bluuebunny/crossref_metadata_2025_split huggingface dataset:
Relative apical sparing in patients with severe aortic stenosis: prevalence and significance
Authors: Abecasis J , Mendes G , Ferreira A , Andrade M.J , Ribeiras R , Ramos S , Masci P.G , Gil V 
Published: November 2020

Abstract

Relative apical sparing (RAS) of LV longitudinal strain (LS) is a red flag for diagnostic suspicion of amyloid cardiomyopathy (AC). However, it may present in pts with aortic stenosis (AS), where the prevalence of transthyretin AC is being increasingly reported.

Aim
To describe the prevalence of RAS deformation pattern in patients with AS and its clinical significance.

Methods
We prospectively studied 53 pts (71±8y, 54.7% men) with severe symptomatic AS - mean gradient (AVM): 54.6 mmHg; aortic valve area 0.74cm2, referred for surgical replacement with no previous history of ischemic cardiomyopathy. Beyond ECG and transthoracic echo (TTE), all pts underwent CMR, with tissue characterization before surgery. RAS was defined as average apical LS / average basal LS + average mid LS &gt;1 at 2D LV LS analysis. Aortic valve replacement and septal myocardial biopsy were already performed in 26 pts. AS severity indexes, LV remodelling and tissue characterization were compared in both groups, with and without RAS.

Results
RAS was present in 16 pts (30.8%). There were neither pseudoinfarct pattern or low voltage at ECG, nor infiltration suspicion from CMR study (native T1 value 1047ms [IQR 1028–1084]; ECV 22% [IQR 18–25]). Furthermore, none of the pts had suspicion of amyloid deposition at histopathology. Median CMR LVEF was 64.5% [IQR 51.3–70.8%] and 36 pts (67.9%) had non-ischemic DE, with a median fraction of 6.0% [IQR 4.9–12.7%] of LV mass. Comparing both groups, RAS cohort showed a significantly higher AVM, relative wall thickness, maximum septal thickness, peak systolic dispersion and higher LV indexed mass, DE and lower LVEF at CMR. RAS group has also higher NT pro BNP (Table).

Conclusions
RAS is common in this group of pts despite the absence of clinical and histological signs of myocardial infiltration. RAS occurs with worse indexes of LV remodeling and fibrosis consistent with a more advanced stage of the disease.

Funding Acknowledgement
Type of funding source: None
Recommendations on echocardiography following surgical aortic valve replacement (SAVR): time for revision?
Authors: Schmiegelow M , Bruun N.E , Carranza C.L , Dahl J , Elming H , Kober L , Sibilitz K , Torp-Pedersen C.L , Schmiegelow S.S 
Published: November 2020

Abstract

Background
ESC guidelines recommend annual echocardiographic evaluation following biological surgical aortic valve replacement (SAVR), and 5 years following mechanical SAVR. Conversely, increased life expectancy result in increasing demand on health care resources.

Purpose
To assess aortic reintervention rates at 1-year, 3-year and 5-year following biological and mechanical SAVR in relation to estimated echocardiographic controls.

Methods
From the nationwide Danish Register of Surgical Procedures, we identified all patients ≥40 years with isolated biological or mechanical SAVR +/− concomitant coronary artery bypass graft surgery (CABG) during 2000–2016. In 90-day reintervention-free survivors we assessed aortic valve reintervention rates at 1-year, 3-years and 5-years until December 31st, 2017. We further assessed cumulative risk of reintervention by age (&lt;60, 60–69, 70–79, ≥80 years at SAVR) accounting for the competing risk of death during the study period.

Results
The population of 90-day reintervention-free survivors included 10,526 patients with biological SAVR (CABG 39.7%) and 3,677 patients with mechanical SAVR (CABG 23.8%). Reintervention rates at 1-year, 3-years and 5-years were comparable across type of SAVR, and generally low (Figure). Accounting for the competing risk of death, reintervention rates at 5-years were 1.4% (95% CI 1.1–1.6) for biological SAVR and 1.5% (95% CI 1.1–1.9) for mechanical SAVR, respectively. In age-stratified competing risk analyses, we observed the highest rates in patients aged 40–59 years (4% [95% CI 1.8–6] at 5 years for biological SAVR, and 2% [95% CI 1.3–3] for mechanical SAVR). Following biological SAVR, annual echocardiographic controls would yield a total of 34,516 scans in our population in the first 5 years following surgery. This contrasts to a total of 66 reinterventions following biological SAVR in our population between years 1–5 of which the majority was preceded by a hospital admission with a primary diagnosis of endocarditis within the last 90 days prior to the reintervention; which are unlikely to have been diagnosed at the annual assessment scan.

Conclusion(s)
In this nationwide study, reintervention rates following biological or mechanical SAVR were very low within the first five years after surgery suggesting a discrepancy between ESC recommendations on echocardiographic controls following SAVR, the benefit for patients, and the associated resource burden on the health care system.
Figure 1

Funding Acknowledgement
Type of funding source: None
Novel staging classification of primary mitral regurgitation based on the presence of cardiac damage
Authors: Van Wijngaarden A.L , Hiemstra Y.L , Van Der Bijl P , Delgado V , Ajmone Marsan N , Bax J.J 
Published: November 2020

Abstract

Background
The indication for surgery in patients with severe primary mitral regurgitation (MR) is currently based on the presence of symptoms, left ventricular (LV) dilatation and dysfunction, atrial fibrillation and pulmonary hypertension. The aim of this study was to evaluate the prognostic impact of a new staging classification based on cardiac damage including the known risk factors but also including global longitudinal strain (GLS), severe left atrial (LA) dilatation and right ventricular (RV) dysfunction.

Methods
In total 614 patients who underwent surgery for severe primary MR with available baseline transthoracic echocardiograms were included. Patients were classified according to the extent of cardiac damage (Figure): Stage 0-no cardiac damage, Stage 1-LV damage, Stage 2-LA damage, Stage 3-pulmonary vasculature or tricuspid valve damage and Stage 4-RV damage. Patients were followed for all-cause mortality.

Results
Based on the proposed classification, 172 (28%) patients were classified as Stage 0, 102 (17%) as Stage 1, 134 (21%) as Stage 2, 135 (22%) as Stage 3 and 71 (11%) as Stage 4. The more advanced the stage, the older the patients were with worse kidney function, more symptoms and higher EuroScore. Kaplan-Meier curve analysis revealed that patients with more advanced stages of cardiac damage had a significantly worse survival (log-rank chi-square 35.2; p&lt;0.001) (Figure). On multivariable analysis, age, male, chronic obstructive pulmonary disease, kidney function, and stage of cardiac damage were independently associated with all-cause mortality. For each stage increase, a 22% higher risk for all-cause mortality was observed (95% CI: 1.064–1.395; p=0.004).

Conclusion
In patients with severe primary MR, a novel staging classification based on the extent of cardiac damage, may help refining risk stratification, particularly including also GLS, LA dilatation and RV dysfunction in the assessment.

Funding Acknowledgement
Type of funding source: None
Advanced pathologic study for definite diagnosis of mitochondrial cardiomyopathy
Authors: Takeda A , Murayama K , Okazaki Y , Imai-Okazaki A , Ohtake A , Takakuwa E , Yamazawa H , Izumi G , Abe J , Nagai A , Taniguchi K , Sasaki D , Tsujioka T , Basgen J 
Published: November 2020

Abstract

Mitochondrial cardiomyopathy (MCM) is usually recognized as one of the phenotypes of systemic mitochondrial disease. However if there are no cardiac symptoms, it is difficult to make a definite diagnosis because of various cardiovascular phenotypes and no diagnostic criteria in pathological examination. To add myocardial pathology to the diagnostic criteria for mitochondrial respiratory chain disorders, which is the gold standard in the diagnosis of mitochondrial diseases, we performed quantitative analysis of mitochondria using electron microscopy and immunohistopathologic analysis with respiratory chain enzyme antibodies. Ten patients with hypertrophic or restrictive cardiomyopathy who had undergone endomyocardial biopsy were studied. Respiratory chain enzymatic assay and genetic study were performed and four patients were diagnosed with MCM. Using electron microscopy with quantitative analysis, volume density of mitochondria within cardiac muscle cells was significantly increased in the MCM group compared to the non-MCM group (p=0.013). Immunohistopathologic results were compatible with the result of the respiratory chain enzymatic assay. These advanced pathological tests can distinguish MCM from other cardiomyopathies.
Results of immunopathologic study

Funding Acknowledgement
Type of funding source: Public grant(s) – National budget only. Main funding source(s): The Practical Research Project for Rare/Intractable Diseases from the Japan Agency for Medical Research and Development, AMED.
Exploring the phenotypic spectrum of desmoplakin cardiomyopathy
Authors: Protonotarios A , Cannie D , Akhtar M , Savvatis K , Sekhri N , Mohiddin S , Lopes L , Syrris P , Elliott P.M 
Published: November 2020

Abstract

Introduction
Desmoplakin (DSP) gene mutations are reported to cause arrhythmogenic right ventricular cardiomyopathy (ARVC), but recent evidence suggests that they manifest as a much broader range of phenotypes.

Purpose
To describe the phenotypic characteristics of DSP mutation carriers in a consecutive cohort of patients with heart muscle disease.

Methods
A retrospective analysis of consecutive patients with heart muscle disease and their relatives that underwent diagnostic or research genetic testing. Only DSP variants classified as pathogenic by the American College of Medical Genetics and Genomics criteria were considered. Dilated cardiomyopathy (DCM), hypokinetic non-dilated cardiomyopathy (HNDC) and ARVC were diagnosed in accordance with current criteria.

Results
109 mutation carriers were identified: 34 (31%) had DCM and 23 (21%) HNDC. ARVC diagnosis was borderline and definite in 39 (36%) and 39 (36%) individuals, respectively. Sixteen (15%) presented with clinically suspected myocarditis. 23 patients met both definite AC and DCM criteria and 13 patients met definite AC and HNDC criteria. In 87 patients with cardiac magnetic resonance imaging, a spectrum of disease was observed (Table) including an intermediate phenotype characterised by subepicardial LV fibrosis in the absence of ventricular dilatation or dysfunction. Ventricular dysfunction was manifest as left dominant disease (n=22, 48%) and biventricular disease (n=24, 52%); none had right dominant disease.

Conclusions
DSP mutations are associated with left and biventricular phenotypes and can present as myocarditis. Current ARVC and DCM diagnostic criteria inadequately describe the clinical subtypes of DSP related disease. A novel approach to disease classification is proposed.

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): British Heart Foundation
Mechanisms of angina in patients with biopsy-proven viral myocarditis: insights from intracoronary acetylcholine testing
Authors: Seitz A , Martinez Pereyra V , Hubert A , Klingel K , Bekeredjian R , Sechtem U , Ong P 
Published: November 2020

Abstract

Background
Patients with myocarditis often present with angina pectoris despite unobstructed coronary arteries. The underlying pathophysiological mechanism of angina in these patients remains to be elucidated. Coronary artery spasm is a well-known cause of angina in patients with unobstructed coronary arteries. In this study, we sought to assess the frequency of coronary vasomotor disorders in patients with biopsy-proven viral myocarditis.

Methods
In total, 700 consecutive patients who underwent endomyocardial biopsy for suspected myocarditis between 2008 and 2018 were retrospectively screened. Of these patients, viral myocarditis was confirmed in 303 patients defined as histological/immunohistological evidence of myocardial inflammation and presence of viral genome confirmed by PCR. Of these patients, 34 patients had angina despite unobstructed coronary arteries and underwent intracoronary acetylcholine (ACh) provocation testing in search of coronary spasm. Epicardial spasm was defined as acetylcholine-induced reproduction of the patient's symptoms associated with ischemic ECG changes and &gt;90% epicardial vasoconstriction. Microvascular spasm was defined as symptom reproduction and ECG changes in the absence of significant epicardial vasoconstriction.

Results
Patients were 49±16 years old, 62% were male and left ventricular ejection fraction was 54±16%. Most frequent viruses were parvovirus B19 (PVB19, 59%) and human herpes virus 6 (HHV6, 26%), 2 patients had combined PVB19/HHV6 infection and 3 patients other herpesviruses (CMV, EBV, VZV). Epicardial spasm was observed in 10 patients (29%) during ACh testing and microvascular spasm was found in 11 patients (32%). The rate of coronary spasm (epicardial and microvascular) was higher in the PVB19 subgroup compared to HHV6 (80% vs. 33%, p=0.031). In particular, there was a higher prevalence of microvascular spasm in PVB19 compared to HHV6 (45% vs. 0%, p=0.018).

Conclusion
We observed a high prevalence of microvascular and epicardial spasm in patients with biopsy-proven viral myocarditis suggesting coronary spasm as a potential underlying mechanism for angina in these patients. Microvascular spasm was most often observed in patients with PVB19-associated myocarditis.

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): Robert-Bosch-Stiftung; Berthold-Leibinger-Stiftung
Prevalence and prognostic value of right ventricular dysfunction in hypertrophic cardiomyopathy
Authors: Marques A , Pereira A.R , Cruz I , Almeida A.R , Alegria S , Briosa A , Grade Santos J , Sebaiti D , Lopes L , Joao I , Pereira H 
Published: November 2020

Abstract

Introduction
Hypertrophic cardiomyopathy (HCM) is the main cause of sudden cardiac death in the young and a cause of heart failure and death at any age. Nevertheless, adverse long-term outcomes are not easy to predict.

Objectives
To assess the prevalence, predictors and prognostic value of right ventricular (RV) dysfunction in patients (pts) with HCM.

Methods
Retrospective single-center study of consecutive pts with HCM evaluated in a specialized medical appointment. Selected those submitted to cardiac magnetic resonance imaging (MRI) as the gold-standard for RV function assessment. The primary endpoint was a composite of cardiovascular death, nonfatal myocardial infarction, nonfatal stroke, ventricular arrhythmias with hemodynamic instability and unplanned heart failure admission.

Results
Were included 112 pts (mean age at first appointment 57±15 years, 63% male). Septal asymmetric phenotype was the most frequent (75%), with a mean septal wall thickness of 18±4 mm. Late gadolinium enhancement was observed in 82%, mostly intramyocardial (67%) and in joint points (47%).
RV dysfunction was detected in 6 pts (5.4%) and RV free wall hypertrophy in 3 pts (2.7%); no patient presented RV dilation.
Factors associated with RV dysfunction were left atria area (HR 1.07/unit, 95% CI 1.01–1.12, p=0.02), left ventricular ejection fraction (HR 0.91/unit, 95% CI 0.86–0.97, p=0.02) and the presence of left ventricle wall motion abnormalities (HR 7, 95% CI 1.3–38, p=0.03) in cardiac MRI.
During a mean follow-up of 60±31 months, the combined primary endpoint occurred in 15 pts (13%), significantly more in pts with RV dysfunction (HR 5.1, 95% CI 1.1–24, p=0.038) (graphic 1). Patients with RV dysfunction also presented more atrial fibrillation / flutter episodes during follow-up (HR 6.4, 95% CI 2.1–20, p=0.001).

Conclusions
Although not common, right ventricular dysfunction was associated with a higher rate of cardiovascular events. These results support a potential role of right ventricular function in the risk stratification of patients with hypertrophic cardiomyopathy.
Figure 1

Funding Acknowledgement
Type of funding source: None
Diastolic stress test echocardiography in patients with hypertrophy cardiomyopathy: association with exercise capacity
Authors: Fava A.M , Sande Mathias I , Alashi A , Saijo Y , Popovic Z , Thamilarasan M , Lever H , Desai M.Y 
Published: November 2020

Abstract

Background
Diastolic dysfunction (DD) is a frequent occurrence in hypertrophic cardiomyopathy (HCM) patients. Symptoms of DD are often unmasked only during exercise, as left ventricular (LV) filling pressure is normal at rest.

Purpose
We sought to establish if abnormal DST parameters are associated with reduced exercise capacity in HCM patients.

Methods
We examined 590 asymptomatic/minimally symptomatic HCM patients (54±14 years, 57% men, body mass index 30±6 kg/m2, 84% on beta-blockers) with HCM by echocardiography at rest & maximal exercise. Septal and lateral [e'] mitral annular velocity, peak early [E] and late [A] mitral inflow velocity, Mitral E/e' and left atrial volume index (LAVI) was recorded. Exercise functional capacity was stablished as age-gender predicted metabolic equivalents (AGP-METs).

Results
Echocardiography at rest had LVEF of 62±6, wall thickness of 16.7±0.5 mm, LVMI of 111±43 g m–2, LVOT gradient of 26±33 mmHg, LAVI 34.5±17 mm, E/A ratio of 1.2±0.6, and average E/e' ratio 12.7±0.6. Following maximal exercise had a mean LVOT gradient of 61±59 mmHg, E/A ratio of 1.2±1.0 and average E/e' ratio 12.7±1.0. Only 42% had &gt;85% of age-sex predicted METs. Logistic regression analysis testing the association between various predictors and 85% AGP-METs are shown in table 1.

Conclusion
There is a significant association between abnormal diastolic response to exercise and reduced exercise capacity in patients with HCM.

Funding Acknowledgement
Type of funding source: None
Targeted septal branch microcirculatory embolization with tris-acryl gelatin microspheres in hypertrophic obstructive cardiomyopathy
Authors: Okutucu S , Aksoy H , Yetis Sayin B , Oto A 
Published: November 2020

Abstract

Background
Alcohol septal ablation (ASA) has been shown to be an effective treatment in patients with hypertrophic obstructive cardiomyopathy (HOCM) who are refractory to medical treatment. ASA may cause some life-threatening complications including conduction disturbances, hemodynamic compromise, ventricular arrhythmias, distant and massive myocardial necrosis. Tris-acryl gelatin microspheres provide consistent and predictable results for effective targeted microcirculatory embolization.

Purpose
We aimed to report our initial experience in tris-acryl gelatin microspheres for septal ablation in HOCM.

Methods
Microspheres are biocompatible, hydrophilic, non-resorbable microspheres which are available in a range of calibrated sphere sizes. In our method, after the cannulation of the left anterior descending by a 6F-7F guiding catheter, a 0.014-inch guidewire is introduced through the catheter and advanced into the septal branch. This septal artery is selectively cannulated with a 4F catheter over the guidewire. Selective angiography of the septal artery is performed to show the anatomy and collateral branches to other coronary arteries. Contrast echocardiography is performed to make sure that the pertinent septal artery is the target vessel supplying the hypertrophied septum. A microcatheter is then advanced deep enough into the septal artery through the 4F catheter. Microspheres/contrast solution infused slowly under fluoroscopic guidance into the targeted septal branches initially using coronary arteriolar sized small particles (diameter 100–300 μm); then the particle size was stepped up to larger particles (diameter 300–500 μm) until a complete block of the arteriolar flow is achieved.

Results
Septal ablation with tris-acryl gelatin microspheres was performed in 6 patients (mean age = 47.8±11.5; 5 males). Immediately after the procedure peak left ventricular outflow (LVOT) gradient reduced significantly both for direct catheter (69.0±13.8 vs. 8.2±3.7 mmHg, P&lt;0.001) and Doppler echocardiographic measurements (78.8±19.9 vs. 12.0±5.1 mmHg, P&lt;0.001). Post-procedure peak serum CK- MB fraction concentration was 82±22 ng/ml (reference range is 0 - 4.9 ng/mL) and peak serum troponin T concentration was 1.2 ng/ml [(interquartile range, 0.4–1.4), (reference range is 0 - 0.017 ng/mL)]. LVOT tract gradient reduction persisted after 6 months follow-up. There was no significant complication during the procedure and within a 6 months follow-up period.

Conclusions
The novel strategy by targeted septal branch microcirculatory embolization with tris-acryl gelatin microspheres seems to be an efficient and safe approach to HOCM. Further experience is needed in order to assess the long-term efficacy and safety of this technique.

Funding Acknowledgement
Type of funding source: None
Prognostic accuracy of common heart failure scales in patients with dilated cardiomyopathy
Authors: Dziewiecka E , Gliniak M , Winiarczyk M , Karapetyan A , Wisniowska-Smialek S , Karabinowska A , Podolec P , Rubis P 
Published: November 2020

Abstract

Introduction
The reliable identification of patients with heart failure (HF) and high mortality risk is crucial for treatment optimalization and prognostication, especially in younger population in dilated cardiomyopathy (DCM). However, the profile of DCM patients significantly differs from the general HF population. Therefore, the applicability of commonly used HF prognostic scales may be sub-optimal in DCM.

Purpose
Comparison of the prognostic accuracy of common HF prognostic scales in DCM.

Methods
Between 2010 and 2018 we analysed 406 DCM in- and outpatients (aged 54±14 years, 76% male, NYHA: 2.5±0.9, symptoms duration: 40±58 months, EF: 26±9%, LVEDd: 66±10mm, NT-proBNP: 3662±7617pg/ml, 10% had ICD, 3.5% had CRT). The mortality risk was assessed by 8 most popular HF prognostic scores. In 2019 information on patients status were gathered after 48±32months.

Results
During first month 4 patients (1.0%) died, after 1 year – 19 (6%), 2 years – 32 (11%), 3 years – 42 (18%), 4 years – 52 (27%), 5 years – 59 (40%). BCN and SHFM calculators had the best prognostic ability for 1–5 years mortality (Table 1).

Conclusions
Mortality risk of DCM patients during 5 years is high and reaches 40%. BCN and SHFM calculators had the best prognostic ability for 1–5 years mortality.

Funding Acknowledgement
Type of funding source: Public Institution(s). Main funding source(s): Department of Scientific Research and Structural Funds of Medical College, Jagiellonian University