bluuebunny/crossref_metadata_2025 huggingface dataset:
P1730Serial high-sensitivity troponin I measurements to discriminate type 2 from type 1 myocardial infarction
Authors: Psyrakis D A , Bormann J , Von Jeinsen B , Wolter J S , Weferling M , Diouf K , Kriechbaum S , Troidl C , Doerr O , Nef H , Liebetrau C , Keller T
Published: October 2019

Background

Acute myocardial infarction (MI) is associated with high morbidity and mortality. A robust differentiation between type 1 and type 2 MI (T1/T2MI) has prognostic and therapeutic implications. We investigated whether serial high-sensitivity cardiac troponin I measurements could reliably discriminate T1MI from T2MI in patients presenting with a non-ST elevation myocardial infarction (NSTEMI).

Methods

We used data from a prospective acute coronary syndrome biomarker registry of patients with suspected MI that presented at or were transferred to one of two study centres. Here, we analysed an unselected group of 265 NSTEMI patients (67.2% males). Blood was drawn on admission and after 3 hours. High-sensitivity troponin I (hs-cTnI) was measured in frozen samples by a technician blinded to patient characteristics. T1MI or T2MI was defined as the gold-standard study diagnosis by two independent cardiologists based on all available data according to the Third Universal Definition of MI.

Results

A diagnosis of T2MI was made in 55 patients (20.8%) in the NSTEMI cohort. T2MI patients did not differ from T1MI patients regarding age, gender, traditional risk factors, or percentage of those with a history of coronary artery disease. Median baseline hs-cTnI levels were higher in T1MI (436.25; IQR 63.7–1918.8 ng/L) than in T2MI patients (48.4; IQR 11.7–305.9 ng/L; p<0.001). Absolute change in hs-cTnI concentration between 0 and 3 h was greater in T1MI than in T2MI patients with Dhs-cTnI 93.6 ng/L (IQR 13.5–815.3 ng/L) vs. 20.4 ng/L (IQR 2.5–106.5 ng/L) (p<0.001). hs-cTnI yielded an area under the receiver operator characteristics (AUROC) curve for identifying T2MI at baseline of 0.71 (IQR 0.64–0.79) and after 3 h of 0.7 (IQR 0.61–0.78).Dhs-cTnI was associated with an AUROC of 0.68 (IQR 0.6–0.76). Regarding a rule-out approach, Youden-optimized cut-offs for hs-cTnI at baseline as well as for the absolute change in hs-cTnI concentration were calculated (186.5 ng/L; 154.4 ng/L). Use of these two criteria yielded a sensitivity of 89% (78–96%) and a negative predictive value of 95% (89–98%) to exclude T2MI. 49 of 55 T2MI patients would have been ruled out using this algorithm.

Conclusion

Our data show that hs-cTnI concentrations differ between patients presenting with T1 and T2MI. The concentration of hs-cTnI and its change over time has the potential to rule out T2MI and therefore to identify patients who might benefit from an early invasive management. The differentiation between T1MI and T2MI by using hs-cTnI is nevertheless challenging, and further research on specific algorithms is needed.

Acknowledgement/Funding

3German Center for Cardiovascular Research (DZHK), Partnersite Rhein Main, Bad Nauheim, Germany
P1735Two-hour algorithm for early diagnosis of acute myocardial infarction using a novel high-sensitivity cardiac troponin I assay
Authors: Nestelberger T , Boeddinghaus J , Greenslade J , Cullen L , Parsonage W , Troester V , Koechlin L , Rubini M , Badertscher P , Puelacher C , Twerenbold R , Mueller C
Published: October 2019

Background

We aimed to derive and externally validate a 0/2h-algorithm using the novel high-sensitivity cardiac troponin I (hs-cTnI-Access) assay.

Methods

We enrolled patients presenting to the emergency department with symptoms suggestive of acute myocardial infarction (AMI) in two prospective chest pain trials. Two independent cardiologists adjudicated the final diagnosis including all available medical information including cardiac imaging. Hs-cTnI concentrations were measured at presentation and after 2h. Primary diagnostic endpoint was the derivation and validation of an hs-cTnI-Access specific 0/2h-algorithm. Primary prognostic endpoint was overall survival of patients after 30- and 720-days of follow-up.

Results

AMI was the adjudicated final diagnosis in 164/1131 (14.5%) patients in the derivation and in 88/1280 (6.9%) patients in the validation cohort. Median hs-cTnI Access concentrations at presentation were significantly higher in patients with AMI as compared to patients with non-AMI in both cohorts (104 ng/L versus 3.4 ng/L and 29 ng/L vs. 2.3 ng/L, p-value both <0.001) Applying the derived hs-cTnI-Access 0/2h-algorithm (Figure 1A) to the validation cohort (Figure 1B), 77.9% of patients were ruled-out (sensitivity 97.7% [95% CI, 92–99.7], negative predictive value [NPV] 99.8% [95% CI, 99.3–100]), and 5.8% of patients were ruled-in (specificity 98.6% [95% CI, 97.7–99.2], positive predictive value [PPV] 77% [95% CI, 65.8–86]). Among 1617 patients ruled-out for AMI in both cohorts together, 3 (0.2%) patients with AMI have been missed, of whom 2 patients had type 2 myocardial infarction (both with tachyarrhythmia). Patients ruled-out by the 0/2h-algorithm had a survival rate of 98.4% and 99.9% after two years or one year of follow up in both cohorts, respectively.

Figure 1

Conclusions

Diagnostic performance of the hs-cTnI Access 0/2h-algorithm for triage of AMI is excellent with high safety for rule-out and high accuracy for rule-in.

Acknowledgement/Funding

European Union, Swiss National Foundation, University Hospital Basel, University Basel
P1745Clinical characteristics and long-term outcome in patients with helicopter-transported acute coronary syndrome after primary percutaneous coronary intervention
Authors: Takahashi N , Ogita M , Tsuboi S , Nishio R , Yasuda K , Takeuchi M , Iso T , Sonoda T , Yatsu S , Wada H , Shiozawa T , Dohi T , Yanagawa Y , Suwa S , Daida H
Published: October 2019

Background

Reducing delay to percutaneous coronary intervention improves functional outcome and reduces long-term mortality. Transportation by helicopter is often quicker than ground transport and thus may improve overall prognosis through reduced ischemic injury and infarction size. Our hospital is located on the medically-depopulated peninsula surrounded by mountain. The journey from the southern tip of the peninsula to the critical care medical center of our hospital take 1.5 hour by a ground ambulance but only 15 minutes by helicopter.

We compared the clinical characteristics and long-term mortality between air and ground transport of ACS patients for primary PCI.

Methods

We conducted an observational cohort study evaluating 2324 patients (mean age 68.5±12.0, male 75.2%) with ACS underwent primary PCI between April 2004 and December 2017 at our hospital.

We divided into three groups according to transportation system type (air, ground, walk-in).

The primary outcome was defined as all-cause death during the long-term follow-up.

Results

Among the entire cohort, 577 patients (24.8%) were transported by air. 1326 (57.1%) patients by ambulance, 421 (18.1%) patients by walk. Baseline characteristics were comparable, but patients by air had a higher prevalence of ST-elevation myocardial infarction.

The rate of long-term mortality was comparable during the median follow up of 6 years (air, 21.1% vs. ground, 21.4% vs. walk-in, 21.1%, respectively, log-rank p=0.72). Multivariate Cox regression analysis showed no significant association between air transportation and long-term mortality (Adjusted HR [vs ground] 1.05, 95% CI 0.60–1.78, p=0.85 and [vs walk-in] 0.94, 95% CI 0.62–1.43, respectively, p=0.77).

Kaplan-Meier curve

Conclusions

The rate of long-term mortality in patients with ACS transported by air was comparable with those transported by ground.
P1750YKL-40 in patients with myocardial infarction is associated with survival rate
Authors: Sciborski K , Gierlotka M , Protasiewicz M , Karolko B , Negrusz-Kawecka M , Mysiak A , Kuliczkowski W
Published: October 2019

Background

YKL-40 is a protein released locally by inflammatory cells. Since inflammation has been implicated in the pathogenesis of atherosclerosis, YKL-40 is also considered as a potential biomarker and prognostic factor in cardiovascular diseases.

Purpose

The aim of the study was to analyze serum concentration of YKL-40 in patients with ischemic heart disease, and to verify if this parameter could be considered as a potential biomarker and prognostic factor in cardiovascular diseases.

Methods

The study included 158 participants, among them 52 patients with stable ischemic heart disease, 67 with acute coronary syndrome, and 39 controls without abnormalities in coronary vessels. Prior to the coronarography, a 5-ml sample of venous blood was collected from a peripheral vein of each patient. Serum concentration of YKL-40 was determined once, with an immunoenzymatic assay. For the next 4 years follow-up of patients was conducted, including hospitalizations and deaths.

Results

Patients with myocardial infarction presented with significantly higher serum concentrations of YKL-40 (ng/ml) than persons with stable ischemic disease (249.6±110.9 vs. 86.0±62.7; p<0.001) and the controls (249.6±110.9 vs. 60.2±20.1; p<0.001). No statistically significant differences were found in YKL-40 protein concentrations in patients with ST elevation (STEMI) and non-ST elevation myocardial infarction (NSTEMI) (255.1±110.3 vs. 236.6±113.9; p=NS).

In the ROC analysis YKL-40 value above 360 ng/ml (AUC 0.93; 95% CI 0.86–0.98; p=0.00012) with 80% sensitivity and specificity predicted higher mortality up to 4 years after acute coronary syndrome (p=0.0014) (Figure 1).

4-year probability of survival

Conclusion

Higher serum concentration of YKL-40 at admission in patients with myocardial infarction is associated with higher mortality up to 4 years after acute coronary syndrome.
P1755Plasma proteomics in healthy individuals predict myocardial infarction
Authors: Hoogeveen R , Belo Pereira J P , Zampoleri V , Bom M J , Koenig W , Catapano A L , Wareham N J , Khaw K T , Boekholdt S M , Groen A K , Levin E , Stroes E S G
Published: October 2019

Background

Currently used models to predict cardiovascular event risk have limited value. It has been shown repetitively that the addition of single biomarkers has modest impact. Recently we observed that a model consisting of a larger array of plasma proteins performed very well in predicting the presence of vulnerable plaques in primary prevention patients. However, the validation of this protein panel in predicting cardiovascular outcomes remains to be established.

Purpose

This study investigated the ability of a 384 preselected protein biomarkers to predict acute myocardial infarction, using state-of-the-art machine learning techniques. Secondly, we compared the performance of this multi-protein risk model to traditional risk engines.

Methods

We selected 822 subjects from the EPIC-Norfolk prospective cohort study, of whom 411 suffered a myocardial infarction during follow-up (median 15 years) compared to 411 controls who remained event-free (median follow-up 20 years). The 384 proteins were measured using proximity extension assay technology. Machine learning algorithms (random forests) were used for the prediction of acute myocardial infarction (ICD code I21–22). Performance of the model was tested against and on top of traditional risk factors for cardiovascular disease (refit Framingham). All performance measurements were averaged over several stability selection routines.

Results

Prediction of myocardial infarction using a machine-learning model consisting of 50 plasma proteins resulted in a ROC AUC of 0.74±0.14, in comparison to 0.69±0.17 using traditional risk factors (refit Framingham. Combining the proteins and refit Framingham resulted in a ROC AUC of 0.74±0.15. Focussing on events occurring within 3 years after baseline blood withdrawal, the ROC AUC increased to 0.80±0.09 using 50 plasma proteins, as opposed to 0.67±0.22 using refit Framingham (figure). Combining the protein model with refit Framingham resulted in a ROC AUC of 0.82±0.11 for these events.

Diagnostic performance events <3yrs

Conclusion

High-throughput proteomics outperforms traditional risk factors in prediction of acute myocardial infarction. Prediction of myocardial infarction occurring within 3 years after inclusion showed highest performance. Availability of affordable proteomic approaches and developed machine learning pave the path for clinical implementation of these models in cardiovascular risk prediction.

Acknowledgement/Funding

This study was funded by an ERA-CVD grant (JTC2017) and EU Horizon 2020 grant (REPROGRAM, 667837)
P1758The novel small leucin-rich repeat protein podocan is an independent predictor of major adverse cardiac events in patients with angiographically-defined coronary artery disease
Authors: Watthanasuntorn K , Kandala J , Shrestha B , Thongprayoon C , Victory J , Scribani M , Jenkins P , Hyink D , Klotman P , Hutter R
Published: October 2019

Background

Smooth muscle cell (SMC) function determines the clinical course of vascular disease via fibrous cap stability. Podocan is an inhibitor of SMC function and is circulating in peripheral blood rendering it a candidate biomarker to predict MACE in patients with Coronary Artery Disease (CAD).

Purpose

We designed a prospective cohort study assessing the predictive value of Podocan for cardiovascular outcome (MI, CVA or death) in patients with CAD.

Methods

308 patients with angiographic evidence of CAD were enrolled. At index cardiac catheterization Syntax Score was calculated. For patient baseline characteristics see Table. Podocan and CRP-1 were measured using a human Podocan and CRP-1 ELISA. The Kaplan-Meier method was used to construct survival curves, which were compared using the log-rank test. Cox proportional hazard modeling was used for all univariate/multivariate analyses. Statistical analysis was performed using STATA.

Results

Podocan was detected in 212 patients (69%) with a detection threshold of 0.01 ng/ml. The median Podocan level observed was 1.4±8.2 ng/ml. 96 patients did not have a detectable Podocan level. Mean CRP-1 was 0.117±0.15 mg/ml. Mean Syntax Score was 12±9. Podocan did not correlate with CRP-1. There was also no association between Podocan and Syntax Score, age, BMI, smoking, LDL, and HDL, HgbA1c, LVEF and GFR. At the univariate level, presence of Podocan was associated with an increased rate of MACE (17% Podocan present vs. 7% Podocan absent, p=0.02). Kaplan-Meier survival analysis showed higher event free survival in patients with no detectable Podocan vs. detectable Podocan level (Figure). In a limited multivariate Cox proportional Hazard analysis, Podocan remained an independent predictor of MACE (HR: 2.5; P=0.042) in addition to diabetes, and LV ejection fraction.

Baseline Characteristics Total (N=308) Chronic ischemic heart disease (N=273) Acute coronary syndrome (N=35) Age (Year) 66.5±9.5 67±9 61±11 Female (Sex) 106 (33%) 90 (31%) 16 (46%) Hypertension 282 (89%) 244 (89%) 26 (74%) Diabetes 142 (44%) 124 (45%) 11 (31%) Hyperlipidemia 269 (87%) 243 (89%) 26 (74%) CRP (mg/dL) 0.11±0.14 0.10±0.13 0.18±0.19 LVEF (%) 49±10 49±10 48±9.5 CRP, C-reactive protein; LVEF, Left ventricular ejection fraction.

Kaplan Meier Survival Curves by Podocan

Conclusion

Podocan is a novel biomarker independently predicting MACE in secondary prevention of CAD warranting to be further studied in a Multicenter Clinical Trial.
P1761Distal transradial approach for primary percutaneous coronary intervention for patients with acute myocardial infarction: a multicentre study
Authors: Izumikawa T , Takeshita S , Yamada T , Mizuguchi Y , Taniguchi N , Nakajima S , Hata T , Takahashi A
Published: October 2019

Background

The distal transradial approach (dTRA) for coronary catheterisation is a newly introduced alternative to the conventional transradial approach. This technique is expected to decrease the incidence of haemorrhagic complications and improve patient comfort. However, limited data are available regarding the application of this technique in patients with acute myocardial infarction (AMI). This study investigated the feasibility and safety of the dTRA for primary percutaneous coronary intervention (PCI) in patients with AMI.

Methods

This study included patients with AMI who underwent primary PCI via the distal radial artery across 3 Japanese hospitals between January 2018 and January 2019. Patients' background, procedural characteristics, and clinical outcomes including the incidence of haemorrhagic complications were analysed.

Results

This study enrolled 95 consecutive patients with AMI, including 68 patients (71.6%) with ST-segment elevation myocardial infarction (STEMI), in whom distal radial artery puncture was attempted for primary PCI. The patients included 70 men (73.7%), and the mean age was 72.2±12.4 years. Among these patients, cannulation was successfully performed in 89 patients (93.7%). A 5-, 6-, or 7-French sheath (conventional or slender) was used in this study. Cannulation was performed using a forearm radial artery approach in patients in whom dTRA failed.

PCI was successfully performed in all patients. The meantime to achieve haemostasis was 6.3±5.3 hours, and no major bleeding complications occurred. Based on The Early Discharge After Transradial Stenting of Coronary Arteries trial haematoma scale, grade I, II, and III subcutaneous haemorrhages were observed in 16 (16.8%), 4 (4.2%), and 1 patient (1.1%), respectively. No patient developed a haematoma > grade IV.

In patients with STEMI, the mean door-to-balloon time was 39.4±31.9 min, and the mean puncture-to-balloon time was 19.7±14.2 min.

Conclusions

The distal radial approach is feasible and safefor primary PCI in selected patients with AMI.The application of the dTRA may serve as a less invasive strategy for the treatment of patients with AMI.
P1764Quality criteria for STEMI care - a national perspective
Authors: Da Conceicao Pedro Pais J A , Picarra B C , Carrington M , Santos A R , Guerreiro R A , Carvalho J , Bras D , Congo K , Neves D , Aguiar J
Published: October 2019

Introduction

The definition of quality criteria in health care is essential to implement structural organization strategies that ensure that patients (P) receive the best care according to the most updated recommendations available at the time they are treated. Quality indicators have recently been defined with regard to the approach and treatment of STEMI in the European Guidelines published in 2017.

Objective

The authors intend to characterize the level of care provided to P with STEMI inserted into a national multicenter registry since 2011 in order to establish a relationship with international recommendations.

Population and methods

Descriptive study based on a national multicenter registry. A total of 2051 P admitted to the hospital with the diagnosis of STEMI were included, 1266 P for the year 2011 and 785 P for the year 2016.

Results

The P of the year 2011 and 2016 presented similar age (64±14 vs 63±13). There was a higher percentage of P admitted by STEMI fast track managed care system in 2016 (36.9% vs 22.2%, p<0,001) and less from the Emergency Department (31.6% vs 56.8%; p<0,001). Regarding the transport to the Hospital, there was an increase in patients transported by prehospital medical teams (28% vs 21%; p<0,001) and less by own means (35.7% vs 45.6%; p<0,001). Regarding in-hospital therapy, it was found that in 2016 more patients received loading doses of P2Y12 inhibitors – Clopidogrel (78.1% vs 70.3% P<0,001) and Ticagrelor (54.7% vs 0.7% P<0,001). In post-discharge therapy, there was also a slight improvement in care in 2016, with more P being treated with P2Y12 inhibitor (96.2% vs 92.4%; p=0.03) and beta-blocker (84.4% vs 78.7%; p<0,001). Regarding the type of reperfusion, there was an increase in angioplasty (95.5% vs 92.2%; p<0,001) and a decrease in fibrinolysis (4.5% vs 7.8%; p=0.03) in 2016. There was a slight worsening of the prehospital delay in 2016 (median 163min vs 120min) and an improvement in door to reperfusion time (median 60min vs 70min). There was also an increase in angioplasties performed in 2016 (87.1% vs 85.1%; p<0,001) as well as an increase in the percentage of left ventricular function evaluation before discharge (98.2% vs 93.9%; p<0,001).

Conclusion

The results presented demonstrate a slight improvement in the quality of the care provided to STEMI P. However, there are areas for improvement, in accordance with international recommendations, in particular with regard to reperfusion times.
P1767Prevalence and clinical significance of sleep-disordered breathing in patients with hypertrophic cardiomyopathy
Authors: Potratz M , Fox H , Fox H , Faber L , Faber L , Oldenburg O , Oldenburg O , Rudolph V , Rudolph V , Bitter T , Bitter T
Published: October 2019

Background

Despite major advances in knowledge on hypertrophic cardiomyopathy (HCM) at the genetic and molecular levels, the understanding of essential clinical aspects remains limited. The aim of this study is to identify the prevalence and clinical significance of Sleep-disordered Breathing (SDB) in a large patient population.

Methods

201 patients (78 women, age 64±15 years) with HCM were screened for obstructive (OSA) or central (CSA) sleep-disordered breathing using multichannel cardiorespiratory polygraphy. Additionally, patients received a spiroergometric examination and echocardiography. SDB was defined as apnea-hypopnea index (AHI) ≥5/h and OSA/CSA differentiated after the majority of events.

Results

SDB was documented in 60% of all patients. OSA was diagnosed in 71 patients (35.3%) and CSA in 44 (21.9%) patients. SDB requiring treatment was found in 106 (52.7%) patients. In patients with AHI ≥15/h NYHA class was increased (2.1 vs. 2.39, p=0.04) and maximal O2 uptake during exercise was lower (20.1 vs. 16.1 p<0.001). Also left atrial diameter was significantly larger (46.9 mm vs. 50.41 mm, p=0.01) and rate of atrial fibrillation was increased (0.5 vs. 0.72 p=0.03). CSA pts had a larger left atrial diameter compared to pts with OSA (52.13 mm vs. 47.82 mm, p=0.02).

Conclusion

There is a high prevalence of SDB in HCM patients. Patients with moderate to severe SDB showed increased atrial fibrillation incidence and reduced cardiopulmonary performance. Whether the SDB has an independent prognostic relevance in patients with HCM needs to be elucidated.
P1769Microvascular resistance increases with left ventricular mass in hypertrophic cardiomyopathy
Authors: Bakar S , Hayman S , McCarty D , Thain A , McLellan A , Wagner C , Lavi S
Published: October 2019

Introduction

Hypertrophic cardiomyopathy is often associated with myocardial ischemia despite lack of focal epicardial coronary stenoses and this is partially attributable to microvascular dysfunction. The index of microvascular resistance (IMR) is a hemodynamic index that is independent of epicardial stenoses and reflects coronary microvascular function.

Purpose

To investigate the relationship between microvascular dysfunction and the degree of hypertrophy in hypertrophic cardiomyopathy patients.

Methods

We performed a prospective study to assess epicardial and microvascular coronary hemodynamics in 12 subjects with hypertrophic cardiomyopathy undergoing diagnostic coronary angiography. A pressure-temperature sensor coronary guidewire was used with intracoronary injections of room-temperature saline to measure mean coronary transit time during rest and hyperemia induced with intravenous adenosine. IMR was calculated by multiplying mean coronary transit time during hyperemia by distal coronary pressure in each subject. Left ventricular mass was calculated from baseline echocardiographic studies using established methods. Continuous variables are shown as mean ± standard deviation.

Results

Six subjects were male; mean age was 59.8±11.6 years. Baseline body mass index was 30.5±6.1 kg/m2 and left ventricular ejection fraction was 76.7% ± 11.0% with mean left ventricular mass 230.1±76.6 grams by echocardiography. Ten subjects had asymmetric septal hypertrophy; one subject had marked apical hypertrophy and one subject had asymmetric septal and inferolateral hypertrophy. Resting left ventricular outflow tract gradient was 59.4±37.8 mmHg and increased to 90.0±54.1 mmHg with Valsalva maneuver. Systolic anterior motion of the mitral valve was present in 10 subjects. Hemodynamic variables included coronary flow reserve (2.1±1.2); hyperemic mean transit time (0.29±0.15 sec.), and IMR (21.7±10.2). Index of microvascular resistance was strongly positively correlated with left ventricular mass (Figure 1; Pearson r=0.68, p=0.021).

Figure 1. IMR is correlated with LV mass

Conclusion(s)

Microvascular dysfunction as assessed by IMR is strongly correlated with left ventricular mass and may contribute to symptoms in patients with hypertrophic cardiomyopathy.
P1770A MYBPC3 founder splicing mutation with high penetrance and early onset of Hypertrophic Cardiomyopathy in men
Authors: Mendez Fernandez I , Fernandez Avila A , Espinosa Castro M A , Lorca Gutierrez R , Cuenca Parra S , Tamargo Delpon M , Rodriguez Crespo J F , Vazquez Aguilera N , Yotti Alvarez R , Bermejo Thomas J , Fernandez Aviles F
Published: October 2019

Introduction

MYBPC3 is the most frequently affected gene in Hypertrophic Cardiomyopathy (HCM). This study aimed to analyze the founder origin and clinical phenotype of the MYBPC3 c.2149–1G>A pathogenic variant, which had been previously described in only one HCM proband.

Methods

HCM probands who underwent genetic test at our institution were reviewed. Clinical data from carriers were collected retrospectively. In order to identify the haplotypes sharing MYBPC3 c.2149–1G>A variant, six markers covering from 46 to 62 Mb of HSA11 were genotyped in 11 carriers and 6 non-carriers. Genotype information for those markers was extracted for Iberian population (n=107) from the 1000 Genomes Browser database. Haplotypes were reconstructed with Phase 2.1 software and phylogenetic analysis with MEGA7 software.

Results

MYBPC3 c.2149–1G>A was identified in 7 of 523 HCM probands and 23 family members. Penetrance in carriers older than 30 years was 80% (100% men, 55% women, p=0.03). Men were significantly younger at diagnoses, with larger left atrium. One patient had an aborted sudden death. The haplotype reconstruction showed the existence of 53 haplotypes. The most probable segregation for MYBPC3 c.2149–1G>A carriers was estimated in two haplotypes, H52 and H53. The phylogeny reconstruction estimated that both H52 and H53 haplotypes may come from common ancestor H16. In fact, H52 comes from one recombination event in H53 haplotype that has recently occurred in one of our families.

Characteristics of affected carriers All (n=16) Men (n=11) Women (n=5) p Age at diagnoses (years) 44 (19) 38 (22) 48 (14.5) 0.036 Maximal LVWT (mm) 19 (4) 19 (3) 17 (6) 0.209 Ejection Fraction (%) 61 (5) 61 (4) 68 (17) 0.131 Left atrium (mm) 42 (13) 44 (15) 35 (11) 0.05 Outflow Tract Obstruction 2 (12.5%) 2 (18.2%) 0 (0%) 0.524 Late Gadolinium Enhancement† 13 (92.9%) 9 (90.0%) 4 (100%) 0.714 Dyspnea (NYHA ≥2) 4 (25%) 4 (36.4%) 0 (0%) 0.242 NSVT 6 (37.5%) 4 (36.4%) 2 (40%) 0.538 Atrial Fibrillation 4 (25%) 3 (27.3%) 1 (20%) 0.635 ICD primary/secondary prevention 7 (43.8%)/1 (6.3%) 5 (45.5%)/1 (9.1%) 2 (40%)/0 (0.0%) 0.750 Data are expressed as median (interquartile range) or number (%). LVWT: left ventricular wall thickness; NSVT: non sustained ventricular tachycardia. †14 man and 4 women underwent cardiac magnetic resonance.

MYBPC3 haplotype tree.

Conclusions

MYBPC3 c.2149–1G>A is a founder pathogenic variant that shows a high penetrance and an early onset of HCM, specially in men.
P1773Predictors of arrhythmic events in patients with non-compaction cardiomyopathy
Authors: Vaikhanskaya T , Kurushka T V , Sivitskaya L N
Published: October 2019

The phenotypic expression of non-compaction cardiomyopathy (NCM) ranges from subclinical arrhythmias to sudden cardiac death.

Objective

Search of identifying predictors of life-threatening ventricular tachyarrhythmias (ltVTA) and analysis arrhythmic risk stratification capacity of ECG (heart rate turbulence – HRT, microvolt T-wave alternans – mTWA, QT interval corrected - QTc, and fragmented QRS - frQRS), Echo and MRI features in pts with NCM was the goal of this study.

Methods

The study enrolled and followed up 59,7±16,2 months 72 pts (family – 13; sporadic – 59; aged 39.2±14.1 years; 43/59.7% male; LVEF 44.7±13.2%) with morphological signs of non-compaction myocardium, confirmed by any three of the four standart criteria: Echo (Jenni, Stöllberger), MRI (Petersen, Jacquier). For analysis as primary end-points the following ltVTA events were accepted: SCD, successful resuscitation, stVT/VF/syncope, appropriated ICD discharges.

Results

As a result of the rank correlation Spearman analysis positive correlations (p<0.0001) of an MRI markers of myocardial fibrosis were revealed with ECG parameters; thus, leads (n) with frQRS (frQRSn: r=0.65) and QT interval corrected (QTc: r=0.66) correlated with T1 native relaxation time (ms) and the LGE myocardial segments (n). Significant correlations (p<0.0005) are found between LGE (%) and frQRS(n): r=0.61; QTc: r=0.56; VTA (r=0.48); systolic dysfunction (LVEF: r=−0.59; GLS: r=0.58; TAPSE: r=−0.55; RVEF: r=−0.54) and biventricular non-compaction (r=0.60). Positive correlations of the frQRS(n) with the pathological mTWA test (mTWA mean: r=0.55; p<0.001), VTA (r=0.61; p=0.0008) and negative correlations with systolic/contractile function (LVEF: r=−0.56, p=0.001; TAPSE: r=−0.51, p=0.003; LV GLS “–”%: r=−0.57, p<0.001) were determined. Surprisingly, significant correlations between NC/C ratio (one of the main diagnostic criteria of LVNC) and other phenotypic signs or clinical complications were not identified.

As a result of step-by-step multiple regression analysis, a predictive model was constructed (R=0.53; F=8.50; p<0.ehz748.05257) and 3 independent predictors of ltVTA were revealed: HRT slope (TS: β=−0.36; p=0.002), QRS fragmentation (nQRSfr: β=0.32; p=0.001), and myocardial fibrosis (LGE%: β=0.27; p=0.02). As a result of ROC analysis following cut-off value were detected: fibrosis (LGE ≥22.5%; AUC 0.742; 95% CI: 0.594–0.889; sensitivity 77%; specificity 78%), pathological HRT (TS ≤2.5 ms/RR; AUC 0.771; 95% CI: 0.629–0.912; sensitivity 67%, specificity 87%), and fragmented QRS (frQRSn ≥5 leads; AUC 0.721; 95% CI: 0.559–0.883; sensitivity 80%, specificity 87%).

Conclusion

These markers (frQRSn along with LGE) reflect pathophysiological substrate triggering fatal arrhythmias; association of higher number of leads with frQRS, expressed LGE and pathological TS-HRT may be used as independent predictors of ltVTA for NCM pts
P1794Futility risk model development and validation among patients with aortic stenosis treated with transcatheter aortic valve replacement
Authors: Fauchier L , Bisson A , Herbert J , Lacour T , Ivanes F , Bourguignon T , Clerc J M , Quilliet L , Lantelme P , Angoulvant D , Babuty D , Guillon Grammatico L , Bernard A , Saint Etienne C
Published: October 2019

Risk-benefit assessment for transcatheter aortic valve replacement (TAVR) is still a matter of debate. A sizeable group of patients do not fully benefit from intervention despite a technically successful procedure. We therefore sought to identify patients with a bad outcome early after the procedure, and to develop a prediction model and calculator for identification of these patients.

Methods

Based on the French administrative hospital-discharge database, the study collected information for all consecutive patients with aortic stenosis treated with transfemoral TAVR in France between 2008 and 2018. Multivariate logistic regression was used to select the risk factors of early all-cause death in first year after TAVR procedure (considered as futility) for the overall population. Score points were assigned to each risk factor using the β coefficient. Internal validation was performed by a bootstrap method. Calibration was assessed with the Hosmer-Lemeshow goodness of fit test and accuracy with the C-statistic.

Results

A total of 47,872 patients with transfemoral TAVR were included in the analysis (mean age 83±7 years). Mean follow-up was 1.31±1.61 years and 9,338 deaths were recorded (yearly rate 14.9%), among which 4,562 (49%) occurred in first year after TAVR procedure. The final logistic regression model included older age, male sex, history of hospital stay with heart failure, history of acute pulmonary oedema, atrial fibrillation, previous stroke, vascular disease, diabetes, renal disease, liver disease, pulmonary disease, anemia, history of cancer, metastasis and denutrition,. The area under the curve (AUC) for the score was 0.696 (95% CI 0.688–0.704). This score outperformed frailty index and Charlson comorbidity index for identifying futility. AUC was 0.677 (95% CI 0.669–0.86) for internal validation. The Hosmer–Lemeshow goodness of fit test had a p-value of 0.10 suggesting that the model was accurate. We further divided the model into 4 groups with 5%, 12%, 19% and 30% futility, respectively. The low-risk group consisted of 60% of the patients and the high-risk group consisted of 4% of these patients.

Conclusion

This futility prediction score established from a large nationwide cohort of patients treated with TAVR may provide a relevant insight for optimizing healthcare decision. It may facilitate identification of patients who, despite an apparently successful procedure, have risk of death that may outweigh the benefit of an anticipated TAVR.
P1799Importance of the parietal band in normal subjects and in patients with repaired tetralogy of Fallot: the unknown muscle of the forgotten ventricle
Authors: Nedaei F , Vejlstrup N G , Madsen P L
Published: October 2019

Introduction

The parietal band (PB) is little acknowledged. The PB traverses the basal part of the right ventricle (RV) with myocardial fibres circumscribing the inlet portion of the RV, anatomical studies suggest the PB may be of significant importance for RV contraction. RV dysfunction is of particular concern in patients with repaired Tetralogy of Fallot (ToF pts.).

Purpose

To study the importance of the PB for RV function in a retrospective cohort study by comparison of normal subjects with ToF pts. with and without resected PB.

Methods

The PB function was compared to RV volume and function by echocardiography and magnetic resonance imaging (CMR) in 89 normal subjects (echo and CMR) and 106 ToF pts. (CMR).

Results

A PB was identified in all normal subjects. In these, the PB shortened by 41±5% (mean±SD) during systole, and correspondingly, the septum-to-free wall distance was shortened by 47±17%. In ToF pts., the PB had been resected in 57.5% of cases. Resection of the PB was not of any consequence for RV dilatation or pulmonary and tricuspid valve regurgitation fraction (all ns), but resection was associated with a lowered RV free-wall to inter-ventricular septum approximation (21±13% vs. 39±9%) and consequently a lowered RV ejection fraction (RVEF; 45±8% vs. 54±8%) (both p<0.001). 84% vs. 48% (p<0.0001) had reduced RVEF if the PB had been resected.

Conclusions

The two groups of ToF pts. may differ in other aspects than presence of the parietal band, but taken together our findings in normal subjects and ToF pts. do suggest that the parietal band is significant importance for right ventricle contraction.
P1800The benefit of atrial septal defect closure in patients with borderline shunt
Authors: Komar M , Przewlocki T , Sobien B , Prochownik P , Gancarczyk U , Libiszewska N , Olszowska M , Podolec P
Published: October 2019

Objective

Closure of atrial septal defect in patients with borderline shunt is controversial. The aim of the study was to evaluate the outcomes of transcatheter closure of secundum atrial septal defect (ASD) in the patients with the borderline shunt.

Methods

215 pts (126 F,89 M) with a mean age of 31.9±18.2 (16–58) years with a small ASD who underwent transcatheter closure were analyzed. All patients had an isolated secundum ASD with pulmonary to systemic blood flow Qp:Qs ratio≤1.5, mean 1.2±0.6 (1.1–1.5).

A symptom-limited treadmill exercise test with respiratory gas exchange analysis, transthoracic color Doppler echocardiographic study and Quality of life (QoL) (using the SF36 questionnaire (SF36q)) were repeated in all pts before and 36 months after the procedure.

Results

The device was successfully implanted in all pts (procedure time 19.1±5.2 (8–38) minutes, fluoroscopy time 8.1±4.4 (6–13) minutes). There were no major complications. The defect echo diameter was 7.7±3.9 (5 - 15) mm. The diameter of the implanted devices ranged from 6 to 18 mm.

After 36 months of ASD closure, all the pts showed a significant improvement of exercise capacity. 7 QoL parameters (except mental health) improved at 36 months of follow-up compared with baseline. The mean SF36q scale increased in 168 (78.1%) pts of mean 40.2±20.1 (9–72). The right ventricular dimension decreased in 173 pts (80.4%) (Table 1).

Table 1 Parameter Before ASD closure 36 months after ASD closure p value Time of exercise (min) 12.1±5.2 18.6±4.3 <0.001 VO2 peak (ml/kg/min) 10.2±4.7 14.8±4.2 <0.001 SF36q scale 0–100 37.3±22.3 79±29.5 <0.001 Right atrial area (cm2) 20.1±1.7 14.7±1.5 <0.001 Right ventricular area (cm2) 18.1±1.4 12.2±1.5 <0.001

Conclusions

ASD closure in patients with borderline shunt resulted in a significant and long-term clinical and hemodynamic improvement after percutaneous treatment.
P1807Manifestations and outcome of cardiac sarcoidosis - does gender matter?
Authors: Simonen P , Lehtonen J , Kupari M
Published: October 2019

Background

Sarcoidosis is characterized by the formation of inflammatory epithelioid-cell granulomas in various organs with cardiac involvement as its most ominous manifestation. A female preponderance in the prevalence of cardiac sarcoidosis (CS) is well known but other possible gender differences remain poorly studied.

Purpose

We set out to evaluate gender-related differences in the manifestations and long-term outcome of CS.

Methods

We reviewed the history, diagnostic procedures, details of treatment and outcome of 158 consecutive patients with histologically confirmed CS diagnosis between 1988 and 2017 at our hospital. Follow-up data were collected up to the end of 2018.

Results

The study population consisted of 51 men and 107 women (68%). At presentation, men were younger than women (mean age 47 years vs 51 years, p=0.045) and had more often a history of pre-existing extracardiac sarcoidosis (25% vs 10%, p=0.013). Isolated CS remained less common in men even after the complete diagnostic process (50% vs 75%, p=0.001). The main presenting CS manifestations were atrioventricular block, ventricular tachyarrhythmias and heart failure in 39%, 30% and 18% of men vs in 54%, 23% and 17% of women, respectively (p=0.183). Left ventricular ejection fraction at presentation averaged 49±11% in men and 49±13% in women (p=0.845). Troponin T was elevated more often in men at the presentation (46% vs 26%, p=0.024). At magnetic resonance imaging, pathological myocardial late gadolinium enhancement was observed in 87% of men and 84% of women (p=0.615). Myocardial “hot spot” at 18-F fluorodeoxyglucose positron emission tomography was also equally common (87% in men, 92% in women, p=0.468). An intracardiac cardioverter-defibrillator was implanted in 78% of men and 75% of women (p=0.693) and nearly all patients (99%, no gender difference) received immunosuppressive therapy. During the mean follow-up of 64 months, 10 of 51 men versus 30 of 107 women either died of a cardiac cause, suffered an aborted sudden cardiac death or underwent transplantation. The composite event-free survival did not differ between genders (Figure 1. Log-rank p=0.852).

Conclusions

Two thirds of CS patients are women. At disease presentation, women are older than men and their sarcoidosis is more often isolated to the heart but the clinical manifestations, diagnostic findings and long-term outcome are comparable in the two genders.
P1813Impact of cardiac MRI imaging on detection rates of myocarditis
Authors: Morgenstern A , Mikolich B , Morgenstern D , Mikolich J R
Published: October 2019

Background

The Lake Louise Criteria for the diagnosis of myocarditis by cardiac MRI (CMR) was published in 2009 (JACC April 2009; 53(17): 1475–1487) utilizing T2 STIR, “early” gadolinium enhancement (EGE) and late gadolinium enhancement (LGE). In 2012, our CMR unit adopted a chest pain protocol to enhance detection of inflammatory heart disease, using all 3 pulse sequences. In 2017, T1 relaxing mapping and T2 relaxation mapping were added to the chest pain protocol.

Purpose

This study was designed to assess the impact of pulse sequence changes in the CMR chest pain protocol on the detection rate of myocarditis.

Methods

An institutional cardiac imaging database was queried for all patients with chest pain who underwent a CMR study from 2009 thru 2018. The number of newly diagnosed cases of myocarditis per year was computed, based on the prevailing CMR diagnostic criteria at that time. The detection rate of new myocarditis cases per year, was calculated by dividing the number of new cases by the total of CMR studies performed for evaluation of chest pain, during each calendar year.

Results

Of the 4,946 patients in the cardiac imaging database, 2,126 patients underwent CMR imaging for complaints of chest pain. Detection rates of myocarditis by year are shown in Figure 1. Prior to inclusion of T1 STIR pulse sequences, CMR detection of myocarditis was nearly nil. Use of T2 STIR imaging allowed for an increased rate of myocarditis detection. However, addition of T1 mapping and T2 mapping in 2017 resulted in another increase in myocarditis detection rates from baseline.

Figure 1. Detection rate of myocarditis.

Conclusions

Detection rates of myocarditis using CMR were improved by using T2 STIR pulse sequences, per the initial Lake Louise Criteria. Further enhancement of myocarditis detection rates is achieved by addition of T1 and T2 mapping. These CMR impact data provide additional support for the adoption of the recently modified Lake Louise Criteria (JACC Dec 2018; 72(24): 3158–3176).
P1824Aortic wall shear stress in bicuspid aortic valve disease - 10-year follow-up
Authors: Hanigk M , Burgstaller E , Latus H , Shehu N , Zimmermann J , Martinoff S , Hennemuth A , Ewert P , Stern H , Meierhofer C
Published: October 2019

Introduction

Bicuspid aortic valve (BAV) disease leads to deviant complex helical flow patterns in the aorta - especially in the mid-ascending (AoA) part. In association with congenital BAV, aortic wall alterations such as aortic dilatation and dissection may occur. Among others, wall shear stress (WSS) could be one parameter to contribute to the prediction of the long-term outcome of patients with BAV. 4D-flow in cardiovascular magnetic resonance has been established as a valid method to estimate WSS.

Purpose

The aim of this study is to reevaluate WSS and comparing it to values generated in the same patient cohort with bicuspid aortic valve disease in 2008. No one of the above had aortic dilation in 2008 but proven helical flow pattern. The long term follow-up study might show changes in WSS over the period of ten years.

Methods

Ten complete 4D flow datasets of patients (age at follow-up: median 34.5 years; range 19–41 years) with bicuspid aortic valve disease without enlargement of the aorta were obtained in 2008 and reevaluated in 2018/2019 in the same patient collective. Mean WSS values were calculated with identical specific software tools. All data were analyzed by two experienced investigators.

Results

Aortic diameters at the level of the mid AoA did not change significantly in the 10-year period. The WSS values were lower in 2018 at all levels of the ascending aorta (Table 1). Indexed aortic diameters at the level of the mid ascending aorta did not change, median difference 0.06 cm/m2 (range −0.1 cm/m2 to 0.2 cm/m2; p=0.28), absolute values of indexed AoA diameters in 2018/2019 ranged from 1.27 cm/m2 to 2.2 cm/m2 (median 1.76 cm/m2).

Table 1 WSS magnitudinal [N/m2] 2008 (n=10) 2018 (n=10) Median difference p-value median range median range Level aortic bulb 0.95 0.80–1.46 0.42 0.34–0.82 −0.53 <0.01 Level mid ascending aorta 0.72 0.40–0.98 0.39 0.34–0.59 −0.33 <0.01 Level brachiocephalic trunc (BCT) 0.71 0.38–1.03 0.40 0.37–0.61 −0.31 <0.01

Conclusions

Indexed AoA diameters in BAV disease did not change significantly over a 10-year period. WSS of AoA was less compared to values generated in 2008. This might be explained by a slight alteration in hemodynamic flow patterns by the aging aorta, but not by changes of the aortic diameters. Possibly a drop of WSS in BAV could serve as a marker for a benign long term course.
P1825The characteristics of type A acute aortic dissection in patients with out-of-hospital cardiopulmonary arrest analyzed by computed tomography
Authors: Takeuchi S , Yoshino H , Yamaguchi Y , Soejima K
Published: October 2019

Background/Introduction

Acute aortic dissection (AAD) is a life-threatening condition associated with high morbidity and mortality rates, and is reported to have a 1–2% fatality rate per hour after onset. Therefore, many cases with AAD are considered to be included in patients with out-of-hospital cardiopulmonary arrest (OHCPA). However at this time, there is a lack of documentation of these cases.

Purpose

In this study, we investigated the characteristics of Stanford type A AAD (TAAD) cases between OHCPA cases and survival cases using acute phase CT.

Methods

An acute phase CT scanning was performed on consecutive patients with OHCPA transported to our hospital in order to estimate the causes of cardiopulmonary arrest (CPA). Based on our emergency outpatient databases, we retrospectively reviewed the acute phase CT findings of OHCPA TAAD cases and survival TAAD cases diagnosed at our hospital during the same period.

Results

Of 1,011 consecutive patients with OHCPA, except for exogenous death from 1 January 2015 to 31 December 2017, 934 patients underwent CT examination in the acute phase. Based on the clinical course and CT findings, 71 of the 934 patients (7.6%) were diagnosed with AAD. Out of 71 AAD patients, 66 were TAAD patients, and all of these patients were enrolled in the OHCPA group. On the other hand, 41 patients were registered as the survival group during the same period. These patients were diagnosed as TAAD and were admitted to our hospital without falling into CPA. Increased Age (78±8 years old vs 69±13 years old, P<0.01), visible intimal flap (63.6% vs 41.5%, P=0.043), bloody pericardial effusion (72.7% vs 26.8%, P<0.01), hemothorax (24.2% vs 0%, P<0.01) were significant in patients with the OHCPA group compared with the survival group. In addition, 56 patients with OHCPA (84.8%) had at least one of massive bloody pericardial effusion or massive hemothorax. Using multivariate logistic regression analysis, increased age and bloody pericardial effusion were both found to be independent factors related to OHCPA.

Conclusion

In this study, it is thought that the main characteristics of the CT findings with OHCPA due to TAAD are a rupture of the aorta into the pericardial cavity or thoracic cavity.