scholarly journals Cardiac remodelling and heart failure symptoms in patients with apical hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Rosca ◽  
D Ciuperca ◽  
L Mandes ◽  
M Trofin ◽  
A Calin ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Although earlier publications suggested a more benign clinical course for patients (pts) with apical hypertrophic cardiomyopathy (ApHCM), recent studies report increased morbidity and mortality, comparable to the prognosis of other HCM variants. Moreover, information regarding cardiac remodelling and its relationship with symptoms in pts with ApHCM is scarce. The aim of our study was to assess left ventricular (LV), right ventricular (RV) and left atrial (LA) remodelling in pts with ApHCM in comparison with non-apical variants of HCM (nonApHCM), and the impact of cardiac remodelling on heart failure symptoms. Methods One hundred fifty-one consecutive pts with HCM (52 ± 16 yrs, 47% men) in sinus rhythm and with preserved LV ejection fraction (16 pts with ApHCM and 135 pts with nonApHCM), were prospectively enrolled. Comprehensive echocardiography was performed in all, including the measurement of maximal LV wall thickness (LVWT), RV free wall thickness (RVWT) and maximal LA volume indexed to body surface area (LAVi). Global LV strain (ɛ), RVɛ, LAɛ and end-diastolic LA strain rate (ASr) were measured using speckle-tracking echocardiography (STE). The ratio of E to average e’ was used to estimate LV filling pressure. The degree of mitral regurgitation (1/2/3) has also been assessed. Heart failure symptoms were defined according to the New York Heart Association (NYHA) classification. Results Forty-eight pts in nonApHCM group had intraventricular obstruction. There were no significant differences between pts with and without ApHCM regarding: age (58 ± 20 vs 52 ± 16 yrs), gender distribution, RVWT, LVɛ (-14.9 ± 2.7 vs -13.9 ± 3.5 %), RVɛ (-19.6 ± 3.6 vs -10.0 ± 5.0%), LAɛ (19.2 ± 5.8 vs 16.4 ± 7.1%)(p > 0.05 for all). Pts with ApHCM had lower values for LVWT (17.2 ± 1.9 vs 21.4 ± 5.2 mm, p = 0.002), E/e’ (12.0 ± 5.8 vs 17.6 ± 8.5, p = 0.02) and LAVi (48 ± 16 vs 61 ± 25, p = 0.03) compared to pts with nonApHCM. Pts with ApHCM had slightly better LA contractile function as assessed by ASr (-1.23 ± 0.50 vs -0.97 ± 0.49 sec-1, p = 0.05). Mitral regurgitation was more often severe in nonApHCM pts (56/42/31 vs 10/4/0, p < 0.001). There was no significant difference between the percentage of symptomatic pts (NYHA class ≥2) in ApHCM vs nonApHCM group (p = 0.3). In the ApHCM group, symptomatic pts had significantly lower ASr compared to asymptomatic pts (-0.98 ± 0.35 vs -1.61 ± 0.46 sec-1, p = 0.01). Conclusions Despite of lower LVWT values, less severe MR and no obstruction, pts with apical HCM have similar prevalence of heart failure symptoms, and similar LV, RV and LA dysfunction compared to pts with non-apical HCM. Symptomatic pts with apical HCM have worse LA contractile function compared to asymptomatic pts.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T Keteepe-Arachi ◽  
A Malhotra ◽  
J Basu ◽  
G Parry-Williams ◽  
B Ensam ◽  
...  

Abstract Background Structural cardiac adaptations due to hypertension (HTN) present a diagnostic challenge when differentiating from hypertrophic cardiomyopathy (HCM), using traditional imaging techniques such as echocardiography (echo). Cardiac magnetic resonance imaging (CMR) offers reproducible anatomical, functional quantification and myocardial tissue characterisation which discriminates between hypertension and HCM. Purpose To identify hypertensive individuals with undiagnosed HCM using CMR imaging. Methods 100 consecutive hypertensive patients underwent CMR at a tertiary centre dedicated blood pressure clinic (55% male, mean age 51 years). In keeping with ESC guidelines, end diastolic wall thickness (EDWT) ≥15mm identified individuals within the “grey zone” between hypertension and with a potential HCM diagnosis. 19 individuals were referred on to the dedicated inherited cardiac conditions clinic for further evaluation. Four patients expressed a definitive LV phenotype and were diagnosed with HCM. CMR parameters were compared in three groups: Hypertensive (HTN), “grey zone” Hypertensive (GZH) and HCM. Results CMR demonstrated end diastolic wall thickness (EDWT) >11 mm in 50% of hypertensives. 73% of the referred patients were Afro-Caribbean (AC) and all 4 HCM patients were AC. All referrals demonstrated EDWTs ≥14mm, 9 (47%) demonstrated late gadolinium enhancement of which 3 (16%) had HCM. Three had asymmetrical septal hypertrophy – 2 were in the HCM cohort and one underwent endomyocardial biopsy confirming HTN. Left ventricular mass index (LVMI) was significantly higher in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0004). EDWT was significantly greater in GZH compared to HTN (p<0.0001) and in HCM compared to HTN (p=0.0002). There was no significant difference in these parameters between GZH and HCM. Table 1. P-values for CMR data in hypertensive (HTN), gray zone hypertensive (GZH) and hypertrophic cardiomyopathy (HCM) cohorts CMR Parameter HCM vs. HTN GZH vs. HTN GZH vs. HCM EDVI NS p=0.025 NS EDWT p=0.0002 p<0.0001 NS LVMI p=0.0004 p<0.0001 NS Conclusion This study reports a 4% prevalence of HCM among hypertensive patients - 20 x greater than in the general population - which would be left undiagnosed using echo alone. Screening hypertensive individuals with CMR is not routine but we advocate its use in these individuals especially in Afro Caribbeans and in those in the “grey zone”, to identify undiagnosed HCM, which has significant implications for lifestyle modification and family screening. Acknowledgement/Funding Cardiac Risk in the Young


2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Marlena Schnieder ◽  
Anneki von Glasenapp ◽  
Amelie Hesse ◽  
Marios N. Psychogios ◽  
Mathias Bähr ◽  
...  

The impact of heart failure on outcome in stroke patients is not fully understood. There is evidence for an increased mortality and morbidity, but it remains uncertain whether thrombectomy in patients with large vessel occlusion (LVO) in the anterior circulation is less effective in patients with heart failure compared to patients without. Retrospectively, we analyzed echocardiographic data of all patients in our stroke database, who underwent mechanical thrombectomy (n=668) for the presence of heart failure. Furthermore, we collected baseline characteristics and neurological and neuroradiological parameters. In the analysis, 373 of the 668 patients of our stroke database underwent echocardiography. Of these 373 patients, 90 patients (24%) suffered from heart failure with reduced left ventricular ejection fraction measured by echocardiography according to the current guidelines. After adjustment for age, the Alberta stroke program early CT score (ASPECTS), and time from symptom onset to recanalization, the analysis revealed that thrombectomy in patients with heart failure and LVO is not associated with less favorable outcome measured by the modified Rankin Scale after 90 days (3 (0-6) vs. 3 (1-5); p=0.380). Moreover, we could not find a significant difference in mortality compared to patients without heart failure (11.0% vs. 7.4%; p=0.313).


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
A Demirtola ◽  
TS Tan ◽  
A Mammadli ◽  
IM Akbulut ◽  
I Dincer

Abstract Funding Acknowledgements Type of funding sources: None. Purpose Cardiac resynchronization therapy (CRT) has  a positive effect on the improvement of functional mitral regurgitation in patients with low ejection heart failure. However geometric changes in the mitral valve apparatus, subvalvular structures and their contribution to  the improvement of mitral regurgitation after CRT have not  been clearly defined. The aim of our study was to evaluate the geometric parameters of mitral valve apparatus measured with 3Dimensional (3D) transesophageal echocardiography (TEE) before CRT implantation and to determine the parameters predicting the improvement of mitral regurgitation after CRT. Methods Thirty patients with moderate or severe mitral regurgitation with low EF heart failure planned for CRT implantation and had an indication for TEE were included in the study. Effective regurgitant orifice (ERO) and regurgitant volume (RV) measurements were performed before CRT implantation. Detailed quantitative measurements of mitral valve were done from recorded images by 3D TEE. ERO, RV measurements were repeated to evaluate mitral regurgitation at the end of 3rd month. Results There were no significant changes in left ventricular EF and left ventricular diameters at the end of 3rd month, whereas ERO and RV values were decreased. A statistically significant difference was found in  posterior leaflet angle between mitral regurgitation responder and non-responder groups.  (28,93 ± 8,41 vs 41,25 ± 10,90, p = 0,006). Conclusion Heart failure patients with moderate or severe functional mitral regurgitation who underwent CRT implantation were found to have lower posterior leaflet angle measured by 3D TEE in the patient group whose mitral regurgitation improved after CRT. Abstract Figure.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A De Marvao ◽  
K McGurk ◽  
S Zheng ◽  
M Thanaj ◽  
W Bai ◽  
...  

Abstract Background Hypertrophic cardiomyopathy (HCM) is caused by rare variants in sarcomere-encoding genes, but little is known about the clinical significance of these variants in the general population. Purpose To determine the population prevalence of HCM-associated sarcomeric variants, characterise their phenotypic manifestations, estimate penetrance, and identify associations between sarcomeric variants and clinical outcomes, we performed an observational study of 218,813 adults in the UK Biobank (UKBB), of whom 200,584 have whole exome sequencing (WES). Methods We carried out an integrated analysis of WES and cardiac magnetic resonance (CMR) imaging in UK Biobank participants stratified by sarcomere-encoding variant status. Computer vision techniques were used to automatically segment the four chambers of the heart (Figure 1). Cardiac motion analysis was used to derive strain and strain rates. Regional analysis of left ventricular wall thickness was performed using three-dimensional modelling of these segmentations. Results Median age at recruitment was 58 (IQR 50–63 years), and participants were followed up for a median of 10.8 years (IQR 9.9–11.6 years) with a total of 19,507 primary clinical events reported. The prevalence of rare variants (allele frequency &lt;0.ehab724.17314) in HCM-associated sarcomere-encoding genes in 200,584 participants was 2.9% (n=5,727; 1 in 35), and the prevalence of pathogenic or likely pathogenic variants (SARC-P/LP) was 0.24% (n=474, 1 in 423). SARC-P/LP variants were associated with increased risk of death or major adverse cardiac events (MACE) compared to controls (HR 1.68, 95% CI 1.37–2.06, p&lt;0.001), mainly due to heart failure endpoints (Figure 2: cumulative hazard curves with zoomed plots for lifetime risk of A) death and MACE or B) heart failure, stratified by genotype; genotype negative (SARC-NEG), carriers of indeterminate sarcomeric variants (SARC-IND) or SARC-P/LP; C) Forest plot of comparative lifetime risk of clinical endpoints by genotype). While males had a higher overall risk of adverse outcomes, the incremental genetic risk from SARC-P/LP mutations was greater in females (HR for females: 2.18 CI 1.65–2.89, p&lt;0.001; HR for males: 1.42 CI 1.05–1.9, p=0.02). In 21,322 participants with CMR, SARC-P/LP were associated with asymmetric increase in left ventricular maximum wall thickness (10.9±2.7 vs 9.4±1.6 mm, p&lt;0.001) but hypertrophy (≥13mm) was only present in 16% (n=7/43, 95% CI 7–31%). Other rare sarcomere-encoding variants had a weak effect on wall thickness (9.5±1.7 vs 9.4±1.6 mm, p=0.002) with no combined excess cardiovascular risk. Conclusions In the general population, SARC-P/LP variants have low aggregate penetrance for overt HCM but are associated with increased risk of adverse cardiovascular outcomes and a sub-clinical cardiomyopathic phenotype. Although absolute event rates are low, identification of these variants may enhance risk stratification beyond familial disease. FUNDunding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): The study was supported by the Medical Research Council, UK (MC-A651-53301); National Institute for Health Research (NIHR) Imperial College Biomedical Research Centre; NIHR Royal Brompton Cardiovascular Biomedical Research Unit; British Heart Foundation (NH/17/1/32725, RG/19/6/34387, RE/18/4/34215).


Kardiologiia ◽  
2019 ◽  
Vol 59 (9) ◽  
pp. 13-19
Author(s):  
Yu. E. Karevа ◽  
V. U. Efendiev ◽  
S. S. Rakhmonov ◽  
A. M. Chernyavsky ◽  
V. L. Lukinov

Aim: to assess effect of correction of moderate ischemic mitral regurgitation (IMR) in patients with ischemic cardiomyopathy (IMC) in immediate and remote period. Materials and methods. We included in a single center prospective study 76 patients with IMC, left ventricular ejection fraction ≤35 %, and moderate IMR. Patients with indications to postinfarction aneurism repair were not included. For randomization we used the method of envelopes. Thirty-eight patients were randomized in the group where coronary artery bypass grafting (CABG) was combined with of mitral valve repair (MVR), and 38 patients in the control group of isolated CABG. Mean age of patients was 57±8 (from 30 to 75 лет) years. For IMR correction we used rigid MEDENG ring. Results. Inhospital mortality was 5.4 % (n=2) after isolated CABG and 10.81 % (n=4) after CABG + MVR. Main cause of death was acute heart failure. One- and 2‑year survival was 84 and 78 %, respectively, after CABG+MVR, and 84 and 71 % after isolated CABG. There was significant difference in three-year survival between groups (hazard ratio [HR] of death 0.457, p=0.04). Five-year survival was 45 and 74 % after isolated CABG and CABG+MVR, respectively (р=0.037). Factors associated with inhospital mortality were pulmonary hypertension (HR 2.177, 95 % confidence interval [CI] 2.299 to 9.831; p=0.043), NYHA class IV chronic heart failure (HR 3.027, 95 % CI 1.605 to 5.707; р=0.001), negative result of stress test echocardiography (HR 0.087, 95 %CI 0.041 to 0.186; р<0.001), atrial fibrillation (HR 4.754, 95 %CI 2.299 to 9.831; р<0.001). Conclusion. Correction of moderate IMR in patients with IMC leads to improvement of parameters of survival in remote period. Five-year survival after isolated CABG was 45 %, while after CABG+MVR – 74 % (р=0.037).


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Sugimoto ◽  
S Boveri ◽  
F Bandera ◽  
M Barletta ◽  
E Alfonzetti ◽  
...  

Abstract Background Right ventricular (RV) contractile function and its coupling with pulmonary circulation (PC) stratify the pattern of clinical phenotypes in heart failure (HF) but its predictive role when measured during exercise is undefined. This study sought to investigate the prognostic impact of RV to PC coupling during exercise in HF patients of any left ventricular ejection fraction (LVEF) categorization. Methods 218 HF patients with reduced (n=120), mid-range (n=55), and preserved (n=43) EF and 51 patients with noncardiac dyspnea underwent exercise with gas exchange analysis combined with echocardiography for assessment of RV-PC coupling assessed by the TAPSE/PASP ratio and left atrial (LA) strain (as a mediator of RV to PC uncoupling). Results TAPSE was negatively correlated with PASP (R=−0.31 and −0.31, P&lt;0.001) both at rest and during exercise. TAPSE/PASP was positively correlated with LA strain (R=0.58 and 0.59, P&lt;0.001) and cardiac output (R=0.28 and 0.58, P&lt;0.001) both at rest and during exercise. The severity of mitral regurgitation was also associated with TAPSE/PASP and LA strain both at rest and during exercise (P for trend &lt;0.001). There was significant difference in peak VO2 and the minute ventilation-carbon dioxide production slope among 3 groups divided by tertile of TAPSE/PASP at exercise. During 5 years follow up, 48 HF patients with reduced (n=34), mid-range (n=8), and preserved (n=6) EF had the composite end point. The age and gender adjusted hazard ratio for the events in HF patients with TAPSE/PASP at exercise &lt;0.33, derived by ROC curve (AUC=0.71, P&lt;0.05), was 2.9 (95% CI: 1.5 to 5.5, figure). Conclusions The impaired RV to PC coupling by TAPSE/PASP ratio during exercise predicts prognosis in patients with HF. The cutoff of 0.33 overlaps with the threshold observed to be predictive at rest in various reports across HF populations. These data further emphasize the usefulness of assessing RV to PC uncoupling in cardiac failure during various clinical conditions. Funding Acknowledgement Type of funding source: None


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
V Galand ◽  
B Ghoshhajra ◽  
J Szymonifka ◽  
S Das ◽  
M Orencole ◽  
...  

Abstract Background Secondary mitral regurgitation (MR) is common in heart failure (HF) patients and results in progressive left ventricular (LV) dilatation, papillary muscle (PM) displacement and mitral valve leaflet tethering. In selected HF patients, cardiac resynchronization therapy (CRT) has been proved to reduce MR by LV reverse remodeling, resynchronization of PM insertion site contraction and reduction in MV tenting area and inter PM distance. However, data regarding the impact of LV wall thickness (WT) on MR improvement are scarce. Methods In this prospective study, a total 54 patients scheduled for CRT, underwent pre procedural CT. Reduced LV WT was defined as WT<6mm and was quantified as a percentage of total LV area. LV was segmented in 17 segments to assess the number of LV segments with reduced WT. End point was 6-month echocardiographic MR improvement by ≥1 class. For this analysis, we focused on patient with mild (class 2) to severe (class 4) MR. Results Among the 54 patients, 38 (70.4%) had mild to severe MR at baseline and a total of 16 (42.1%) experienced MR improvement by ≥1 class at 6 months. there was no difference regarding the co-morbidities, electrocardiogram and echocardiographic parameters between patients with or without MR improvement. However, patients without MR improvement had significant higher NT-pro BNP level at baseline. Interestingly, patients without MR improvement had larger LVWT <6mm area (41.541.5±19.4 vs. 22.4±16.1%, p=0.003) associated with higher number of papillary muscle (PM) inserted in reduced LV WT area. In multivariate analysis, an area ≥25% of LVWT<6mm including at least 1 PM insertion was the only predictor of no MR improvement at 6 months (HR 18.4 (1.25–271.75), p=0.034). Lastly, patients with MR improvement had significant lower rate of basal segments with reduced WT, especially in the lateral location. Of note, patients with MR improvement exhibited fewer rate of postero-lateral WT <6mm segments. Left ventriculat segmentation Conclusion LV WT evaluated using CT is a strong predictor of no MR improvement in HF patients with mild to severe MR and who scheduled for CRT implantation.


Author(s):  
Vasilisa S. Ermolenko ◽  
I. V. Silnova ◽  
E. N. Basargina

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.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Maria DeFonte ◽  
Jonathan Goldstein ◽  
Daniele Massera ◽  
Alexandra Stepanovic ◽  
Alexander Gee ◽  
...  

Introduction: Hypertrophic Cardiomyopathy (HCM) is a relatively common inherited heart disease with variable phenotypic expression. While pathogenic variants in sarcomeric genes are considered responsible for the development of left ventricular (LV) hypertrophy, environmental modulation of phenotype may explain the known heterogeneity. Obesity is common in HCM patients and is associated with increased LV mass in the general population. Hypothesis: We hypothesized that weight loss in obese patients with HCM would be associated with a decrease in LV mass and maximal wall thickness. Methods: Patients with HCM who achieved therapeutic weight loss and underwent cardiac MRI (CMR) or computed tomography (CT) before and afterwards were included. Standard LV measurements including wall thickness in an 18-segment model were performed blindly with respect to name, time and BMI for un-biased comparison. Results: We included 6 patients (2 female, age 55 ± 6.5 years, baseline BMI = 36.7 ± 5.2 kg/m 2 ) who achieved 16.3 ± 10.8 kg weight loss after 35 ± 12 months with diet and exercise (n=4) or bariatric surgery (n=2). After weight loss, we observed a mean proportional decrease in total LV mass of 25 ± 16% (p=0.004), and a 19 ± 7% decrease in indexed LV mass (p=0.007). Furthermore, there was a numerical decrease in mean wall thickness in 14 out of 18 LV segments measured; 9 segments had more than a 10% decrease. The most noticeable changes were at the basal inferolateral wall (25 ± 13% decrease) and basal inferoseptum (19 ± 18%) decrease (Table). Conclusions: In this series of patients with HCM, weight loss favorably affected LV mass and wall thickness. Further research is needed to explore the impact of weight loss on HCM phenotypic expression and symptoms.


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