LVEI did not differ between infants that succeeded and failed extubation. However, it correlated with pulmonary artery pressure during both systole (r=0.62) and diastole (r=0.53) and with hemodynamically significant patent ductus arteriosus (r=0.27 and r=0.46, respectively).ConclusionLUSsc predicts extubation success in preterm infants with RDS whereas LVEI correlates with high PAP.

Abstract 11093: Layer-specific Quantification of Myocardial Deformation by Strain Echocardiography May Reveal the Mechanism of Preserved Left Ventricular Ejection Fraction in Patients With Hypertrophic Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Jing Ping Sun ◽  
Xianda Ni ◽  
Tingyan Xu ◽  
Min Xu ◽  
Xing Sheng Yang ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ejection Fraction ◽  
Wall Thickness ◽  
Radial Strain ◽  
Left Ventricular ◽  
Relative Wall Thickness ◽  
Left Ventricular Ejection ◽  
Compensatory Mechanisms ◽  
Ventricular Ejection Fraction ◽  
Bull's Eye

Purpose: We aimed to evaluate compensatory mechanisms in hypertrophic cardiomyopathy (HCM) patients (pts) with preserved left-ventricular (LV) ejection fraction (EF). Methods: Speckle-tracking echocardiography (Vivid E9, GE) was performed in 50 HCM with preserved LV EF (38 m; 49± 14 y, all LV EF > 55%) and 50 age, gender matched controls (38 m; 49±12 y). The global and segmental longitudinal (LS), circumferential (CS) and radial strain (RS) strains of endocardia (End), mid-wall and epicardia layers were analyzed using a novel layer-specific TTE. The ratio of End to epicardia strain (End/Epi) was calculated. Results: The LV EF were similar in pts and controls (64±8 vs 64±7 %, p=0.95). The diastolic function was significantly impaired in HCM pts compared with controls (E/E’:18.4±8.4 vs 8.6 ±2.4, p<0.0001). The absolute value of LS and CS was reserved at apical End layers (-34±7 vs -35±6, p=0.44); the remaining segments and LV global LS and CS of three layers were significantly smaller (LS,-16±5 vs -22±3; CS -24±8 vs -33±7; p<0.0001), and LS and CS End/Epi (1.7±0.3 vs 1.3±0.1, 3.4±1.1 vs 1.7±0.2 respectively, P <0.0001) was significantly higher in HCM pts than in controls. The RS and LV twist were preserved in all LV segments (27±10 vs 24±12, p=0.19; 20±8 vs 18±5, p=0.33; respectively). In HCM pts, the LV LS value at basal and middle levels revealed significant negative correlations with LV relative wall thickness (r=–0.65, –0.59 and –0.60, –0.54, respectively , p< 0.0001); and mild negative correlations (r=-0.33,-0.29, p<0.0001). The LV CS value at all levels revealed mild correlations with relative wall thickness (r=-0.22, p<0.05) . The LS were significantly reduced at the hypertrophic segments (Figure). Conclusions: In HCM patients with preserved LVEF, LV GLS was impaired, but apical End LS and basal End CS, LV RS as well as LV twist were maintained as the compensation for reduction LV LS and CS. The Bull’s eye of LS may help us to localize the lesion segments and define the type of HCM.

scholarly journals P955 left ventricle cardiac remodeling among lithuanian football versus basketball players

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Aldujeli ◽  
J Laukaitiene ◽  
R Unikas
Keyword(s):  
Left Ventricle ◽  
Wall Thickness ◽  
Cardiac Remodeling ◽  
Interventricular Septum ◽  
Posterior Wall ◽  
Left Ventricular ◽  
Relative Wall Thickness ◽  
Football Players ◽  
Basketball Players ◽  
Lv Mass

Abstract Background Regular physical exercise causes a continuous gradual increase of the cardiac left ventricular (LV) mass known as physiological adaptive hypertrophy. The extent of LV remodeling depends on the type, amount, and intensity of the exercise. Purpose The aim of this study was to compare structural changes of the heart among Lithuanian football, basketball players and unathletic controls. Methods A total of 50 Lithuanian males aged between 20-29 years volunteered to participate in the study. Football players (n = 15) playing for local II league football clubs,and Basketball players (n = 15) playing for local minor league basketball teams. All athletes had been regularly engaged in their sport for at least three years. Inactive healthy volunteers (n = 20) of similar age served as controls. Routine transthoracic echocardiographic examinations to measure end-diastolic LV dimensions were performed by cardiology fellow under the supervision of a fully licensed cardiologist. Statistical analyses were performed using the SPSS 20.0 software. The value of p &lt; 0,05 was considered as statistically significant. Results No structural or functional pathologies were evident during the echocardiographic examination in any of the subjects. Absolute interventricular septum (IVS) thickness and LV posterior wall thickness, but not LV diameter, were higher in athletes than in inactive controls (P &lt; 0,001). Indexed LV diameter was higher in football players as compared with non-athlete controls and basketball players (P &lt; 0,05). Left ventricular mass of all athletes were higher as compared with controls (p &lt; 0.001). Relative wall thickness was not increased in football players but was higher in basketball players as compared with controls (p &lt; 0.05). Conclusion Cardiac remodeling in Lithuanian football players resulted in left ventricle eccentric hypertrophy due to the LV dilation, increased LV mass and relatively normal relative wall thickness. However in Lithuanian basketball players we noticed an increase in both relative wall thickness and LV mass resulting in LV concentric hypertrophy. Echocardiographic characteristics Groups n End-diastolic LV diameter(mm) End-diastolic Interventricular septum (mm) End-diastolic LV posterior wall LV mass Football Players 15 56.9 10.8 10.8 242 Basketball players 15 53.6 11.5 11.3 254 Inactive individuals 20 53.2 9.1 9.5 182 P value 0.01 &lt;0.001 &lt;0.001 &lt;0.01 Abstract P955 Figure.

scholarly journals Alcohol septal ablation in hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Juan José Santos Mateo ◽  
Juan R Gimeno
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Clinical Results ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Alcohol septal ablation (ASA) has become an alternative to surgical myectomy in obstructive hypertrophic cardiomyopathy since it was first introduced in 1994 by Sigwart. The procedure alleviates symptoms by producing a limited infarction of the upper interventricular septum, resulting in a decrease in left ventricular outflow tract (LVOT) gradient. The technique has been improved over time and the results are comparable with those of myectomy. Initial concerns about long-term outcomes have been largely resolved. In this review, we discuss indications, technical aspects, clinical results and patient selection to ASA.

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