scholarly journals Arrhythmic risk stratification in patients with clinically-suspected left ventricular arrhythmogenic cardiomyopathy

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
G Peretto ◽  
A Villatore ◽  
C Basso ◽  
P Della Bella ◽  
S Sala

Abstract Background Arrhythmic risk of patients with left ventricular arrhythmogenic cardiomyopathy (LVAC) is unpredictable. Purpose To identify risk facors associated with major ventricular tachyarrhythmias (VTA) in clinically-suspected LVAC patients. Methods We enrolled 127 consecutive patients (69% males, age 46±13 y, LVEF 54±7%) with clinically-suspected LVAC. All patients presented with either major (VT, VF) or minor VTA (NSVT, frequent VEB), and underwent extensive diagnostic workup to rule-out alternative diagnoses. Medical treatment and ICD implant were clinically-driven. Prospective follow-up was obtained via sequential 24h-Holter ECG (2–4/y) with or without continuous arrhythmia monitoring (ICD or implantable loop recorders, ILR). The primary endpoint was occurrence of major VTA (VT/VF/ICD therapy) by 24-month follow-up. Results At presentation, 56 (44%) and 71 patients (56%) had, respectively, major and minor VTA. Variants in desmosomal genes were identified in 7 of the 9 patients with clinically-indicated genetic test. Delayed gadolinium enhancement (DGE, average 23±12% of the LV mass) had anteroseptal distribution in 43 cases (34%). Monitoring strategy included ICD (n=64), ILR (n=33), or sequential Holer ECGs (n=30). By 24-month follow-up, major VTA occurred in 32 patients (25%). At univariable anlysis, major VTA onset (HR 16.8, 95% CI 5.4–52.2, p<0.001) and anteroseptal DGE (HR 3.0, 95% CI 1.3–6.9, p=0.010) were significantly associated with major VTA by 24-month follow-up. Among patients presenting with minor VTA, the only factor significantly associated with the primary endpoint was anteroseptal DGE (3/4 vs. 14/67, p=0.004). Conclusion Our preliminary experience suggests that, in patients with clinically-suspected LVAC, major VTA onset and anteroseptal DGE are relevant risk factors for major arrhythmic events by 24-month follow-up. FUNDunding Acknowledgement Type of funding sources: None.

Author(s):  
Vidhu Anand ◽  
Garvan C Kane ◽  
Christopher G Scott ◽  
Sorin V Pislaru ◽  
Rosalyn O Adigun ◽  
...  

Abstract Aims  Cardiac power is a measure of cardiac performance that incorporates both pressure and flow components. Prior studies have shown that cardiac power predicts outcomes in patients with reduced left ventricular (LV) ejection fraction (EF). We sought to evaluate the prognostic significance of peak exercise cardiac power and power reserve in patients with normal EF. Methods and results  We performed a retrospective analysis in 24 885 patients (age 59 ± 13 years, 45% females) with EF ≥50% and no significant valve disease or right ventricular dysfunction, undergoing exercise stress echocardiography between 2004 and 2018. Cardiac power and power reserve (developed power with stress) were normalized to LV mass and expressed in W/100 g of LV myocardium. Endpoints at follow-up were all-cause mortality and diagnosis of heart failure (HF). Patients in the higher quartiles of power/mass (rest, peak stress, and power reserve) were younger and had higher peak blood pressure and heart rate, lower LV mass, and lower prevalence of comorbidities. During follow-up [median 3.9 (0.6–8.3) years], 929 patients died. After adjusting for age, sex, metabolic equivalents (METs) achieved, ischaemia/infarction on stress test results, medication, and comorbidities, peak stress power/mass was independently associated with mortality [adjusted hazard ratio (HR), highest vs. lowest quartile, 0.5, 95% confidence interval (CI) 0.4–0.6, P < 0.001] and HF at follow-up [adjusted HR, highest vs. lowest quartile, 0.4, 95% CI (0.3, 0.5), P < 0.001]. Power reserve showed similar results. Conclusion  The assessment of cardiac power during exercise stress echocardiography in patients with normal EF provides valuable prognostic information, in addition to stress test findings on inducible myocardial ischaemia and exercise capacity.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
H Maqsood ◽  
H.A Shakeel ◽  
H.F Shoukat ◽  
M.D Khan ◽  
S.A.Y Shah ◽  
...  

Abstract Introduction Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular (LV) hypertrophy in the absence of pressure overload. Manifestations of the disease include heart failure associated with diastolic dysfunction and atrial and ventricular tachyarrhythmias. Pathological features of HCM include myocyte hypertrophy, interstitial fibrosis, and myocyte disarray and are mediated by angiotensin II. Purpose This study aimed to evaluate the effects of candesartan on left ventricular (LV) hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM). Methods In double-blind fashion, 30 patients (6 women, 24 men; age: 55±11 years) with HCM were randomly assigned to receive placebo (n=13) or candesartan 50 mg twice a day (n=17) for 1 year. To measure LV mass and extent of fibrosis, cardiac magnetic resonance imaging was performed at baseline and 1 year as assessed by late gadolinium enhancement. Results There was a trend toward a significant difference in the percent change in LV mass (median: +5% with placebo vs. −5% with candesartan; p=0.06). There was a significant difference in the percent change in the extent of late gadolinium enhancement, with the placebo group experiencing a larger increase (+30±27% with placebo vs. −22±44% with candesartan; p=0.03). Conclusion Our study concludes reduction of the progression of myocardial hypertrophy and fibrosis with candesartan in patients with hypertrophic cardiomyopathy. Our study population was limited so we warrant larger trials to confirm a place for angiotensin receptor blockers in the management of patients with hypertrophic cardiomyopathy. Figure 1 Funding Acknowledgement Type of funding source: Other. Main funding source(s): Self funding


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pankaj Garg ◽  
Hosamadin Assadi ◽  
Rachel Jones ◽  
Wei Bin Chan ◽  
Peter Metherall ◽  
...  

AbstractCardiac magnetic resonance (CMR) is emerging as an important tool in the assessment of heart failure with preserved ejection fraction (HFpEF). This study sought to investigate the prognostic value of multiparametric CMR, including left and right heart volumetric assessment, native T1-mapping and LGE in HFpEF. In this retrospective study, we identified patients with HFpEF who have undergone CMR. CMR protocol included: cines, native T1-mapping and late gadolinium enhancement (LGE). The mean follow-up period was 3.2 ± 2.4 years. We identified 86 patients with HFpEF who had CMR. Of the 86 patients (85% hypertensive; 61% males; 14% cardiac amyloidosis), 27 (31%) patients died during the follow up period. From all the CMR metrics, LV mass (area under curve [AUC] 0.66, SE 0.07, 95% CI 0.54–0.76, p = 0.02), LGE fibrosis (AUC 0.59, SE 0.15, 95% CI 0.41–0.75, p = 0.03) and native T1-values (AUC 0.76, SE 0.09, 95% CI 0.58–0.88, p < 0.01) were the strongest predictors of all-cause mortality. The optimum thresholds for these were: LV mass > 133.24 g (hazard ratio [HR] 1.58, 95% CI 1.1–2.2, p < 0.01); LGE-fibrosis > 34.86% (HR 1.77, 95% CI 1.1–2.8, p = 0.01) and native T1 > 1056.42 ms (HR 2.36, 95% CI 0.9–6.4, p = 0.07). In multivariate cox regression, CMR score model comprising these three variables independently predicted mortality in HFpEF when compared to NTproBNP (HR 4 vs HR 1.65). In non-amyloid HFpEF cases, only native T1 > 1056.42 ms demonstrated higher mortality (AUC 0.833, p < 0.01). In patients with HFpEF, multiparametric CMR aids prognostication. Our results show that left ventricular fibrosis and hypertrophy quantified by CMR are associated with all-cause mortality in patients with HFpEF.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E Galli ◽  
OA Smiseth ◽  
JM Aalen ◽  
CK Larsen ◽  
E Sade ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Objective The best modality to assess diastolic function in CRT-candidates is an object of debate and the relationship between diastolic function, CRT-response and survival are not clearly understood. Purpose of the study: to assess diastolic patterns in patients undergoing CRT according to the 2016 recommendations of the American Society of Echocardiography/European Association of Cardiovascular Imaging and to evaluate the prognostic value of diastolic dysfunction (DD) in CRT candidates. Methods 193 patients (age: 67 ± 11 years, QRS width: 167 ± 21 ms) were included in this multicentre prospective study. Patients were stratified according to DD grades (grade I to III). CRT-response was defined as a reduction of left ventricular (LV) end-systolic volume &gt;15% at 6-month follow-up (FU). The primary endpoint was defined as a composite of heart transplantation, LV assisted device implantation or all-cause death during FU. Results During FU, 132 (68%) patients were CRT-responders. CRT delivery was associated with diastolic function degradation in non-responders. Grade I DD was able to predict CRT-response with a sensitivity, specificity and accuracy of 70%, 65%, and 63%, respectively. After a median period of 35 months, the primary endpoint occurred in 29 (15%) patients. Grade I DD was associated with a better outcome [HR 0.26 95% CI: (0.10-0.66)], independently from ischemic cardiomyopathy, LV dyssynchrony and CRT-response (Table 1). Non-responders with grade II or grade III DD had the worse prognosis (HR 4.36, 95%CI: 2.10-9.06) Figure 1. Conclusions Grade I DD is associated with LV remodelling after CRT and is an independent predictor of prognosis in CRT candidates. Abstract Figure.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
O Itzhaki Ben Zadok ◽  
A Eisen ◽  
Y Shapira ◽  
D Monakier ◽  
Z Iakobishvili ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of already advanced cardiomyopathy. Aims to describe early echocardiographic features in patients subsequently diagnosed with CA and to delineate disease progression. Methods Pre-amyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic amyloid subtype (immunoglobulin light-chain (AL) or amyloid transthyretin (ATTR)). Abnormalities were defined based on published guidelines. Results Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 (IQR 1.0, 1.5)cm) ≥3 years before the diagnosis of CA and relative wall thickness (RWT) was increased (0.47 (IQR 0.41, 0.50)) ≥7 years pre-diagnosis. Between 1 to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction (LVEF)≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased RWT (0.74 (IQR 0.62, 0.92) vs. 0.62 (IQR 0.54, 0.76), p = 0.004) and LV mass index (144 (IQR 129, 191) vs. 115 (IQR 105, 146)g/m2,p = 0.020) and reduced LVEF (50 (IQR 44, 58) vs. (60 (IQR 53, 60)%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. Conclusions Increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography Abstract Figure. Schematic proposed timeline of CA


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Daniel H Katz ◽  
Usman A Tahir ◽  
Debby Ngo ◽  
Mark Benson ◽  
Yan Gao ◽  
...  

Background: Increased left ventricular (LV) mass is associated with future adverse cardiovascular events including heart failure (HF). Both increased LV mass and HF disproportionately affect black individuals. To understand the mechanisms that drive disease, particularly in black individuals, we undertook a proteomic screen in a black cohort and compared it to a white cohort. Methods: We measured 1305 plasma proteins using an aptamer-based proteomic platform (SOMAscan™) in 1772 black participants in the Jackson Heart Study (JHS) with available baseline LV mass as assessed by 2D echocardiography, as well as 1600 free of HF with follow-up assessment of incident cases. Mean follow-up time was 11 years; 152 cases of incident HF hospitalization were identified. Models were adjusted for age, sex, body mass index, estimated glomerular filtration rate (as calculated by CKD-EPI equation), systolic blood pressure, hypertension treatment, presence of diabetes, total/HDL cholesterol, prevalent coronary disease, and current smoking status. Incident HF models were also adjusted for incident coronary heart disease. We then compared protein associations in JHS to those observed in whites from the Framingham Heart Study (FHS) to examine significant differences. Results: In JHS, there were 112 proteins associated with LV mass and 10 proteins associated with incident HF hospitalization with FDR <5%. Several proteins showed expected associations with both LV mass and HF, including N-terminal pro-BNP (β = 0.04 [0.02, 0.05], p = 1.0 x 10 -8 , HR = 1.46 [1.20, 1.79], p = 0.0002). The strongest association with LV mass was more novel: leukotriene A4 hydrolase (LKHA4) (β = 0.05 [0.04, 0.06], p = 2.6 x 10 -15 ). Conversely, Fractalkine/CX3CL1 showed a novel association with incident HF (HR = 1.32 [1.14, 1.54], p = 0.0003). While proteins like Cystatin C and N-terminal pro-BNP showed consistent effects in FHS, LKHA4 and Fractalkine were significantly different. Conclusions: We identify several novel biological pathways specific to black individuals hypothesized to contribute to the pathophysiologic cascade of LV hypertrophy and incident HF including LKHA4 and Fractalkine. Further studies are needed to validate these results and elucidate the detailed underlying mechanisms.


2001 ◽  
Vol 12 (12) ◽  
pp. 2759-2767 ◽  
Author(s):  
Gérard M. London ◽  
Bruno Pannier ◽  
Alain P. Guerin ◽  
Jacques Blacher ◽  
Sylvain J. Marchais ◽  
...  

ABSTRACT. Left ventricular (LV) hypertrophy (LVH) is a risk factor for mortality in patients with end-stage renal disease (ESRD). Whether the attenuation of LVH has a positive effect on survival of patients with ESRD has not been documented. The aim of this study was to determine the effect of parallel treatment of hypertension and anemia on LV mass (LVM) and to determine the effect of LVM changes on survival. A cohort of 153 patients receiving hemodialysis was studied. The duration of follow-up was 54 ± 37 mo. All patients had echocardiographic determination of LV dimensions and LVM at baseline and regular intervals until the end of the follow-up period. During the study, BP decreased from (mean ± SD) 169.4 ± 29.7/90.2 ± 15.6 to 146.7 ± 29/78 ± 14.1 mmHg (P< 0.001), and hemoglobin increased from 8.65 ± 1.65 to 10.5 ± 1.45 g/dl (P< 0.001). The LV end-diastolic diameter and mean wall thickness decreased from 56.6 ± 6.5 to 54.8 ± 6.5 mm (P< 0.001), and from 10.4 ± 1.6 to 10.2 ± 1.6 mm (P< 0.05), respectively. The LVM decreased from 290 ± 80 to 264 ± 86 g (P< 0.01). Fifty-eight deaths occurred, 38 attributed to cardiovascular (CV) disease and 20 attributed to non-CV causes. According to Cox analyses after adjustment for age, gender, diabetes, history of CV disease, and all nonspecific CV risk factors, LVM regression positively affected the survival. The hazard risk ratio associated with a 10% LVM decrease was 0.78 (95% confidence interval, 0.63 to 0.92) for all-causes mortality and 0.72 (95% confidence interval, 0.51 to 0.90) for mortality due to CV disease. These results show that a partial LVH regression in patients with ESRD had a favorable and independent effect on patients’ all-cause and CV survival.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
AM Caggegi ◽  
P Capranzano ◽  
S Scandura ◽  
S Mangiafico ◽  
G Castania ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background – Although percutaneous mitral valve repair is an attractive alternative treatment option for patients with severe mitral regurgitation (MR) at high surgical risk, residual MR is commonly observed after the procedure and little is known about its impact on outcomes after MitraClip therapy, expecially in patients with severe left ventricular (LV) impairment. Purpose – The aim of this prospective, observational study was to evaluate the impact of residual MR (MR ≤1+ vs. MR &gt;1+) on long-term outcomes of mitral valve repair with the MitraClip System in high surgical risk patients presenting with moderate-to-severe or severe MR and with severe reduction of LV ejection fraction (EF). Methods – Patients enrolled in the prospective Getting Reduction of Mitral Insufficiency by Percutaneous Clip Implantation (GRASP) with functional MR and EF ≤30% who were eligible at almost five-year follow-up were included in the present analysis.  The primary endpoint was death at 5-year follow-up.  Also echocardiographic parameters at baseline and 5-year follow-up and rehospitalization rates were assessed. Results – A total of 139 patients were included: 92 (66.2%) with post-procedural residual MR ≤1+ and 47 (33.8%) with residual MR &gt; 1+ (41 patients with residual MR 2+, 5 with residual MR 3+, 1 with residual MR 4+).  Comparable clinical and echocardiographic baseline characteristics were observed between the two groups except for NYHA functional class IV and implanted pace-maker (more frequent in patients with residual MR &gt;1+) and previous myocardial infarction (more frequent  in patients with residual MR ≤1+). At 5-year follow-up, no significant differences were reported in the primary endpoint (49.6% in patients with residual MR ≤ 1+ vs. 65.3% in patients with residual MR &gt; 1+, p 0.203) and in cardiac death (37.8% in patients with residual MR ≤ 1+ vs. 42.6% in patients with residual MR &gt; 1+, p 0.921). Cox regression analysis identified residual MR &gt; 1+ as an independent predictor of re-hospitalization (HR 0.51, 95% CI 0.28-0.92, p =0.026). At 5-year follow-up,  a significant reduction in left ventricular end-systolic volume was  observed in patients with residual MR ≤ 1+. Conclusions – At 5-year follow no significant differences in survival emerged in patients with severe  LV dysfunction undergoing MitraClip therapy regardless residual MR. Nevertheless residual MR &gt; 1+ emerged as an indipendent predictor of re-hospitalization.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Alfonso Valle ◽  
Mercedes Nadal ◽  
Jordi Estornell ◽  
Nieves Martinez ◽  
Miguel Corbi ◽  
...  

The identification of prognostic markers in patients with heart failure of both ischemic and non ischemic etiology is an increasing need in the era of devices therapy. Risk stratification for sudden cardiac death (SCD) remains problematic with reliance on left ventricular function which predicts total mortality rather than arrhythmic events (AE). Recently cardiac magnetic resonance was employed to predict susceptibility for malignant arrhythmias. This study sought to determine the utility of late gadolinium enhancement (LGE) to predict AE. Three hundred consecutive patients with symptomatic heart failure and systolic dysfunction of both ischemic and non ischemic cause undergoing CMR, were classified into two groups attending to the presence (n 160) or absence of LGE (n 140), and were followed prospectively during 842 days. The primary endpoint was the combined of SCD or Ventricular tachycardia (VT). 23 patients had AE (8 SCD/15 VT) during the follow-up, 19 of them presenting LGE (83%). The presence of LGE was associated to a significantly higher AE rate (11.8.% vs 2.8% p< 0.001)(figure ). Compared to patients without LGE, midwall fibrosis and an ischemic pattern of LGE predicted AE. (3% vs 5% vs 14%, p= 0.001) LGE is a new non-invasive predictor of AE in patients with heart failure and systolic dysfunction. This suggest a potential role for risk stratification and better selection of patients who needs device therapy


Circulation ◽  
2016 ◽  
Vol 133 (suppl_1) ◽  
Author(s):  
Bryan R Wilner ◽  
Sonia Garg ◽  
Colby R Ayers ◽  
Satyam Sarma ◽  
Anand Rohatgi ◽  
...  

Introduction: Obesity is linked to an adverse cardiac structural phenotype in cross-sectional studies. However, the effects of longitudinal changes in generalized and central adiposity on left ventricular (LV) remodeling are unknown. Methods: Participants without baseline cardiovascular disease or LV dysfunction in the Dallas Heart Study underwent assessment of body composition and cardiac structure by MRI at baseline and then 7 years later. Associations between change in weight and waist circumference with alterations in structure and function were assessed using multivariable linear regression. Results: The study cohort (n=1262) had a mean age of 44 years and was 43% (545 of 1262) male, 44% (556 of 1262) African-American, and 36% (460 of 1262) obese at baseline. At 7 years follow-up, 7% (85 of 1262) had >10% weight loss, 8% (108 of 1262) had 5-10% weight loss, 44% (551 of 1262) had <5% weight change, 20% (248 of 1262) had 5-10% weight gain, and 21% (270 of 1262) had >10% weight gain. Those who gained >10% weight were younger, had lower BMI and LV mass at baseline, and had greater increases in blood pressure, glucose, triglycerides, LDL cholesterol, and hs-CRP over follow-up. In multivariable models adjusted for age, sex, race, and baseline and interim development of comorbidities, 1-standard deviation increases in body weight and waist circumference over follow-up were significantly associated with higher LV mass, LV wall thickness, and concentricity; but minimally or not significantly associated with LV end-diastolic volume or ejection fraction (EF) (Table). Conclusion: Increases in generalized and central adiposity are characterized primarily by concentric remodeling, with a more modest impact on LV volume and EF. These results support the notion that the development of specific obesity patterns may impact cardiac remodeling with potential implications for the development of cardiac hypertrophy and heart failure.


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