bluuebunny/crossref_metadata_2025 huggingface dataset:
The effect of underweight on mid-term outcome following transcatheter aortic valve implantation: an insight from multicenter registry
Authors: Tezuka T , Higuchi R , Hagiya K , Saji M , Takamisawa I , Shimizu J , Iguchi N , Takanashi S , Doi S , Okazaki S , Sato K , Tamura H , Takayama M
Published: November 2020

Background

Obesity has the adverse prognostic impact in the general population, whereas paradoxical effect of obesity has been reported in patients with heart failure. Several studies have suggested the same obesity paradox in patients undergoing transcatheter aortic valve implantation (TAVI), however, they included limited number of underweight patients.

Purpose

The aim of this study was to clarify the effect of underweight on outcome following TAVI.

Methods

We retrospectively analyzed consecutive 1,027 patients undergoing TAVI between April 2010 and June 2019. The patients were categorized according to body mass index (BMI) as follows: underweight (<18.5 kg/m2, n=150), normal weight (18.5 to 25 kg/m2, n=657), and overweight (>25 kg/m2, n=220). BMI was defined as body weight (kg) divided by the square of body height (m) measured at the hospital admission. We compared the short- and mid-term outcome after TAVI among three groups, and all clinical events were accordance with Valve Academic Research Consortium-2 criteria.

Results

Underweight patients were more often female, and had a higher prevalence of hypertension, dyslipidemia, peripheral artery disease, anemia, and hypoalbuminemia. They also presented lower ejection fraction, smaller aortic valve area, and higher surgical risk score. In procedural findings, device unsuccess and major vascular complication more occurred in underweight patients, but 30-day mortality was equivalent among three groups. The mid-term survival of the underweight was inferior to the other two groups (figure).In the multivariate analysis, female (hazard ratio [HR] 0.52, 95% confidence interval [CI] 0.37–0.73, P=0.0002), atrial fibrillation (HR 2.22, 95% CI 1.56–3.17, P<0.0001), albumin value (HR 0.37 per 1-g/dl increase, 95% CI 0.25–0.55, P<0.0001), Society of Thoracic Surgeons score (HR 1.06 per 1% increase, 95% CI 1.02–1.06, P=0.0039), 30-day combined endpoint (HR 2.12, 95% CI 1.33–3.38, P=0.0017), and underweight (HR 1.59, 95% CI 1.04–2.37, P=0.026) were associated with the survival after TAVI.

Conclusion

The underweight had a worse mid-term prognosis, representing the obesity paradox in the TAVI population.

Kaplan-Meier curves

Funding Acknowledgement

Type of funding source: None
MitraClip therapy in patients with acute severe mitral regurgitation
Authors: Turyan Medvedovsky A , Tahiroglu I , Tonchev I , Planer D , Danenberg H.D , Beeri R , Shuvy M
Published: November 2020

Background

There is a lack of data to support the optimal management of high-risk patients with acute severe mitral regurgitation (MR). The role of the MitraClip implantation in treatment of acute severe mitral regurgitation (MR) is undetermined.

Methods

We screened all patients who underwent MitraClip implantation at the Hadassah Medical Center between October 2015 and December 2019. We evaluated immediate, 30-day and 1-year outcomes after the procedure. We evaluated patients with severe MR due to ruptured cord because of degenerative disease and acute-on-chronic functional MR due to ischemic (after a recent myocardial infarction (MI)) or non-ischemic etiology (secondary to decompensated HF).

Results

From a cohort of 151 patients, who underwent MitraClip implantation in our center, we identified 35 patients (23.2%) with acute severe mitral regurgitation (4+) and decompensated refractory heart failure. Patients' mean age was 74.15 years, 66.9% - were males. One, 2, or 3 clips were implanted. Reduction of MR from 4+ to 1+ was achieved in 34 patients (66.7%). Twenty-eight patients (80%) had acute-on-chronic severe MR and refractory heart failure including 6 cases after recent MI, other 7 patients presented with heart failure and acute severe MR secondary to ruptured cord due to degenerative disease. After MitraClip implantation, reduction of MR severity was achieved in all patients. Seven patients were withdrawn from intravenous therapy and intra-aortic balloon pump 2–3 days after the procedure. Four patients died during hospitalization, three of them due to sepsis. 30-day follow-up showed improvement of NYHA functional class and a tendency toward improvement in left ventricle systolic function with signs of reverse remodeling. Nevertheless, we observed high 30-day and 1-year mortality rate (11.4 and 23.8% respectively).

Conclusions

MitraClip therapy could be an alternative option for treatment of patients with acute and acute-on-chronic severe MR of ischemic and non-ischemic etiology.

Funding Acknowledgement

Type of funding source: None
Early intervention or watchful waiting for asymptomatic severe aortic stenosis: a systematic review and meta-analysis
Authors: Ullah W.U , Gowda S.G , Khan M.S.K , Sattar Y.S , Al-Khadra Y.A , Rashid M.R , Mohamed M.O.M , Alkhouli M.A , Kapadia S.K , Bagur R.B , Mamas M.A.M , Alraies M.C.A
Published: November 2020

Background

The management of patients with severe but asymptomatic aortic stenosis (AS) is a challenge of daily practice. Evidence on early aortic valve replacement (AVR) versus symptom-driven approach is controversial.

Methods

Electronic databases were searched and articles comparing early-AVR with conservative management for severe asymptomatic AS were identified. The pooled unadjusted odds ratio (OR) was computed using a random-effects model.

Results

A total of 10 studies (observational=9, and randomized clinical trials=1) consisting of 5,291 patients were included. On a mean follow up duration of 4 years, the odds of early-AVR vs conservative management were in favour for early intervention in terms of cardiovascular mortality (OR 0.26, 95% CI 0.13–0.53, p=0.0002, figure-1) and all-cause mortality (OR 0.27, 95% CI: 0.17–0.41, p≤00001). The number needed to treat (NNT) by performing early AVR (either SAVR or TAVR) to prevent one death secondary to either cardiovascular or all-cause was 8 (95% CI: 6–10), and 5 (95% CI, 4.2–5.4) respectively. The odds of all-cause mortality in a selected patient population undergoing surgical AVR (SAVR) (OR 0.17, 95% CI 0.10–0.29, p≤0.00001) or transcatheter AVR (TAVR) (OR 0.45, 95% CI 0.31–0.64, p≤0.0001) were significantly lower compared to patients who were managed conservatively. A subgroup sensitivity analysis based on severe vs. very severe AS and early vs. symptom based AVR also mirrored the findings of overall results.

Conclusions

Patients with asymptomatic aortic valve stenosis have lower odds of all-cause and cardiovascular mortality when managed with early-AVR compared to conservative management.

Figure 1

Funding Acknowledgement

Type of funding source: None
The usefulness of cardiac computed tomography in the diagnosis of perivalvular complications in patients with prosthetic valve endocarditis
Authors: Zatorska K , Hryniewiecki T , Zakrzewski D , Nieznanska M , Kusmierczyk M , Michalowska I
Published: November 2020

Background and purpose of the study

Echocardiography remains the gold standard for the diagnosis of prosthetic valve endocarditis (PVE). But in around 15% of cases, its results can be false negative due to the acoustic shadow of artificial valves. The aim of the study was compare cardiac computed tomography (CT) with other imaging modalities in the diagnosis of perivalvular complications in patients with PVE.

Material and methods

The study included 35 consecutive patients with PVE. Thirty of them had an artificial aortic valve (17- mechanical valve, 13-biological valve), 7-mechanical mitral valve, and one patient was after biological pulmonary valve implantation. Three patients were after aortic and mitral valve replacement. Each patient underwent transthoracic (TTE) and transesophageal (TEE) echocardiography. ECG-gated CT examinations were performed with a dual source CT system. All patients were qualified for surgical treatment. The assessment included the presence of vegetation, perivalvular abscess/pseudoaneurysm, inflammatory infiltration and prosthesis dehiscence.

Results

Intraoperative assessment revealed the presence of vegetations in 16 patients. The sensitivity of echocardiography (TTE+TEE) and CT examinations was 100% and 93% respectively. Twenty one abscesses/pseudoaneurysms were found intraoperatively. The sensitivity of echocardiography and CT examinations was 76% and 85%, respectively. The analysis of total TTE, TEE and CT findings showed that supplementing echocardiography with CT had increased the sensitivity of the method for detecting abscess/pseudoaneurysms to 95%. In a patient in whom no abscesse was found, inflammatory infiltration was diagnosed in echocardiography. Inflammatory infiltration was diagnosed intraoperatively in 13 patients. The sensitivity of echocardiography and CT was 69% in both examinations. The sensitivity of the combination TTE + TEE + CT was 92%. Perivalvular leakage was found intraoperatively in 17 patients. The sensitivity of echocardiography and CT for the diagnosis of this complication was 100% and 87%, respectively.

Conclusions

CT is better than echocardiography in diagnosing abscesses/pseudoaneurysms and has the same sensitivity in detecting inflammatory infiltration. Adding CT to echocardiography improves the sensitivity of these complications detection. CT is not superior to echocardiography in the diagnosis of vegetations and perivalvular leakage, but it can be a useful tool when echocardiography findings are inconclusive.

Funding Acknowledgement

Type of funding source: None
Diagnostic yield of Electroanatomic voltage mapping in guiding Endomyocardial biopsies; a comparison with an MRI-guided approach
Authors: Bergonti M , Dello Russo A , Gasperetti A , Catto V , Vettor G , Ribatti V , Dessanai M.A , Mustaq S , Conte E , Sommariva E , Andreini D , Basso C , Natale A , Tondo C , Casella M
Published: November 2020

Background

Electroanatomic voltage mapping (EVM) is a promising modality for guiding Endomyocardial biopsies (EMB). Previous experiences on this techniques have reported safety and feasibility of this approach. These reports however, resulted limited by sample size or imperfect designs, preventing reliable comparisons of the effectiveness of this new methods with a conventional or a cardiac magnetic resonance (CMR) imaging guided approach.

Aim

We now report the largest cohort of patients undergoing EVM-guided EMB in order to show its diagnostic yield and comparing it with a cardiac magnetic resonance (CMR) guided approach.

Methods

One-hundred and sixty-two consecutive patients undergoing EMB at our Institution from 2010 to 2019 were included. Pathological areas identified at EVM and CMR underwent EMB. According to EMB results, CMR and EVM sensitivity and specificity regarding the identification of pathological substrates of myocardium were evaluated.

Results

A gadolinium-enhanced CMR had been performed in 143 (88.9%) of the population and yielded pathological findings in 121 (85.8%) of such cases. Late gadolinium enhancement (LGE) was present in 94 (70%) of the patients, while EVM identified areas of low voltages in 61%. Right (73%), left (19%) or both ventricles (8%) underwent sampling. EVM proved to have similar sensitivity to CMR (74% vs. 77%; P=0.479), with non-significantly higher specificity (70% vs. 47% P=0.738). In 12 patients with EMB-proven cardiomyopathy, EVM identified pathological areas, which had been undetected at CMR evaluation (concordance rate 53.8%; k = 0.26). Sensitivity of pooled EVM and CMR was as high as 95%. Five cases (3,8%) of cardiomyopathies were undetected by both CMR and EVM. Complications rate was low (4,9%), mostly vascular access related, with no patients requiring urgent management.

Conclusion

EVM proved to be a promising tool for targeted-EMB due to its sensitivity and specificity in identifying myocardial pathological substrates. EVM demonstrated to have an accuracy similar to CMR. EVM and CMR together conferred EMB a positive predictive value of 89%.

Funding Acknowledgement

Type of funding source: None
The clinical atlas of cardiomyopathies: data from the prospective DZHK TORCH study
Authors: Sedaghat-Hamedani F , Trebing J , Kindermann A , Kayvanpour E , Tan K , Preussler S , Sluka S , Gi W.T , Dieterich C , Weitmann K , Eschenhagen T , Hoffmann W , Katus H.A , Meder B
Published: November 2020

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)
Lymphocytic myocarditis: a genetically predisposed disease?
Authors: Artico J , Merlo M , Delcaro G , Cannata A , Gentile P , De Angelis G , Paldino A , Bussani R , Dal Ferro M , Sinagra G
Published: November 2020

Background

Clinical presentation of myocarditis is extremely heterogeneous from asymptomatic to overt severe heart failure (HF). A complex interaction between pre-existing genetic background and inflammation might be responsible for this heterogeneity.

Purpose

The aim of the present study was to investigate whether positive genetic background for pathogenic cardiomyopathy-related variants might underlie a higher susceptibility to left ventricular dysfunction in patients with active lymphocytic myocarditis.

Methods

We prospectively performed genetic tests in 36 patients (46±15 years; 61% males; no relatives included) with biopsy-proven active lymphocytic myocarditis according to Dallas criteria and immunohistochemistry. Only pathogenic (P) or likely pathogenic (LP) variants were considered.

Results

After genetic test, 31% of patients (n=11) were carriers of P/LP truncating variants in structural Cardiomyopathy related genes: Titin (TTN, n=8, 73%), Desmoplakin (DSP, n=1), Filamin C (FLNC, n=1) and RNA binding protein 20 (RBM20, n=1). Among the 27 patients presenting with HF and LV dysfunction, the positive genetic yield was similar to the total cohort (n=9, 34%; 90% with TTN). Two out of six arrhythmic patients (30%) were carriers in arrhythmogenic genes (i.e. DSP and FLNC), whereas no patients with infarct-like presentation were carriers. During follow-up, 44% of patients (n=16) presented normal Left Ventricular Ejection Fraction (LVEF). Carriers had a lower rate of LVEF normalization compared to non-carriers (18% vs 56%, respectively; p=0.035).

Conclusion

Positive genetic testing for cardiomyopathy-related-genes might be found in a non-negligible percentage of patients with biopsy-proven myocarditis, especially if presenting with heart failure and LV dysfunction. Compared to non-carriers, carriers of P/LP variants show lower likelihood of LVEF normalization during follow-up.

Funding Acknowledgement

Type of funding source: None
Myocardial work analysis in left ventricular non-compaction, and its association with cardiovascular outcomes
Authors: Anwer S , Heiniger P.S , Rebellius L , Schoenenberger-Berzins R , Kuzo N , Guastafierro F , Erhart L , Gregr P , Schmid D , Kindler C , Gruner C , Tanner F.C
Published: November 2020

Introduction

Left ventricular (LV) non-compaction (LVNC) is a rare cardiomyopathy characterised by a two-layered LV myocardium with prominent trabeculae separated by deep recesses perfused from the LV cavity. Myocardial work analysis (MWA) is a novel echocardiographic method that calculates pressure-strain loops from longitudinal strain and bedside blood pressure measurement. Our study investigates the MWA features of LVNC patients and their association with outcomes.

Methods

We compared 40 LVNC with preserved LVEF (≥50%) (pEF-LVNC) and 40 LVNC patients with reduced LVEF (<50%) (rEF-LVNC) to 40 healthy matched controls. GE EchoPAC® (Version 203) was used for echocardiographic analysis. A combined endpoint was defined as heart failure progression, sustained ventricular tachycardia, thromboembolic accidents, and atrial fibrillation.

Results

Clinical characteristics, conventional echocardiography, and strain analysis results are detailed in Table 1 and Figure 1. Myocardial work index was significantly reduced in rEF-LVNC (1088 [138–1211 mm.Hg%]) and pEF-LVNC (1394 [1138–1501 mm.Hg%]) in comparison to control (1827 [1758–1981 mm.Hg%]), but there was no difference between the two LVNC groups. Global constructive work (GCW) was significantly lower in rEF-LVNC (1094 [947–1281 mm.Hg%]) than in pEF-LVNC (1730 [1368–1691 mm.Hg%]) (p=0.001), while both LVNC groups were lower than control (2201 [1965–2406 mm.Hg%]) (pEF-LVNC p=0.001, rEF-LVNC p<0.0001). Global wasted work (GWW) was significantly lower in rEF-LVNC (204 [181–231 mm.Hg%]) than in pEF-LVNC (154 [115–171 mm.Hg%], p<0.0001) and control (61 [45–98 mm.Hg%], p<0.0001). Global work efficiency (GWE) was significantly lower in rEF-LVNC (18.3 [15.4–19.6%], p=0.001 than in pEF-LVNC and control. In both pEF-LVNC and rEF-LVNC, impaired GWE and GCS were the parameters most significantly associated with increased risk of cardiovascular events as detailed in Figure 2.

Conclusion

MWA is a promising parameter for risk assessment of LVNC patients especially because it is less load-dependent and, unlike LVEF, incorporates left ventricular haemodynamics.

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): 2018 research grant from the Swiss Heart Foundation
Is alcohol septal ablation in women performed too late?
Authors: Batzner A , Aicha D , Seggewiss H
Published: November 2020

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.

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
Predictors of sudden cardiac death and ventricular tachyarrhythmias in patients with hypertrophic cardiomyopathy
Authors: Rineiska N , Zakharava E , Komissarova S , Haidel I
Published: November 2020

Background

Determining additional predictors that allow more accurate identification of patients who need primary prevention of sudden cardiac death (SCD) and ventricular tachyarrhythmias (VT) in patients with hypertrophic cardiomyopathy (HCM) is one of the most important tasks. High-risk patients can be more accurately identified using modern non-invasive research methods.

Purpose

Identification of new risk predictors for SCD and ventricular tachyarrhythmias based on cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and T1-mapping in patients with hypertrophic cardiomyopathy.

Materials and methods

Clinical data of 98 HCM subjects (58 males and 40 females, median age 46.5 [35.2; 54.7] treated in our centre in the period between 2013 and 2017 have been studied. All patients underwent CMR with LGE and T1-mapping.

Results

According to the existing ESC-2014 scale, 45 (46%) of the 98 patients included in the study had a low risk, an intermediate risk was identified in 26 (26%) patients and a high risk of SCD in 27 (28%). During the follow-up period 16 episodes of sudden cardiac death were recorded. Of these, only 8 (50%) patients had a high risk on the ESC-2014 scale. High-risk patients show significantly greater myocardial fibrosis (Me 28.5%; [21.9; 44.1]) compared with patients with intermediate (Me 17.6% [8.0; 22.5]) and low risk of SCD (Me 11.7%; [5.8; 17.6]), p<0.001. A threshold level of fibrosis volume associated with an adverse outcome was determined, which was 15% (based on determining the maximum rank of statistics). For patients with 15% fibrosis volume, the cumulative survival rate on the Kaplan-Mayer curve over the observation period was 96% (95% CI 88.6–100), while for patients with fibrosis volume ≥15% - 72.4% (95% CI 60.6–86.4). The study showed that in 15 (93.7%) patients out of 16 who had an adverse outcome, the volume of myocardial fibrosis was ≥15%. Episodes of nonsustained ventricular tachycardia were detected in 48 patients (48.7%) according to daily ECG monitoring. To identify patients with ventricular tachyarrhythmias by ROC analysis, the threshold value of extracellular myocardial volume was determined using CMR with LGE and T1 mapping, which was 32.5% (sensitivity 74% and specificity 86%). According to multivariate analysis, one of the independent predictors of ventricular tachyarrhythmias was the level of extracellular myocardial volume ≥32.5% (HR 1.2; 95% CI 1.03–1.4).

Conclusion

Predictors detected by CMR with LGE and T1 mapping can identify patients with HCM with a high risk of sudden cardiac death and ventricular tachyarrhythmias.

Funding Acknowledgement

Type of funding source: None
The late-onset dilated cardiomyopathy
Authors: Cannata A , Merlo M , Manca P , Dal Ferro M , Paldino A , Artico J , Gentile P , Jirikowic J , Todd E , Salcedo E , Graw S , McDonagh T , Taylor M , Mestroni L , Sinagra G
Published: November 2020

Background

Dilated Cardiomyopathy (DCM) represents a specific subgroup of non-ischemic cardiomyopathies. Little is known about the genotypic characterization of dilated cardiomyopathy (DCM) patients diagnosed over 60 years of age.

Aim

To investigate prevalence, characterization and prognostic impact of the genetic background of late-onset DCM patients.

Methods

We analyzed a study population of 566 DCM patients from two international referral centers. Genetic background was analyzed and patients were grouped into typical-onset DCM (<60 years of age at diagnosis) or late-onset DCM (>60 years of age at diagnosis).

Results

Approximately 12% of patients (n=70) had late-onset DCM and female sex was significantly more frequent in the late-onset DCM cohort (p<0.001). Diagnostic yield of genetic testing was comparable between typical- and late-onset DCM (53% vs 50%, respectively p=0.438) whereas the prevalence of Titin gene truncation variants (TTNtv) was higher in the late-onset DCM group compared to the younger cohort (23% vs 13% respectively; p<0.05). Notably, patients with late-onset genetic DCM had comparable long-term outcomes to those with typical-onset DCM.

Conclusions

Late-onset DCM patients have nearly double the rate of TTNtv mutations and are more likely to be female compared to younger DCM patients. These observed differences in mutational makeup and sex may reveal insights into age and sex dependent mechanisms for TTNtv and should prompt further study. Notably, the increased prevalence of TTNtv and female sex did not translate into noticeable differences in rates of measurable cardiac events.

Funding Acknowledgement

Type of funding source: None
Myocarditis and arrhythmogenic right ventricular cardiomyopathy: a diagnostic challenge
Authors: Bergonti M , Dello Russo A , Catto V , Gasperetti A , Sicuso R , Vettor G , Ribatti V , Tundo F , Moltrasio M , Sommariva E , Andreini D , Basso C , Natale A , Tondo C , Casella M
Published: November 2020

Background

Current arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnostic criteria are mostly based on ventricle function and dimension. Previous studies have reported a significant overlap between ARVC and chronic myocarditis, at non-invasive assessment.

Purpose

Tto compare biopsy-proven ARVC and myocarditis patients, in order to identify clinical, imaging and invasive electroanatomic voltage mapping (EVM) differences between the two groups.

Methods

Patients with borderline diagnosis of ARVC or suspected myocarditis underwent compete assessment with cardiac magnetic resonance (CMR). All patients underwent endomyocardial biopsy (EMB) with targeted tissue sampling guided by EVM. All patients with an histological diagnosis of myocarditis or ARVC were included.

Results

83 patients were included, divided into 35 (42.2%) ARVC and 48 (57.8%) myocarditis. Among ARVC patients, 25 (71.4%) had right dominant ARVC, 5 (14.3%) left dominant patter and 5 (14.3%) bi-ventricular involvement. Nine patients (23.1%) with suspected clinic diagnosis of ARVC before EMB, received and histological diagnosis of myocarditis. Two (5.7%) patients with suspected myocarditis were proven to have ARVC. When comparing patients with ARVC and patients with myocarditis, univariate analysis showed that age, sex, family history, arrhythmic disorders at presentation and ECG abnormalities were similar between the two groups (P>0.05 for all the variables). There was also no significant difference with regards to bi-ventricular function and dimension at CMR evaluation. More patients with myocarditis resulted positive at late gadolinium enhancement (LGE) evaluation, although non-significantly (P=0.082). Oedema was more frequently present in patients with myocarditis (P=0.01), while adipose tissue infiltration and segmental wall motion abnormalities were more often observed in patients with ARVC (P=0.002 and P<0.001 respectively). At EVM analysis, a significant greater number of patients had a pathological uni- and bi-polar EVM (P<0.05 in all cases) and the scar-area was greater in patients with ARVC: 18.8 vs 11.0 cmq (P=0.041).

Conclusion(s)

A significant number of patients who received a clinical diagnosis of Myocarditis or ARVC according to current guidelines, were subsequently reclassified after histological analysis. Patients with ARVC and myocarditis were not distinguishable on the basis of clinical features and ventricular function and dimensions. Conversely, tissue analysis with CMR demonstrated how patient with ARVC had less oedema, more adipose tissue infiltration and had more extensive scar at EVM evaluation.

Funding Acknowledgement

Type of funding source: None
The myocardial phenotype of Fabry disease pre-hypertrophy and pre-detectable storage
Authors: Bicho Augusto J.A , Johner N , Shah D , Nordin S , Knott K , Lau C , Alfarih M , Seraphim A , Vijapurapu R , Ramaswami U , Steeds R , Kozor R , Hughes D , Moon J , Namdar M
Published: November 2020

Background

Cardiac involvement in Fabry Disease (FD) occurs prior to left ventricular hypertrophy (LVH) and is characterized by low myocardial native T1 with storage reflected by cardiovascular magnetic resonance (CMR) and ECG changes.

Objectives

We hypothesize that a pre-storage myocardial phenotype might occur even earlier, prior to T1 lowering.

Methods

FD patients and age, sex and heart rate matched healthy controls underwent same-day ECG with advanced analysis and multiparametric CMR (cines, global longitudinal strain [GLS], T1 and T2 mapping, stress perfusion [myocardial blood flow, MBF] and late gadolinium enhancement [LGE]).

Results

114 Fabry patients (46±13 years, 61% female) and 76 controls (49±15 years, 50% female) were included. In pre-LVH FD (n=72, 63%), a low T1 (n=32/72, 44%) was associated with a constellation of ECG and functional abnormalities compared to normal T1 FD patients and controls. However, pre-LVH FD with normal T1 (n=40/72, 56%) also had abnormalities compared to controls: reduced GLS (−18±2 vs −20±2%, P<0.001), microvascular changes (lower MBF 2.5±0.7 vs 3.0±0.8mL/g/min, P=0.028), subtle T2 elevation (50±4 vs 48±2ms, p=0.027) and limited LGE (%LGE 0.3±1.1 vs 0%, P=0.004). ECG abnormalities included shorter P wave duration (88±12 vs 94±15ms, P=0.010) and T wave peak time (Tonset–Tpeak; 104±28 vs 115±20ms, P=0.015), resulting in a more symmetric T wave with lower T wave time ratio (Tonset–Tpeak)/(Tpeak–Tend) (1.5±0.4 vs 1.8±0.4, P<0.001) compared to controls.

Conclusions

FD has a measurable myocardial phenotype pre-LVH and pre-detectable myocyte storage with microvascular dysfunction, subtly impaired GLS and altered atrial depolarization and ventricular repolarization intervals.

Proposed stages of cardiac involvement

Funding Acknowledgement

Type of funding source: None
Electrocardiographic differences between Anderson-Fabry and sarcomeric hypertrophic cardiomyopathy and correlation with cardiac magnetic resonance
Authors: Vitale G , Di Nicola F , Tanini I , Camporeale A , Graziani F , Ditaranto R , Zanoni R , Ferrara V , Lombardi M , Olivotto I , Rapezzi C , Galie N , Biagini E
Published: November 2020

Background

Differential diagnosis between Anderson-Fabry (AF) and sarcomeric hypertrophic cardiomyopathy (HCM) is often very challenging particularly in AF patients with late onset cardiac involvement.

Purpose

To gain new insights from standard electrocardiogram (ECG) in AF disease for differential diagnosis from sarcomeric HCM. Additionally, to better understand ECG features in AF patients, a correlation substudy ECG-cardiac magnetic resonance (CMR) has been performed.

Methods

From 162 patients with definite diagnosis of AF disease, 111 [65 males, median age 57 (51–67) years] with pathologic left ventricular hypertrophy (LVH) (Group A) were compared with 111 sarcomeric HCM patients (Group B) sex, age and maximal wall thickness matched by 1:1 propensity score.

Results

AF patients showed shorter PR interval [155 (140–180) vs 163 (149–184) msec; p=0.005) and wider QRS interval [110 (100–134) vs 100 (90–106) msec; p<0.0001). Additionally AF patients had a higher prevalence of complete (22% vs 3%; p<0.0001) and incomplete (13% vs 1%; p<0.0001) right bundle branch block (RBBB) and a higher percentage of ST segment depression (12% vs 1%; p=0.001) and inferior negative T waves (34% vs 19%; p=0.01). No differences in terms of Sokolow-Lyon and Cornell scores were found whereas total QRS score was higher in Group A [20 (16–27) vs 18 [14–22] mV; p=0.0004). Low QRS voltages and inferior Q waves were not present in AF patients. Among the 69 AF patients who underwent MRI, the 44 with late gadolinium enhancement (LGE) were older [59 (52–66) vs 53 (40–59) years; p=0.017] and had more frequently negative T waves on ECG, particularly in the inferior leads (64% vs 8%; p<0.0001), compared to the 25 without LGE. At multivariate analysis, age and negative T waves were independently associated to the presence of LGE on CMR.

Conclusions

Compared to matched sarcomeric HCM, AF patients had a shorter PR, wider QRS and a higher percentage of RBBB in relation to to the different aetiology (storage vs “pure” hypertrophy). The higher total QRS score and the absence of inferior Q waves could reflect the more frequent concentric distribution of LVH. Additionally negative T waves, especially in inferior leads, are related to the presence of LGE on CMR (often in the postero-lateral wall).

Funding Acknowledgement

Type of funding source: None
Differences of ST level at J-point on 12-lead electrocardiography can distinguish takotsubo syndrome and acute anterior myocardial infarction
Authors: Shimizu M , Cho S , Hara K , Ohmori M , Kaneda T , Tateishi R , Yamakami Y , Shimada H , Manno T , Isshiki A , Kimura S , Fujii H , Suzuki M , Nishizaki M , Sasano T
Published: November 2020

Background

Qualitative difference of ST elevation/depression on 12-leads electrocardiography (ECG) at onset was reported in patients with Takotsubo syndrome (TTS) and acute anterior myocardial infarction (ant AMI). However, quantitative difference of those was not elucidated.

Purpose

To investigate differences of ST level at J point on ECG between TTS and ant AMI by automated calculating system.

Methods

We firstly enrolled consecutive 40 patients of TTS, and among 500 ant AMI patients, one to two random matching was done by their age and gender. Finally, 40+80 patients (74.5±11.2 years, 87 females) were enrolled. ECG at onset of both group was measured by automated system (ECAPs12c: Nihon-Koden).

Results

ST level of TTS at J-point in I/II/V4–6 lead was significantly elevated comparing to that of ant AMI. Conversely, Conversely, significant ST depression in aVR and no ST elevation in V1 of TTS was observed in TTS. Logistic regression analysis revealed that ST elevation in I lead and no ST elevation in V1 lead showed high odds ratio and low P value.

Conclusion

Automated measured ST level at J-point was a powerful tool to distinguish TTS and ant AMI at onset.

Funding Acknowledgement

Type of funding source: None
Prognostic value of acute mitral insufficiency among patients admitted with Takotsubo syndrome. Preliminary data from a prospective multicenter registry
Authors: Mallardi A , Santoro F , Leopizzi A , Vitale E , Zimotti T , Ieva R , Caldarola P , Iacoviello M , Cannone M , Brunetti N.D
Published: November 2020

Background

Takotsubo syndrome (TTS) is an acute and reversible left ventricular dysfunction, whose pathophysiological mechanisms are not completely known. Aim of the study is to evaluate the incidence and prognostic value of acute mitral insufficiency among TTS patients.

Methods

Three hundred sixteen consecutive patients admitted for TTS were enrolled from July 2007 to December 2019 in a prospective registry among four hospitals. Clinical features, laboratory data, ECG and echocardiographic parameters and in-hospital complications were recorded.

Results

Eighty-five (27%) patients, mean age 76±8 years, presented with moderate/severe mitral insufficiency (msMI). Compared to the control group, patients that presented with msMI were older (76±8 vs 71±12 years, p<0.01), had lower LVEF (34±7% vs 37±9% p=0.02) and experienced higher rate of in-hospital complications (42 vs 32% p=0.04).

The incidence of in-hospital adverse events was higher in case of both msMI and LVEF <35% than in one of the two previous conditions or neither (69%, 42%, 23% respectively, p<0.05). Differences remained statistically significant even after correction for age and gender in the multivariate analysis.

There was a reduction of mitral insufficiency from moderate/severe into mild in 30 out of 87 (34%) pts after 48h from admission and in 52 out of 87 pts at discharge (60%).

Left ventricular outflow tract obstruction (LVOTO) was found in 13 out of 87 (15%) pts with msMI at admission and was transient in 11 out of 13 pts (84%).

At long term follow up (650 days) patients with msIM when compared to those without, experienced higher rate of mayor cardiovascular events (31.7% vs 20% p=0.03).

Conclusions

Patients with TTS and moderate/severe mitral insufficiency at admission have an increased risk of in and out-of hospital mayor cardiac adverse events.

Funding Acknowledgement

Type of funding source: None
Acute pericarditis: who should stay and who should go?
Authors: Ferreira J , Luis M , Baptista R , Monteiro S , Goncalves L
Published: November 2020

Background

The European Society of Cardiology (ESC) guidelines for the diagnosis and management of pericardial diseases identify predictive factors of poor prognosis and advise either in favour or against hospitalisation accordingly. Our aim is to evaluate the adequacy of hospitalisation criteria in a cohort of patients presenting to the emergency department (ED) with acute pericarditis.

Methods

Retrospective analysis of patients admitted in ED with acute pericarditis, from 2009 to 2019. All patients were evaluated by a cardiologist during ED stay who decided if the patient was to be discharged or hospitalized. Hospitalized and discharged patients were compared regarding primary outcome, defined by a composite of: need for pericardiocentesis and/or cardiac surgery, pericarditis recurrence and all-cause death. Clinical decision was then counterpoised with ESC guidelines.

Results

A total of 192 patients were included in the analysis (aged 46±18.5 years-old, 83.3% male) of which 87 (45.5%) were hospitalized. A total of 25% registered the primary outcome, mainly due to acute pericarditis recurrence, occurring in 21.9%. Predictors of recurrence were: glucocorticoid therapy (OR=11.93, 95% CI 3.13–45.5, p<0.001), fever at admission (OR=2.67, 95% CI 1.29–5.49, p=0.008), immunosuppression (OR=4.03, 95% CI 1.280–12.659, p=0.017) and increased cardiothoracic index (OR 3.85, CI 95% 1.67–8.86, p=0.002). Regarding hospitalisation/discharge decision, the ESC guidelines were respected in 72.9% of the cases. However, no significant difference in primary outcome was noted whether the ESC guidelines were respected or not (27.5% vs. 24.3%, p=0.707).

Conclusions

Discrepancy between current guidelines and clinical decision did not translate into a different outcome.

Funding Acknowledgement

Type of funding source: None
Analysis of risk factors associated with recurrence of acute pericarditis in patients diagnosed in the emergency room
Authors: Izquierdo Ribas M , Martinez-Nadal G , Cepas P , Aldea A , Matas A , Lopez-Sobrino T , Lopez-Barbeito B , Andrea R , Miro O
Published: November 2020

Background

Around 5% of patients consulting to the emergency room (ER) for non-ischemic thoracic pain are diagnosed of acute pericarditis (AP). The good prognosis of this pathology is well known, with a mortality of 1% and a low incidence of serious complications, which has led the research to focus on recurrences. Female sex, corticoid treatment and treatment adherence are related with higher risk of recurrence. Colchicine has been associated with less recurrences.

Purpose

To analyse the factors associated with recurrence after the diagnosis of AP in the ER of a third-level hospital.

Methods

Retrospective review of ER consultations oriented as AP, prospectively documented during 10 years (2008–2018). In 2019, a follow up was done in order to identify the recurrences and to search for associated factors (univariate and multivariate analysis).

Results

610 patients were diagnosed of AP, 175 (29%) recurrences were documented. Factors associated with an increased risk of recurrence were: previous AP, immunosuppression or history of autoimmune disease, fever or increased acute-phase reactants (CRP; ESR), hospitalization and corticoid treatment. Factors associated with less risk of recurrence were: age, non-steroidal anti-inflammatory drug (NSAID) treatment and idiopatic/viral etiology. No association with sex or colchicine treatment was identified. Multivariate analysis identified 3 factors that were independently associated with the risk of recurrence in a direct way: previous history of AP, [OR (IC95%): 2.09 (1.11–3.92)]; increased CRP [OR (IC95%): 1.09 (1.03–1.15)]; hospitalization [OR (IC95%): 2.65 (1.07–6.58)]. 2 factors were inversely associated with the risk of recurrence: age [OR (IC95%): 0.98 (0.96–0.99)]; NSAID treatment [OR (IC95%): 0.56 (0.32–0.97)].

Conclusions

29% of the patients were readmitted to the ER for an AP recurrence. Previous AP, increased CRP and the need of hospitalization were associated with a higher risk of recurrence. Age and NSAID treatment, on the other hand, were associated with less risk of recurrence.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Ajuts per la Recerca Josep Font
Syncope in relation to pulmonary arterial obstructive burden, hemodynamic status and short- and long-term outcome in patients with acute pulmonary embolism
Authors: Keskin B , Tokgoz H.C , Akbal O.Y , Hakgor A , Tanyeri S , Karagoz A , Turkday S , Kulahcioglu S , Tanboga I.H , Dogan C , Bayram Z , Ozdemir N , Kaymaz C
Published: November 2020

Background and aims

Although syncope (S) has been reported as one of the presenting findings in patients (pts) with acute pulmonary embolism (APE), its clinical and haemodynamic correlates and impacts on the long-term outcome in this setting remains to be determined. In this single-centre study we evaluated the clinical and haemodynamic significance of S in APE in initial asessment, and during short- and long-term follow-up period.

Methods

Our study was based on the retrospective and prospective analysis of the overall 641 pts (age 65 (51–74 IQR) yrs, 56.2% female) with diagnosis of documented APE who underwent anticoagulant (n=207), thrombolytic (n=164), utrasound-facilitated thrombolysis (UFT) (n=218) or rheolytic thrombectomy (RT) (n=52). The systematic work- up including multidetector computed tomography (MDCT), Echo, biomarkers, and PE severity indexes were performed in all pts, and Qanadli score (QS) was used as the measure of the thrombotic burden in the pulmonary arteries (PA).

Results

The S as the presenting symptom In 30.2% of pts with APE. At baseline assessment, S(+) vs S(−) APE subgroups had a significantly shorter symptom-diagnosis interval, a higher risk status according to the significant elevations in troponin T, D-dimer, the higher PE severity indexes, a more deteriorated right ventricle/left ventricle ratio (RV/LV r), right atrial/left atrial ratio (LA/RAr) and RV longitudinal function indexes including tricuspid annular planary excursion (TAPSE) and tissue velocity (St), a significantly higher PA obstructive burden as assessed by QS and PA pressures. Thrombolytic therapy (36.2% vs 21%, p<0.001) and RT (11.9% vs 6.47%, p=0.037) were more frequently utilized S(+) as compared to S(−) group. However, all these differences between two subgroups were found to disappear after evidence-based APE treatments. In-hospital mortality (IHM) (12.95% vs 6%, p=0.007) and minor bleeding (10.36% vs 2.9%, p<0.001) were significantly higher in S(+) pts as compared to those in S(−) subgroup. Binominal logistic regression analysis revealed that PESI score and RV/LVr independently associated with S while IHM was only predicted by age and heart rate. The COX proportional hazard method showed that RV/LVr at discharge and malignancy were independently associated with cumulative mortality during follow-up duration of 620 (200–1170 IQ) days.

Conclusions

The presence of S in pts with APE was found to be asociated with a higher PA obstructive burden, a more deteriorated RV function and haemodynamics and higher risk status which may need more agressive reperfusion treatments. However, in the presence of the optimal treatments, S did not predict neither in-hospital outcome, nor long-term mortality.

Funding Acknowledgement

Type of funding source: None