The clinical atlas of cardiomyopathies: data from the prospective DZHK TORCH study

Abstract Introduction Cardiomyopathies (CMPs) are leading causes of heart failure (HF) and sudden cardiac death (SCD). Comparative data of the multiple cardiomyopathy forms are largely missing. The TranslatiOnal Registry for CardiomyopatHies (TORCH) is the largest prospective multicentre CMP registry world-wide. Enrolled patients are comprehensively phenotyped by clinical examinations, state-of-the-art imaging, and molecular investigations. In this study, we present the baseline and 1-year follow-up data. Methods TORCH is a national, prospective, multicentre registry within the German Centre for Cardiovascular Research (DZHK) and includes 2300 patients with non-ischemic (primary and secondary) CMP from 20 centres. The minimum follow up was one year. The DZHK-wide harmonization of datasets and SOPs ensure a high level of data quality and comparability across different CMP forms. Results Dilated cardiomyopathy (DCM) has the highest prevalence with 64% of all enrolled patients, followed by hypertrophic cardiomyopathy (HCM) with 16%. At baseline, patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) were treated more often with ICD implantation and showed high rates of adequate ICD therapies (65.8%, p<0.05 and 47.8%, p<0.05, respectively). The prevalence of stroke or transient ischemic attack (TIA) was in multivariate analysis significantly higher (p<0.05) in left ventricular non-compaction cardiomyopathy (LVNC, 14.9%), while atrial fibrillation was lower than in other cardiomyopathy forms. Patients with amyloidosis had the worst outcome (HR: 6; 95% CI: 2.5–14.5, P<0.05) with annual mortality of >15% and 12% receiving heart transplantation. In DCM, reverse remodelling with improvement of functional parameters and biomarkers was more often observed in idiopathic and inflammatory cases compared to familial ones. HCM patients had the most favourable outcome. Conclusion and outlook TORCH is the largest prospective study focusing on CMPs. We provided for the first time prospectively the clinical data of patients with diverse cardiomyopathies with outcome. Furthermore, comparing the different CMP forms on the clinical and molecular level will be an important step to enable translational research projects. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): German Centre for Cardiovascular Research (DZHK)

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Performance of different myocardial tissue tracking algorithms and acquisition-based strain imaging to characterise myocardial pathology

European Heart Journal ◽

10.1093/ehjci/ehaa946.0237 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

S.J Backhaus ◽

G Metschies ◽

V Zieschang ◽

J Erley ◽

S.M Zamani ◽

...

Keyword(s):

Intraclass Correlation ◽

Correlation Coefficients ◽

Left Ventricular ◽

Funding Source ◽

Post Processing ◽

Cardiovascular Research ◽

National Budget ◽

Myocardial Deformation Imaging ◽

Cmr Tagging ◽

Public Grant

Abstract Background Myocardial deformation imaging is superior in risk-stratification compared to volumetric approaches. Myocardial Feature-Tracking (FT) allows easy post-processing of routinely acquired cine images. Since there is no clear recommendation regarding FT post-processing we sought to compare different FT-strains with reference standard techniques including tagging and strain encoded (SENC) magnetic resonance imaging. Methods CMR-FT software from 4 different vendors (TomTec, Medis, Circle, Neosoft), CMR tagging (Segment) and fastSENC (MyoStrain) were used to determine left ventricular (LV) global longitudinal and circumferential strains (GLS and GCS) in 12 healthy volunteers and 12 heart failure patients. Variability and agreements were assessed using intraclass correlation coefficients, coefficients of variation and Bland Altman plots. Results Compared to tagging, FT-based strain was software independently significantly higher except for GCS using Medis (p=0.178). Compared to fSENC, mean-differences of GLS were smaller within a range of ±1.5%. For GCS this only applied to CVI and Medis (<1.5%) but not TomTec (>7%) or Neosoft (>4%). Absolute agreements comparing FT to tagging were best for CVI (GLS ICC0.70) and Medis (GCS ICC0.85). Compared to fSENC agreement of GLS was generally excellent (ICC>0.77), but only CVI and Medis revealed excellent agreement for GCS (ICC0.88 and 0.85). Consistency and correlation of GLS were software independently high compared with tagging and fSENC (ICC>0.86, r>0.76) while being lower for GCS (ICC>0.68, r>0.72). Conclusion Although agreement differs between deformation assessment approaches, consistency and correlation are high irrespective of the method chosen, thus indicating reliable strain assessment. Further standardisation and introduction of uniform references is warranted for clinical routine implementation. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): DZHK - German Centre for Cardiovascular Research

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Cardiomyopathy phenotyping and risk-stratification in danon disease

European Heart Journal ◽

10.1093/ehjci/ehaa946.0220 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

M Rigolli ◽

A Khan ◽

M Brambatti ◽

F Contijoch ◽

E Adler

Keyword(s):

Heart Failure ◽

Risk Stratification ◽

Heart Transplant ◽

Left Ventricular ◽

Clinical Severity ◽

Funding Source ◽

Icd Implantation ◽

Danon Disease ◽

Imaging Characteristics

Abstract Background Danon Disease (DD) is a rare, X-linked vacuolar myopathy due to mutations in Lysosomal Associated Membrane Protein 2 (LAMP-2). Though it is strongly associated with severe cardiomyopathy, heart failure and sudden death, there is no data on typical cardiac magnetic resonance (CMR) imaging characteristics in DD and their association with clinical severity and outcome. Purpose To phenotype and risk-stratify DD patients. Methods CMR scans of confirmed DD patients recruited in a global registry were prospectively analyzed for biventricular volumes, ejection fraction (EF), left ventricular (LV) strain, mass (LVM) and late gadolinium enhancement (LGE) in a core-lab blinded fashion. A major adverse cardiac event (MACE) was a composite of death, heart transplant and implantable cardioverter defibrillator (ICD) for secondary prevention. Results 12 DD patients (5 males [42%], median age 13 yrs [interquartile range (IQR) 5]) were included. LV hypertrophy (LVH) was present in 10/12 (83%), associated with LV dilation in 2 females. LVH was typically asymmetric in females (5/7) and concentric in males (3/5); right ventricular (RV) hypertrophy frequent in females (4/7). LV strain was reduced (global circumferential strain [GCS] −12±4%) and LGE common (73%), often extensive and always sparing the basal-mid septum. LGE was strongly associated with heart failure (BNP r=0.9, p=0.0021). Patients with MACE (6 [50%], median follow-up 2.9 yrs) had elevated LVM (241±63 g, p=0.032), impaired LV strain (GCS: −9.8±3.9, p=0.02) and higher LGE mass (median 56 g [IQR 35], p=0.021) compared to those without events during follow-up (LVM 155±56 g, GCS −14.9±1.6, LGE mass 0 g [IQR 8]). CMR characteristics were predictors of MACE (LV strain: hazard ratio [HR] 1.4, p=0.021; LGE mass: HR 1.1, p=0.03). Conclusions LGE sparing the basal-mid septum was pathognomonic in DD. LVH with reduced LV strain was the most common DD phenotype but the spectrum included LV dilation and RV hypertrophy in females. CMR characteristics (LV strain and LGE) were associated with heart failure and predicted worse outcome (heart transplant and fatal arrhythmias). CMR phenotyping and risk-stratification of this severe and underrecognized cardiomyopathy may aid diagnosis and clinical management in DD patients who need selection for early heart transplant, ICD implantation and targeted gene therapy. Danon Disease Phenotypes and Outcome Funding Acknowledgement Type of funding source: Other. Main funding source(s): National Institute of Health and Rocket Pharmaceuticals

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Transcatheter intervention of coarctation of the aorta (CoA): a multinational population-based registry – effect on hypertension

European Heart Journal ◽

10.1093/ehjci/ehaa946.2197 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

P Eriksson ◽

J Pihkala ◽

A.S Jensen ◽

G Dohlen ◽

P Liuba ◽

...

Keyword(s):

Pharmacological Treatment ◽

Coarctation Of The Aorta ◽

Life Time ◽

Population Based ◽

Funding Source ◽

Transcatheter Intervention ◽

National Budget ◽

Need For Treatment ◽

Public Grant

Abstract Background CoA is associated with hypertension caused by reduced wind kessel function in the aortic arch, general hypoplasia of the arch and/or essential hypertension. In patients with a native or recurrent/rest CoA, a gradient >20 mmHg by non-invasive meassurement if associated with hypertension is an ESCguideline indication for intervention. We studied the persistence and presence of hypertension after transcatheter intervention of a CoA Methods All consecutive patients undergoing catheter interventions for CoA from 1st of January 2000 to 31st of December 2016 were identified by each of the particpating nine centers. The nine centers perform all catheter interventions for CoA for a complete population coverage of 25 millions inhabitants. A common protocoll was filled out from medical records. Hypertension was defined as a pre-intervention blood pressure above 140/80 or pharmacological treatment of hypertension. Exclusion criteria were weight less than 20 kg at the time of intervention or Norwood surgery Results 590 interventions were performed on 520 patients: two interventions n=76, three: n=11, four n=2 and one patient underwent five interventions. Before intervention, 437 (74%) of the patients were hypertensive and 285 were on pharmacologocal treatment; 134 (48%) were treated with one drug, 79 patients (28%) with two drugs, 41 patients (15%) with three drugs and 14 (5%) with four drugs. After the intervention during follow up hypertension was present in 294 patients (50%, p<0.001 vs pre) of whom 270 (46%) were on pharmacological treatment; with one drug, n=128 (48%), two drugs n=93 (34%), three drugs n=34 (13%) or 4 drugs n=7 (3%). Conclusions Catheter intervention of CoA reduced the presence of hypertension significantly from 74% down to 50% but many patients will remain hypertensive and in need for treatment. Life time follow up also after transcatheter CoA intervention seems warranted. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): ALF-LUA, Heart and Lung Foundation

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The relationship between glycosylation of proBNP at threonine 71, BNP, BNP1–32 and obesity in patients with heart failure

European Heart Journal ◽

10.1093/ehjci/ehaa946.1001 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

L.K Lewis ◽

S.D Raudsepp ◽

T.G Yandle ◽

C.J Pemberton ◽

R.N Doughty ◽

...

Keyword(s):

Heart Failure ◽

New Zealand ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Funding Source ◽

Obese Patients ◽

Ventricular Ejection Fraction ◽

National Budget ◽

National Heart Foundation ◽

Public Grant

Abstract Background Heart failure (HF) is a leading cause of morbidity and mortality worldwide. Measurement of BNP and NTproBNP are used in HF for diagnosis and prognosis but levels of these peptides are inappropriately low in obesity, a condition which is associated with increased HF. Cleavage of proBNP to produce BNP and NT-proBNP requires proBNP to be unglycosylated at threonine 71 (T71). Gycosylation at T71 is affected by obesity, resulting in lower plasma NT-proBNP concentrations in patients with higher BMI. However the relationships between BMI, proBNP glycosylation and BNP (particularly the bioactive cardio-protective peptide BNP1-32) have not previously been described. Methods Validated in-house assays for BNP, BNP1-32, proBNP, proBNP unglycosylated at T71 (NG-T71) and the commercial Roche assay for NT-proBNP were applied to plasma samples obtained from patients with HF (n=321, PEOPLE study: Prospective Evaluation of Outcome in Patients with Left Ventricular Ejection Fraction). Results Median (IQR) concentrations of BNP, BNP1-32, proBNP, NG-T71 and NTproBNP were 10.7 (5–21), 5 (2–9), 27.8 (9–62), 6.2 (3–22) and 217 (104–425) pmol/L respectively. BMI was inversely related to NG-T71, NT-proBNP, BNP and BNP1-32 (r=−0.19, −0.40, −0.36 and −0.34 respectively, all p<0.01) but not proBNP (r=0.11, ns). ProBNP levels in patients with BMI above or below 30 kg/m2 were similar (29.8 (11.2–56.6) and 22.5 (3.9–65) pmol/L, p=0.51), whereas NG-T71, NT-proBNP, BNP and BNP1-32 levels were increased (p<0.001) in patients with BMI <30 (11.6 (3–25.6), 263 (153–486), 13.8 (6.5–25.5) and 6.3 (2.8–10.4)) compared to BMI >30 (3 (1–16), 127 (63–274), 7.8 (3–14) and 3.6 (1.1–7) respectively. The BMI >30 group had increased ProBNP:NT-proBNP, ProBNP:BNP and ProBNP:BNP1-32 ratios (all p<0.001) and proBNP:NG-T71 (p=0.037), whereas ratios of NG-T71 to BNP, BNP1-32 or NT-proBNP were not related to BMI. Patients with diabetes (n=90) also had lower BNP, BNP1-32 (both p<0.01), NG-T71 and NT-proBNP concentrations (both p<0.05), but not proBNP (p=0.46), and a trend towards a higher proBNP:BNP1-32 ratio (p=0.06). Discussion and conclusion The negative association between BMI and plasma NT-proBNP and BNP is not well understood. We recently reported that obese patients with HF have reduced circulating levels of proBNP unglycosylated at T71. In this expanded sample we show that whilst proBNP remains unaffected by BMI, both immunoreactive BNP and more specifically bioactive BNP1–32 levels, and NT-proBNP, are decreased with obesity in conjunction with increased glycosylation at T71. Increased glycosylation at proBNP-T71 reduces the amount of proBNP cleaved to form NT-proBNP and BNP resulting in decreased production and lowered circulating concentrations of these clinically used marker peptides. Our results provide a robust mechanism to explain the reduction in NT-proBNP and BNP levels observed in obese patients and confirm this is associated with reduced bioactive BNP1–32. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Heart Foundation of New Zealand, nHealth Research Council of New Zealand

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Association of left ventricular end-diastolic diameter with incidence of thromboembolism in patients with non-valvular atrial fibrillation: the Fushimi AF Registry

European Heart Journal ◽

10.1093/eurheartj/ehab724.0466 ◽

2021 ◽

Vol 42 (Supplement_1) ◽

Author(s):

Y Tezuka ◽

K Doi ◽

Y Hamatani ◽

Y An ◽

M Ishii ◽

...

Keyword(s):

Atrial Fibrillation ◽

Thrombus Formation ◽

Oral Anticoagulants ◽

Japanese Patients ◽

Left Ventricular ◽

Rank Test ◽

National Budget ◽

Public Grant ◽

The Impact

Abstract Background Atrial fibrillation (AF) is a common arrhythmia and a risk factor for thromboembolism including ischemic stroke (IS) and systemic embolism (SE). Thrombus formation mainly occurs in the left atrium (LA), but we reported that relative wall thickness (RWT) of left ventricle (LV) was independently associated with IS/SE among patients with non-valvular AF. Little is known about the impact of LV size on thromboembolism in patients with AF. We investigated the relationship between left ventricular end-diastolic diameter (LVDd) and incidence of IS /SE, using data from the Fushimi AF Registry. Methods The Fushimi AF Registry, a community-based prospective survey, was designed to enroll all of the AF patients in the community. Follow-up data were available for 4,472 patients as of November 2020, and the median follow-up period was 1,820 days. Of them, we excluded 226 patients with mitral stenosis or prior cardiac valve surgery. Among 4,246 non-valvular AF patients, follow-up data including LVDd at the baseline from echocardiography were available for 3,311 patients. We divided these patients into three groups according to LVDd tertile (T1:<44.0 mm; n=1,091, T2: 44.0–48.5; n=1,112, T3: 48.5 or above; n=1,108), and compared the clinical characteristics and outcomes. Results Percentage of female (T1 vs. T2 vs T3; 56.5 vs. 37.5 vs. 34.1%; p<0.01), age (76.2±9.9 vs. 72.8±10.7 vs. 71.9±11.1 years; p<0.01), height (156.3±9.9 vs. 160.4±9.4 vs. 163.7±9.5 cm; p<0.01), BMI (21.8±3.9 vs. 23.3±3.6 vs. 24.2±4.4 kg/m2; p<0.01), prevalence of hypertension (58.8 vs. 64.1 vs. 67.7%; p<0.01), vascular disease (7.4 vs. 8.8 vs. 15.2%; p<0.01), CHA2DS2-VASc score (3.59±1.68 vs. 3.27±1.70 vs. 3.31±1.73; p<0.01) and the prescription of oral anticoagulants (OACs) were different among groups. LV ejection fraction (65.9±8.7 vs. 65.0±9.1 vs. 57.7±14.4%; p<0.01), LA diameter (40.7±7.9 vs. 42.8±7.3 vs. 45.8±7.6 mm; p<0.01), LVRWT (0.471±0.090 vs. 0.411±0.064 vs. 0.366±0.063; p<0.01) and LV mass index (79.6±21.4 vs. 93.5±20.4 vs. 117.6±30.3 g/m2; p<0.01) were different. Prevalence of prior stroke/SE and prior major bleeding were comparable among groups. In Kaplan-Meier analysis, the incidence of IS/SE was different among the groups during the median follow-up period of 1,826 days (T1 vs. T2 vs T3; 2.1 vs. 1.9 vs. 1.1 per 100 person-years; p<0.01, by log-rank test) (Figure). LVDd (All age, hazard ratio [95% confidential interval]: T1/T3 1.57 [1.09–2.29]; p=0.02, T2/T3 1.55 [1.11–2.17]; p<0.01: less than 75 years, T1/T3 2.27 [1.24–4.22]; p<0.01, T2/T3 1.54 [0.89–2.70]; p=0.12: 75 years or above, T1/T3 1.33 [0.83–2.14]; p=0.08, T2/T3 1.57 [1.04–2.40]; p=0.03) was an independent predictor of the incidence of IS/SE after adjustment by the components of CHA2DS2-VASc score, LA diameter, LVRWT, type of AF and the prescription of OACs (Table). Conclusion LVDd was independently associated with IS/SE among Japanese patients with non-valvular AF. FUNDunding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): the Practical Research Project for Life-Style-related Diseases including Cardiovascular Diseases and Diabetes Mellitus from Japan Agency for Medical Research and Development

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Association between the progression of aortic valve calcification and coronary atherosclerotic plaque volume

European Heart Journal ◽

10.1093/ehjci/ehaa946.0183 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

S.E Lee ◽

M.J Budoff ◽

E Conte ◽

M Hadamitzky ◽

J.A Leipsic ◽

...

Keyword(s):

Aortic Valve ◽

Coronary Atherosclerosis ◽

Funding Source ◽

Linear Regression Models ◽

Aortic Valve Calcification ◽

Plaque Volume ◽

Valve Calcification ◽

National Budget ◽

Public Grant

Abstract Background It is unclear whether the annual progression of aortic valve calcification (AVC) is associated with the progression of coronary atherosclerosis. Purpose We explored the association between AVC and the total and compositional plaque volume (PV) progression. Methods We performed a prospective multinational registry of consecutive patients who underwent serial coronary computed tomography angiography (CTA) at ≥2-year intervals. AVC, and total and compositional PV at baseline and follow-up were quantitatively analyzed. Multivariate linear regression models were constructed. Results Overall, 594 patients (56% male, 61.5±9.7 years old) were included (mean coronary CTA interval, 3.9±1.5 years). At baseline, AVC was 30.9±117.3. Normalized total PV at baseline was 122.3±219.4mm3, encompassing 41.9±116.8mm3 of calcified PV and 80.4±131.5mm3 of non-calcified PV. After adjustment of age, sex, clinical risk factors, and drug use, AVC at baseline was independently associated with total and all compositional PVs (all p<0.001). However, at follow-up, the annual progression of AVC was only associated with the annual progression of calcified PV (β=0.149, p=0.0089), but not with total and non-calcified PVs (all p>0.05) (Table, Figure). Conclusion The overall burden of coronary atherosclerosis is associated with AVC at baseline. However, the progression of AVC is associated only with the progression of calcified PV but not with that of total and non-calcified PV. Representative case Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): The National Research Foundation (NRF) of Korea funded by the Ministry of Science and ICT (MSIT)

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Impact of epicardial adipose tissue increase after ST elevation Myocardial Infarction (STEMI) on IL-13 and left ventricular remodelling

European Heart Journal ◽

10.1093/ehjci/ehaa946.1585 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

V Parisi ◽

S Cabaro ◽

V D'Esposito ◽

L Petraglia ◽

M Conte ◽

...

Keyword(s):

Adipose Tissue ◽

Epicardial Adipose Tissue ◽

Protective Role ◽

Left Ventricular ◽

Funding Source ◽

National Budget ◽

Group 2 ◽

Public Grant ◽

Group 1

Abstract Background The role of epicardial adipose tissue (EAT) in myocardial diseases is well established, and several evidence suggest that EAT may negatively affect left ventricular (LV) remodelling through an imbalanced production and secretion of pro and anti-inflammatory cytokines. Of these, the IL-13 it is known to play a positive activity on cardiac remodelling. Nowadays, the crosstalk between EAT and the myocardium is still poorly understood and the effects of myocardial ischemia on morphological and functional properties of EAT are almost unknown. Purpose In the present study we explored whether an increase of EAT thickness after STEMI might be associated with unfavourable LV remodelling at 3 months (T1). We also evaluated the relationship between changes (Δ) of EAT thickness and systemic levels of Interleukin (IL)-13 which is known to play a favourable activity on LV remodelling after STEMI. Methods We enrolled 66 patients with first STEMI, undergoing primary percutaneous angioplasty. At baseline and at 3 months we performed a complete echocardiogram, including EAT maximal thickness assessment, and determined circulating levels of IL-13. Results At 3 months after STEMI, the population was stratified into two groups according to different EAT remodelling after cardiac event: Group 1, patients with an increased EAT thickness (Δ EAT>1; 30 patients) and Group 2, patients with unchanged or decreased EAT thickness (Δ EAT<1). The two groups did not differ for age, gender and atherosclerotic risk factors. Group 1 had a worse LV remodelling at 3 months with higher LV diastolic and systolic volumes, lower LV ejection fraction (p=0.003; p=0.013; p=0.013 respectively) and worse diastolic function (E/e'; p=0.011). Of interest, EAT thickness increase was paralleled by circulating IL-13 reduction (p=0.022). Conclusion Myocardial injury can result in EAT increase which is associated to worse LV remodelling probably through the loss of the protective role of IL-13. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): This research has been funded by the University of Naples “Federico II” and “Compagnia di San Paolo e l'Istituto Banco di Napoli” within the competitive grant STAR 2018; Valentina Parisi is the principal investigator

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Quantitative 4D Flow CMR analysis of intracardiac blood flow energetics in ischemic cardiomyopathy patients

European Heart Journal ◽

10.1093/ehjci/ehaa946.0245 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Riva ◽

A Camporeale ◽

F Sturla ◽

S Pica ◽

L Tondi ◽

...

Keyword(s):

Myocardial Infarction ◽

Ischemic Cardiomyopathy ◽

Inverse Correlation ◽

Lv Function ◽

Funding Source ◽

4D Flow ◽

Anterior Myocardial Infarction ◽

National Budget ◽

4D Flow Cmr ◽

Public Grant

Abstract Background Ischemic cardiomyopathy (ICM) is often associated with negative LV remodelling after myocardial infarction, sometimes resulting in impaired LV function and dilation (iDCM). 4D Flow CMR has been recently exploited to assess intracardiac hemodynamic changes in presence of LV remodelling. Purpose To quantify 4D Flow intracardiac kinetic energy (KE) and viscous energy loss (EL) and investigate their relation with LV dysfunction and remodelling. Methods Patients with prior anterior myocardial infarction underwent a CMR study with 4D Flow sequences acquisition; they were divided into ICM (n=10) and iDCM (n=10, EDV>208 ml and EF<40%). 10 controls were used for comparison. LV was semi-automatically segmented using short axis CMR stacks and co-registered with 4D Flow. Global KE and EL were computed over the cardiac cycle. NT-proBNP measurements were correlated with average and peak values, during systole and diastole. Results Both LV volume and EF significantly differ (P<0.0001) between iDCM (EDV=294±56 ml, EF=24±8%), ICM (EDV=181±32 ml, EF=34±6%) and controls (EDV=124±29 ml, EF=72±5%). If compared to controls, both ICM and iDCM showed significantly lower KE (P≤0.0008); though lower than controls, EL was higher in iDCM than ICM. Within the iDCM subgroup, diastolic mean KE and peak EL reported good inverse correlation with NT-proBNP (r=−0.75 and r=−0.69, respectively). EL indexed (ELI) to average KE during systole was higher in the entire ischemic group as compared to controls (ELI(ischemic) = 0.17 vs. ELI(controls) = 0.10, P=0.0054). Conclusions 4D Flow analyses effectively mapped post-ischemic LV energetic changes, highlighting the disproportionate intraventricular EL relative to produced KE; preliminary good correlation between LV energetic changes and NT-proBNP will deserve further investigation in order to contribute to early detection of heart failure. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Italian Ministry of Health

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Is alcohol septal ablation in women performed too late?

European Heart Journal ◽

10.1093/ehjci/ehaa946.2077 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Batzner ◽

D Aicha ◽

H Seggewiss

Keyword(s):

Hospital Stay ◽

Pacemaker Implantation ◽

Posterior Wall ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Left Ventricular ◽

Icd Implantation ◽

Alcohol Septal Ablation ◽

Significant Difference ◽

Surgical Myectomy

Abstract Introduction Alcohol septal ablation (PTSMA) was introduced as interventional alternative to surgical myectomy for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM) 25 years ago. As gender differences in diagnosis and treatment of HOCM are still unclear we analyzed baseline characteristics and results of PTSMA in a large single center cohort with respect to gender. Methods and results Between 05/2000 and 06/2017 first PTSMA in our center was performed in 952 patients with symptomatic HOCM. We treated less 388 (40.8%) women and 564 (59.2%) men. All patients underwent clinical follow-up. At the time of the intervention women were older (61.2±14.9 vs. 51.9±13.7 years; p<0.0001) and suffered more often from NYHA grade III/IV dyspnea (80.9% vs. 68.1%; p<0.0001), whereas angina pectoris was comparable in women (62.4%) and men (59.9%). Echocardiographic baseline gradients were comparable in women (rest 65.0±38.1 mmHg and Valsalva 106.2±45.7 mmHg) and men (rest 63.1±38.3 mmHg and Valsalva 103.6±42.8 mmHg). But, women had smaller diameters of the left atrium (44.3±6.9 vs. 47.2±6.5 mm; p<0001), maximal septum thickness (20.4±3.9 vs. 21.4±4.5 mm; p<0.01), and maximal thickness of the left ventricular posterior wall (12.7±2.8 vs. 13.5±2.9 mm; p<0.0001). In women, more septal branches (1.3±0.6 vs. 1.2±0.5; p<0.05) had to be tested to identify the target septal branch. The amount of injected alcohol was comparable (2.0±0, 4 in women vs. 2.1±0.4 ml in men). The maximum CK increase was lower in women (826.0±489.6 vs. 903.4±543.0 U / l; p<0.05). During hospital stay one woman and one man died, each (n.s.). The frequency of total AV blocks in the cathlab showed no significant difference between women (41.5%) and men (38.3%). Furthermore, the rate of permanent pacemaker implantation during hospital stay did not differ (12.1% in women vs. 9.4% in men). Follow-up periods of all patients showed no significant difference between women (5.7±4.9 years) and men (6.2±5.0 years). Overall, 37 (9.5%) women died during this period compared to only 33 (5.9%) men (p<0.05). But, cardiovascular causes of death were not significantly different between women (2.8%) and men (1.6%). Furthermore, the rates of surgical myectomy after failed PTSMA (1.3% in women vs. 2.3% in men), ICD implantation for primary prevention of sudden cardiac death according to current guidelines (4.1% in women vs. 5.9% in men) or pacemaker implantation (3.6% in women vs. 2.0% in men) showed no significant differences. Summary PTSMA in women with HOCM was performed at more advanced age with more pronounced symptoms compared to men. While there were no differences in acute outcomes, overall long-term mortality was higher in women without differences in cardiovascular mortality. Therefore, women may require more intensive diagnostic approaches in order not to miss the correct time for gradient reduction treatment. Funding Acknowledgement Type of funding source: None

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Sustained ventricular tachycardia of left, right or both bundle branch block morphology in patients with Arrhythmogenic Cardiomyopathy

European Heart Journal ◽

10.1093/ehjci/ehaa946.0452 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Milman ◽

M Laredo ◽

R Roudijk ◽

G Peretto ◽

A Andorin ◽

...

Keyword(s):

Ventricular Tachycardia ◽

Right Bundle Branch Block ◽

Left Bundle Branch Block ◽

Clinical Course ◽

Left Ventricular ◽

Funding Source ◽

Arrhythmogenic Cardiomyopathy ◽

Icd Implantation ◽

Bundle Branch Block ◽

Group 3

Abstract Aims In arrhythmogenic cardiomyopathy (ACM) sustained monomorphic ventricular tachycardia (VT) typically displays left bundle branch block (LBBB) morphology. Sustained VT with right bundle branch block (RBBB) morphology is very rare despite the frequent left ventricular involvement. The present study sought to assess the prevalence of spontaneous sustained LBBB-VT, RBBB-VT or both as well as clinical and genetic differences associated with these VT types. Methods and results Twenty-six centers from 11 European countries provided information on 952 patients with ACM and >1 episode of sustained VT observed during the patients' clinical course. VT was classified as: LBBB-VT; RBBB-VT or LBBB+RBBB-VT. Among 952 patients, 881 (92.5%) had LBBB-VT alone, 71 (7.5%) had RBBB-VT [alone in 42 (4.4%) patients or with LBBB-VT in 29 (3.0%) patients]. Male prevalence was 90.5%, 79.2% and 55.9% in the RBBB-VT, LBBB-VT and LBBB+RBBB-VT groups, respectively (P=0.001). Patients' age at first VT did not differ amongst the 3 VT groups. ICD implantation was more frequent for the RBBB-VT and the LBBB+RBBB groups (≈90% each) vs. 67.9% for the LBBB-VT group (P=0.001). Death incidence (9.5%–17.2%) was not significantly different between the 3 groups (P=0.425). Plakophylin-2 mutations predominated in the LBBB-VT and LBBB-VT+RBBB-VT groups (47.2% and 27.3%, respectively) and Desmoplakin mutations in the RBBB-VT group (36.7%). Conclusion This large European survey demonstrates: 1) Sustained RBBB-VT is documented in 7.5% patients with ACM; 2) Males markedly predominate in the RBBB-VT and LBBB-VT groups but not in the LBBB+RBBB VT group; 3) Distribution of desmosomal mutations appears to be different in the 3 VT groups. Funding Acknowledgement Type of funding source: None

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