bluuebunny/crossref_metadata_2025 huggingface dataset:
Predictors of increased mortality in untreated moderate aortic stenosis
Authors: Gupta A , Liu T , Pounds C , Sharma R P , Yong C , Krumholz H M , Leon M B
Published: October 2021

Background

The natural history of native valvular moderate aortic stenosis (AS) is poorly understood.

Purpose

To examine the prognosis of patients with native valvular moderate AS, and identify high risk-features associated with increased mortality.

Methods

Natural language processing of electronic health records in the claims-linked Optum® database (2011–2018) from over 200 hospitals and 7000 clinics in the United States identified severity of AS (mild/moderate/severe) from echocardiograms and physician notes of patients more than 65 years old. Our cohort included 30,204 patients (3594 with mild, 9938 with moderate, and 16672 with severe AS). We used competing risk Kaplan-Meier analysis to compare 5-year mortality (obtained from the Social Security Death Masterfile) between the different AS severity groups adjusted for demographics and comorbidities, censoring for aortic valve replacement. The relationship between AS severity and survival was examined during median 1049 days (interquartile range: 597 to 1652 days) of follow-up. We performed Fine-Gray modeling to examine risk factors associated with long-term mortality in patients with moderate AS.

Results

A higher proportion of comorbidities like atrial fibrillation, diabetes, and coronary artery disease were observed with increasing severity of AS (Figure 1). After adjustment, compared with mild AS (5-year mortality: 31.6%), patients with untreated moderate (5-year mortality: 40.0%, HR 1.34, 95% CI 1.25 – 1.44) and untreated severe AS (5-year mortality: 51.1%, HR 1.88, 95% CI 1.75– 2.01) have increasing risk of long-term mortality (Figure 2). Significant predictors of 5-year mortality in patients with moderate AS include older age (HR 1.38, 95% CI 1.29 –1.47), male sex (HR 1.21, 95% CI 1.15 – 1.27), heart failure (HR 1.37, 95% CI 1.29 – 1.44), coronary artery disease (HR 1.20, 95% CI 1.13 – 1.27), and atrial fibrillation (HR 1.08, 95% CI 1.02 – 1.14). In a subcohort of 5189 patients for whom left ventricular ejection fraction (LVEF) information was available, reduced LVEF was associated with worse long-term mortality (LVEF<40%: HR 1.17, 95% CI 1.05 –1.31; LVEF 40–50%: HR 1.08, 95% CI 0.97 –1.20).

Conclusion

Our findings suggest that moderate AS is associated with poor long-term survival with higher risk associated with LVEF <40%, and comorbidities including heart failure, coronary artery disease and atrial fibrillation. Whether patients with moderate AS, particularly with high-risk features, benefit from treatment remains unknown.

Funding Acknowledgement

Type of funding sources: None. Baseline CharacteristicsAdjusted KM curves with Competing Risk
Differential characteristics associated with progression of mitral and aortic regurgitation in patients undergoing kidney transplantation
Authors: Kim M J , Kim D R , Lee J H , Seo J W , Cho I S , Huh K H , Hong G R , Ha J W , Shim C Y
Published: October 2021

Background

Heart valve regurgitation is common in patients with end-stage renal disease (ESRD). However, there are no data on the fate of mitral regurgitation (MR) and aortic regurgitation (AR) after kidney transplantation (KT). In this study, we sought to investigate regression or progression rates of MR and AR after KT in patients with ESRD. Moreover, we aimed to explore clinical and echocardiographic factors associated with the progression of MR and AR in patients undergoing KT.

Methods

Among 1,734 patients who underwent KT from 2005 to 2018 at a single tertiary hospital, 674 patients (407 men; mean 48±12 years) who underwent both pre- and post-KT echocardiography were analyzed comprehensively. Pre-KT echocardiography was performed within three months of KT, and post-KT echocardiography was done between 6 months and 24 months after KT. Severities of MR and AR were graded as no/trivial, mild, moderate, and severe according to the current guidelines. Regression was defined if the severity decreased by one or more grades, while progression was defined if the severity increased by one or more grades.

Results

Figure 1 shows the regression or progression of MR and AR after KT. 78 (11%) patients showed MR regression, but 41 (6%) experienced MR progression. 13 (2%) revealed AR regression, while 23 (4%) presented AR progression. In patients with MR progression, there were more cases of receiving a second KT, having mitral annular calcification, and showing lesser reduction of left atrial volume after KT. Patients with AR progression showed a longer hemodialysis duration, persistent hypertension after KT, and aortic root dilatation. Factors related to the progression of MR and AR showed statistically meaningful predictive values in a stepwise manner (Figure 2)

Conclusions

In patients undergoing KT, MR and AR may progress in patients with certain distinct characteristics. Different clinical and echocardiographic characteristics before KT, and reduction of hemodynamic loads after KT determine the progression of MR and AR. Further echocardiographic surveillances after KT are needed in patients with clinical and echocardiographic factors for progression of valve regurgitation.

Funding Acknowledgement

Type of funding sources: None.
Prognostic utility of global longitudinal strain (GLS) in patients with severe primary mitral regurgitation undergoing mitral valve surgery: a systematic review and meta-analysis
Authors: Agustin R D , Ramiro V R , Villanueva D L , Tiongson M D , Ramos J D , Ramirez-Ragasa R , Magno J D , Punzalan F E
Published: October 2021

Background

Mitral valve (MV) surgery is recommended for the treatment of chronic severe mitral regurgitation (MR). However, the optimal timing of surgery in patients with asymptomatic severe primary MR remains elusive. Global longitudinal strain (GLS) may provide additional prognostic value in predicting postoperative outcomes in patients with chronic severe primary mitral regurgitation (MR) undergoing MV surgery.

Purpose

We aimed to determine the prognostic value of GLS in predicting postoperative mortality and LV dysfunction among patients with chronic severe primary MR.

Methods

Systematic search of Pubmed, Cochrane library, Clinicaltrials.gov, and Herdin.ph from inception to July 2019, using the terms “mitral insufficiency”, “mitral regurgitation”, “mitral valve surgery”, and “global longitudinal strain” was done without language restriction. We included and extracted data from cohort studies of patients with chronic severe primary MR who underwent MV surgery and which GLS, mortality and ejection fraction. We used the Newcastle Ottawa Scale to assess the quality of included studies. Review Manager 5.3 was used to perform analysis. Forest plots with summary hazard ratios (HR) and odds ratios (OR) with 95% confidence intervals (CI), I2 test for heterogeneity, and funnel plots were reported.

Results

Our search yielded 12 cohort studies with 2,843 patients; 7 prospective and 5 retrospective studies were included in the qualitative synthesis with 11 good quality studies. Cut-off GLS values ranged from −21.7% to −18.1%. In terms of postoperative all-cause mortality, the summary HR for worse versus better preoperative GLS is 1.22 (95% CI 1.04–1.44, p value <0.ehab724.16031, I2 95%). In terms of postoperative LV dysfunction, the summary OR is 1.74 (95% CI 1.14–2.66, p value 0.01, I2 94%) with significant heterogeneity.

Conclusion

Left ventricular GLS has prognostic value in terms of predicting postoperative mortality and LV dysfunction. However, significant heterogeneity exists between studies. Larger studies with well-defined inclusion criteria need to be performed and standardized GLS cut-offs need to be determined.

Funding Acknowledgement

Type of funding sources: None. GLS in Predicting All-Cause MortalityGLS in Predicting Post-op Dysfunction
Regression of electrocardiographic left ventricular hypertrophy after transcatheter aortic valve replacement for aortic stenosis
Authors: Tobe A , Tanaka A , Tokuda Y , Akita S , Fujii T , Miki Y , Furusawa K , Ishii H , Usui A , Murohara T
Published: October 2021

Background

The changes in electrocardiographic left ventricular hypertrophy (ECG-LVH) after transcatheter aortic valve replacement (TAVR) are not fully elucidated.

Methods

This retrospective single-center study investigated 156 patients who underwent TAVR for severe aortic stenosis. Twelve-leads ECGs obtained before and at 2 days and 1, 6 and 12 months after TAVR were analyzed. Patients with an implanted pacemaker, with a bundle branch block before or after TAVR or whose electrocardiogram at any measuring point was not available were excluded. As an assessment of ECG-LVH, Sokolow-Lyon voltage, Cornell voltage, Cornell product and Peguero–Lo Presti voltage were evaluated.

Results

Finally, 64 patients were included. Sokolow-Lyon voltage, Cornell voltage, Cornell product and Peguero–Lo Presti voltage significantly decreased between 1 and 6 months after TAVR compared to baseline. The prevalence of ECG-LVH criteria for each parameter decreased as well. QRS duration significantly decreased at 6 months after TAVR and the number of patients with strain pattern decreased significantly at 1 month when compared to baseline. When the patients were divided into two groups, those with and without ECG-LVH at baseline, ECG-LVH parameter values decreased significantly, especially in patients with ECG-LVH at baseline.

Conclusion

Regression of ECG-LVH after TAVR was demonstrated as per various criteria of ECG-LVH. The voltages and prevalence of ECG-LVH decreased significantly between 1 and 6 months after TAVR.

Funding Acknowledgement

Type of funding sources: None.
Incidental findings on computed tomography for preoperative assessment before transcatheter aortic valve implantation in japanese patients
Authors: Tobe A , Tanaka A , Tokuda Y , Akita S , Fujii T , Miki Y , Furusawa K , Ishii H , Usui A , Murohara T
Published: October 2021

Background

Extra-cardiovascular incidental findings (IFs) on preoperative computed tomography (CT) are frequently observed in transcatheter aortic valve implantation (TAVI) candidates. However, the backgrounds of TAVI candidates and comorbidities differ based on the race and/or country, and data on IFs in a specific population are not always applicable to another.

Objective

To assess the prevalence, type, and clinical impact of IFs in Japanese TAVI candidates.

Methods

This was a retrospective, single-center, observational study. CT reports of 257 TAVI candidates were reviewed, and IFs were classified as (a) insignificant: findings that did not require further investigation, treatment, or follow-up; (b) intermediate: findings that needed to be followed up or were considered for further investigation but did not affect the planning of TAVI; and (c) significant: findings that required further investigation immediately or affected the planning of TAVI.

Results

At least one IF was found in 254 patients (98.8%). Insignificant, intermediate, and significant IFs were found in 253 (98.4%), 153 (59.5%), and 34 (13.2%) patients, respectively. Newly indicated significant IFs were found in 19 patients (7.4%). In 2 patients (0.8%), TAVI was canceled because of significant IFs. In patients who consequently underwent TAVI, the presence of significant IFs was not associated with the duration from CT performance to TAVI [28 (19–40) days vs. 27 (19–43) days, p=0.74] and all-cause mortality during the median follow-up period of 413 (223–805) days (p=0.44).

Conclusion

Almost all Japanese TAVI candidates had at least one IF, and the prevalence of significant IFs was not negligible. Although the presence of significant IFs was not associated with mid-term mortality, appropriate management of IFs was considered important.

Funding Acknowledgement

Type of funding sources: None.
Cerebral protection in TAVR – can we do without? Impact on stroke rate, length of hospital stay and 12-month mortality
Authors: Dona C , Koschutnik M , Nitsche C , Winter M P , Mach M , Andreas M , Bartko P , Kammerlander A , Goliasch G , Lang I , Hengstenberg C , Mascherbauer J
Published: October 2021

Stroke associated with transcatheter aortic valve replacement (TAVR) is a potentially devastating complication. Until recently, the Sentinel™ Cerebral Protection System (CPS; Boston Scientific) has been the only commercially available device for mechanical prevention of TAVR-related stroke. However, its effectiveness is still undetermined.

Methods

Between January 2019 and August 2020 consecutive patients were randomly assigned to TAVR with or without Sentinel™ in a 1:1 fashion. We defined as primary endpoint clinically detectable cerebrovascular events within 72 hours after TAVR, and as secondary endpoints LOS and 12-month mortality. Logistic and linear regression analyses were used to assess associations of Sentinel™ use with endpoints.

Results

Of 411 patients (80±7 y/o, 47.4% female, EuroSCORE II 6.3±5.9%), Sentinel™ was used in 213 (51.8%), with both filters correctly deployed in 189 (46.0%). 20 (4.9%) cerebrovascular events were recorded, 10 (2.4%) of which were disabling strokes. Sentinel™ reduced cerebrovascular events in univariate analysis by 71% (OR 0.29, 95% CI 0.11–0.82; p=0.02) and after multivariate adjustment by 75% (adj. OR 0.25; 95% CI 0.08–0.80; p=0.02). Sentinel™ use was also significantly associated with shorter LOS (8.4±9.6 versus 6.7±6.1 days; p=0.03) and lower 12-month all-cause mortality (15.7% versus 7.5%, p=0.01).

Conclusions

In the present prospective all-comers TAVR cohort, Sentinel™ significantly 1) reduced cerebrovascular events, 2) shortened LOS, and 3) improved 12-month survival. These data promote the use of a CPS when implanting TAVR valves.

Funding Acknowledgement

Type of funding sources: None.
Midterm outcomes after aortic valve neocuspidalization with autologous pericardium. Single center experience
Authors: Semagin A P , Zybin A A , Karpushkina E M , Shubin E I
Published: October 2021

Objective

The aim of the study was to analyze the midterm results of the aortic valve neocuspidalization with autologous pericardium.

Methods

From August 2017 through October 2018, 40 patients were treated with aortic valve neocuspidalization using autologous pericardium, their medical records were retrospectively reviewed.

Results

There were 40 patients (19 males, 21 females) with aortic stenosis, 17 patients had bicuspid valve. The median age was 66 (26–77) years. Preoperative echocardiography revealed a peak pressure gradient had mean value of 84.5±23.32 mm Hg, and mean pressure gradient average of 48.2±16.4 mm Hg. Surgical annular diameter was 21.2±2.16 mm. Body mass index was 29.7±3.4 kg / m2. Severe patient-prosthesis mismatch was predicted in 18 cases, moderate – in 14 cases (Hancock II prosthesis was used as a reference). Intraoperative control of neocuspidalized valve (coaptation, pressure gradient) was performed with transesophageal echocardiography. There was no conversion to a prosthetic valve replacement. There were no in-hospital mortalities. Postoperative echocardiography revealed a peak pressure gradient mean value of 16.3±5.3 mm Hg, and mean pressure gradient average of 8.5±3.1 mm Hg on the 5th postoperative day, the mean height of coaptation was 12.3±3.1 mm. Mean observation period was 17 months. During the observation period 4 (10%) patients died from non-surgery related causes. 1-year after surgery echocardiography revealed a peak pressure gradient mean value of 14.5±5.0 mm Hg, and mean pressure gradient average of 9.2±4.0 mm Hg, mild-to-moderate (grade 2) aortic insufficiency was identified in two patients. Reoperation was required in one case – patient was found to have severe aortic regurgitation due to rupture of the commissural suture.

Conclusion

Our short-term and mid-term results demonstrate satisfactory neovalves' hemodynamic performance. The simplicity and reproducibility of aortic valve reconstruction with autologous pericardium may make this method an option for surgical treatment of aortic valve diseases.

Funding Acknowledgement

Type of funding sources: None.
Stroke volume index and outcomes in low gradient severe aortic stenosis
Authors: Snir A , Ng M K , Stewart S , Playford D , Strange G , Celermajer D S
Published: October 2021

Background

Conventionally, stroke volume index (SVI) ≤35ml/m2 is considered as “low-flow” in severe aortic stenosis (AS). Recent evidence suggests a lower threshold SVI (30ml/m2) may be more prognostically informative.

Objectives

To assess the association between SVI and survival in patients with low-gradient severe AS, with either preserved (LVEF ≥50%) or reduced (LVEF<50%) ejection fraction.

Methods

Data were derived from the National Echocardiography Database of Australia (NEDA). Of 192,060 adults (aged 62.8±17.8 years) with comprehensive aortic valve profiling between 2000–2019, 12,013 (6.3%) had severe AS. Based on standard echocardiographic parameters, 1,623 patients had severe low-gradient AS with preserved LVEF and 744 patients had severe low-gradient AS with reduced LVEF. All-cause and cardiovascular-related mortality were assessed for each low-gradient group on an adjusted basis (age, sex, BMI, AVA indexed and RVSP), according to SVI. Mean follow-up was 81±42 months.

Results

Compared to patients with “normal” flow (SVI >35ml/m2), those with “low-flow” (SVI ≤35ml/m2) had significantly higher BMI, smaller AVA index and lower AV mean gradient and peak velocity (p≤0.001). In low-gradient with preserved LVEF patients, adjusted survival at 1- and 3-years was significantly lower only for SVI ≤30ml/m2 compared with SVI>35ml/m2 (p<0.001 and p<0.03, respectively). In low-gradient with reduced LVEF patients, adjusted survival at 1- and 3-years was significantly lower for SVI ≤35ml/m2 compared with SVI>35ml/m2 (p=0.015 and p=0.018, respectively).

Conclusions

Taken together with previous data, our results suggest that a SVI threshold of ≤30ml/m2 (rather than ≤35 ml/m2) is prognostically significant in severe low-gradient AS with preserved LVEF.

Funding Acknowledgement

Type of funding sources: None. Survival plots LGAS with preserved EFSummary of results
Impact of catheter ablation on functional tricuspid regurgitation in patients with atrial fibrillation
Authors: Nishiwaki S , Watanabe S , Yoneda F , Tanaka M , Komasa A , Yoshizawa T , Kojitani H , Shizuta S , Morimoto T , Kimura T
Published: October 2021

Background

Since atrial functional tricuspid regurgitation (AF-TR) is associated with increased heart failure and mortality, the management of AF-TR is clinically important. Atrial fibrillation (AF) plays the main role in AF-TR. However, the effectiveness of catheter ablation (CA) and mechanism of improvement of AF-TR haven't been fully evaluated.

Purpose

We sought to investigate the impact of CA for AF on AF-TR in patients with moderate or more TR.

Methods

We retrospectively investigated consecutive 2685 patients with AF who received CA from February 2004 to December 2019 in Japan. The current study population consisted of 102 patients with moderate or greater TR who underwent CA for AF. The echocardiographic parameters were compared between pre-ablation and post-ablation transthoracic echocardiography (TTE), and the recurrence rate of AF/ atrial tachycardia (AT) was measured.

Results

The mean age was 73.2 years, 53% were women. TR severity and TR jet area significantly improved after CA for AF (TR jet area: 5.8 [3.9–7.6] cm2 to 2.0 [1.1–3.0] cm2, p<0.001). In addition, mitral regurgitation (MR) jet area, left atrial (LA) area, mitral valve diameter, right ventricular (RV) end-diastolic area, right atrial (RA) area, tricuspid valve (TV) diameter decreased after CA (p<0.001, <0.001, <0.001, = 0.02, <0.001, and <0.001, respectively). There was no significant difference between one-year recurrence of AF/AT and TR severity at pre-ablation TTE (moderate 28.6%, moderate to severe 37.2%, and severe 31.6%, p=0.72).

Conclusions

TR severity and jet area improved after CA in patients with AF and moderate or more TR. RV size, RA size, TV diameter also decreased after CA, which may be associated with TR improvement. There was no significant difference between one-year recurrence of AF/AT and TR severity at pre-ablation TTE.

Funding Acknowledgement

Type of funding sources: None.
Evolving trends in infective endocarditis over three decades in a developing country
Authors: Maaroufi A , Zahidi H , Abdulhakeem M , Arous S , Benouna E G , Drighil A , Azzouzi L , Habbal R
Published: October 2021

Introduction

Infective endocarditis (IE) is a rare serious disease for which diagnosis and treatment and prophylaxis continue to develop.

Purpose

The aim of our work were to analyze evolution in its epidemiologic, clinical, and microbiologic characteristics, as well as the outcomes.

Methods

This study compare data from a retrospective study conducted by Bennis and Al based on 157 cases of infectious endocarditis admitted in tertiary care Hospital between January 1983 and December 1994, theses cases are referred as Group A; and 103 cases of infective endocarditis according to Duke criteria admitted to the same center three decades afterwards from december 2013 to January 2020, which are referred as Group B.

Results

The mean age of the patients has became older over decades, with a mean of 27.5 years (11–65 years) in the group A versus 39.2 years [13–84] in the group B, with preservation of the male predominance (62.8% in group A vs 62.1% in group B).

Infectious endocarditis secondary to rheumatic valvular heart disease has significantly decreased from 63.% of patients in group A to 27.5% in group B.

Mitral or mitro-aortic valve involvement stayed predominant in both groups with a increase of mechanical prosthetic valve involvement with 9.7% in group B.

A portal of entry of the infection was identified in 63% of patients in group A versus 41% in group B, we noted a significant decrease of dental-related cases from 64% in group A to 29% in group B, and a predominance of invasive procedure/devices cases in group B with 36% of identified cases.

In group A Blood cultures were positive in 42% of cases with a predominance of Staphylocci (30%) and coagulase-negative Staphylococci (25.7% of cases) whilst in group B blood cultures were positive in 30% of cases with a predominance of Staphylococci (36.6%) and Streptococci (20% of cases).

Echocardiography as a useful diagnostic tool demonstrated specific lesions of infectious endocarditis (abcess or vegetations) in 73.2% of cases in group A and 82% in group B. The clinical course in group A was complicated by congetive heart failure CHF (47.8%) or neurological lesions (11.5%) while in group B CHF was noted in 35% of cases and 8% of neurological lesions and The global mortality went from 28.7% in group A to 15% in group B related mostly to cardiogenic shock.

Conclusion

Our work demonstrated changes in the epidemiologic characteristics of IE that parallel changes in demographic and risk factors. The progress of diagnosis and treatement options in developing countries explains the better prognosis for this condition today.

Funding Acknowledgement

Type of funding sources: None.
Prognostic value of myocardial injury biomarker in COVID-19: a systematic review and meta-analysis
Authors: Chen S , Marcella E , Tandiono J , Heriyanto R , Jonatan C , Kurniawan A
Published: October 2021

Background

An accurate prognostic tool that can help with risk stratification at first admission is crucial in COVID-19 patients, as severe complications and mortality rate can be reduced with prompt treatment and close follow up. The myocardial injury biomarker, Troponin, is reported to be a potential predictor of mortality in COVID-19 cases.

Purpose

This study aims to investigate the utility of cardiac troponin level as a prognosis tool in COVID-19 patients.

Methods

On March 12th, 2021, data was collected from PMC, PubMed, Google Scholar, Science Direct, and Scopus, using combination of keywords associated with myocardial injury biomarker, Troponin, and its prognostic value for mortality in COVID-19 patients. Included publications are original clinical trials, limited to those with English manuscripts, and all were reviewed and evaluated by 5 authors to minimise bias. Inclusion criteria for studies are original clinical trials with a study population of adults (≥18 years old) patients with confirmed SARS-CoV-2 infection through PCR. The quality of each studies included was assessed using the Newcastle-Ottawa Scale (NOS) and data synthesis and statistical analysis were carried out using Review Manager software.

Results

A total of 19 studies consisting of 13,789 COVID-19 patients were included. All 19 studies were good quality according to the NOS. Studies found that myocardial injury, indicated by the elevation of its biomarker, Troponin, is a strong predictor of in-hospital mortality. Furthermore, elevated troponin level itself is an independent marker of mortality, regardless of its concomitant cardiac disease. Meta-analysis of 9 included studies revealed a significant association between elevated cardiac troponin levels and mortality (HR 4.13; 95% CI 3.42 - 5.00; P<0.001). Based on GRADE, the result of this systematic review and meta-analysis proved to be moderate in quality as there are no inconsistency and variability in results and there are no publication biases.

Conclusion

Cardiac troponin level is found to be a strong predictor of mortality in COVID-19 patients and therefore should be treated both as an evaluation for cardiac involvement and as an important prognostic marker. Patients with elevated troponin levels within admission should be closely monitored and given prompt treatment to decrease mortality.

Funding Acknowledgement

Type of funding sources: None. Forest plot figure
Clinical characterisation of hypertrophic cardiomyopathy caused by MYH7 gene variants in children
Authors: Kadirrajah V , Acquaah V , Norrish G , Field E , Dady K , Cervi E , Kaski J P
Published: October 2021

Background

Variants in the cardiac Beta Myosin Heavy chain 7 gene (MYH7) are a common cause of hypertrophic cardiomyopathy (HCM) in adults, but their role in paediatric-onset HCM has not been systematically characterised. This study aims to describe the presentation, clinical characteristics and outcomes of childhood HCM secondary to disease-causing MYH7 variants.

Methods

Retrospective, longitudinal, data from 70 individuals meeting diagnostic criteria for HCM under the age of 18 years with disease-causing MYH7 variants from a single specialist centre (1991–2019) were collected. A Major Adverse Cardiac Event was defined as sudden cardiac death (SCD), heart failure-related death, cardiac transplantation, haemodynamically-compromising sustained ventricular arrhythmia or appropriate implantable cardioverter defibrillator (ICD) therapy.

Results

Median age at diagnosis was 9.2 years (IQR 4.2–13.3 years); 47 patients (67.1%) were less than 12 years and 7 (10.0%) were under the age of 1 at diagnosis. Twenty-two patients (31.4%) were probands. MYH7 variants were missense (n=67) or truncating (n=1). Reason for presentation were: family screening (n=45, 64.3%); cardiac symptoms (n=12, 17.1%); incidental finding (n=11, 15.7%); and out of hospital cardiac arrest (n=2, 2.9%). At baseline, mean maximum left ventricular wall thickness (MLVWT) z-score was 9.6 (±5.8), 11 patients (15.7%) had resting left ventricular outflow tract obstruction (left ventricular outflow tract gradient ≥30mmHg). Baseline phenotype did not significantly differ between probands and non-probands (MLVWT Z score 11.9 (±4.5) vs 8.5 (±6.1), p-value 0.0675). Over a median follow up of 3.6 years (IQR 1.8–7.9 years), 10 patients (14.3%) underwent a left ventricular septal myectomy at a median age 6.4 years (IQR 3.4–12.1 years) and 27 (38.6%) had an implantable cardioverter defibrillator (ICD) for primary (n=24, 34.3%) or secondary (n=3, 4.3%) prevention. Three patients (4.3%) died (SCD, n=1; heart-failure related, n=1; non-cardiac, n=1) and 3 (4.3%) underwent cardiac transplantation. Ten patients (14.3%) experienced a MACE. Patients who experienced a MACE were more likely to be probands [n= 6 (60.0%) vs n=16 (26.7%); p=0.036] but did not differ in terms of baseline phenotype (p=0.134).

Conclusion

MYH7 variants can cause infantile and childhood-onset disease, which is associated with significant early cardiac morbidity and mortality. Adverse outcomes were more common in those presenting as probands.

Funding Acknowledgement

Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Medical Research Council, Great Ormond Street Hospital charity.
Analysis of characteristics and prognostic impact of phenotypes in hereditary ATTR
Authors: Sguazzotti M , Caponetti A G , Saturi G , Ponziani A , Massa P , Dal Passo B , Accietto A , Longhi S , Bonfiglioli R , Mattana F , Guaraldi P , Cortelli P , Galie N , Biagini E , Gagliardi C
Published: October 2021

Background

Hereditary transthyretin-related amyloidosis (h-ATTR) is a systemic infiltrative disease caused by a single amino acid mutation on the transthyretin (TTR) gene, which destabilizes the protein and can determine its deposition on multiple organs, including heart and peripheral nervous system.

Purpose

We aimed to characterize and compare clinical, instrumental, and prognostic features of patients affected by h-ATTR by dividing the population into the disease's main phenotypes (unaffected carriers, cardiac, neurological or mixed phenotype).

Methods

Two hundred and eighty-five subjects of a single-centre cohort with a recognized pathogenic mutation on TTR gene were retrospectively included in the analysis. Phenotypes of disease were defined at baseline. Neurological phenotype (NP) was defined according to sensorimotor and/or autonomic dysfunction, while cardiac phenotype (CP) was defined in the presence of unexplained maximum wall thickness >12 mm and other typical echocardiographic findings. Unaffected carriers (UC) and mixed phenotypes (MP) presented none or both of the above-mentioned features, respectively.

Results

Two hundred and ten patients showed clinical signs of the disease, 37 (13%) with CP, 65 (23%) with NP and 108 (38%) with MP, while 75 subjects (26%) were UC. Ile68Leu was the most represented mutation (96 subjects, 34%), followed by Val30Met (21%) and Glu89Gln (13%). NP patients (mostly Val30Met) had mPND score >1 in 45% of patients, were younger at diagnosis (mean 47 years, p<0,001 vs CP/MP), and sex was equally distributed. In contrast, CP patients were older at diagnosis (mean 70 years, p<0,001 vs CP/MP), predominantly male (as well as in MP) with a higher incidence of tunnel carpal syndrome and a shorter time interval between onset of symptoms and diagnosis (mean 17 months, p<0,001 vs CP/MP). NYHA class, ECG findings, left ventricular wall thickness and ejection fraction did not significantly differ between CP and MP. After a mean follow-up of 59 months, 98 (34%) patients died. On a Kaplan-Meier survival analysis, mean survival times were 208, 123, 150 and 95 months for UC, CP, NP and MP, respectively, with a statistically significant difference in affected patients between NP and MP (p=0.012).

Conclusions

H-ATTR is a rare systemic disorder whose natural history, including age of onset, clinical characteristics and instrumental findings, is strongly influenced by primary phenotypes, ranging from the excellent prognosis of unaffected carriers to the inauspicious outcome of mixed phenotypes.

Funding Acknowledgement

Type of funding sources: None.
Aortic Z-scores in paediatric patients with marfan syndrome and other hereditary thoracic aortic diseases
Authors: Eisfeld M , Weiss K , Yigitbasi M , Pieske B , Berger F , Gehle P
Published: October 2021

Introduction

Marfan syndrome (MFS) and other hereditary thoracic aortic diseases (HTAD) are characterized by dilatation of the aorta caused by mutations in the FBN1-gene and other aortic genes. To prevent aortic dissection or rupture, patients are prophylactically treated by aortic surgery. Different z-scores of the aortic root are used for diagnosis and monitoring in paediatric patients.

Purpose

What are the differences between the various aortic Z-scores in their use for monitoring, assessing the severity of the aortic disease, and establishing the indication for surgery in this age group?

Methods

We retrospectively analyzed the diameters of aortic roots measured by echocardiography in 180 children and young adults (1 month to 25 years) with MFS and other HTADs presenting at our specialized outpatient clinic between January 2010 and August 2019. Thirteen patients underwent surgery during this period and 121 were monitored for a duration of one up to nine years. Five aortic z-scores were compared: Pettersen, Gautier, Cantinotti, Lopez, Boston. For evaluation of the severity of dilatation and indication for surgery, we used the last value before operation or the last follow-up value. For monitoring, we used the difference between the first and the last value. For comparison of the z-scores, values of the sinuses of Valsalva were analyzed. We investigated theoretic backgrounds and reference groups of each z-score.

Results

The z-score values differ significantly (p<0,001) for each score. The medians reach from 2.06 to 3.45, in operated patients 4.90 to 8.52, non-operated patients 1.86 to 3.31 (p<0,001). 93 aortic diameters to 136 were classified as dilatated (z-score ≥2). There were significant differences of almost all z-scores in the comparison with one another. During follow-up, the increase of the median z-score ranged from 0.11 to 0.24, in operated patients from 0.73 to 1.93, in non-operated patients from 0.02 to 0.21, respectively. The median monitoring time was 4.14 years and 2.93 years, for patients monitored for more than one year and all patients, respectively. In the operated subgroup, a weak correlation showed that the longer the monitoring time and the closer the surgery arrived, the faster the z-scores rose. Lopez, Boston and Cantinotti have higher values and label a value quicker than dilatated, but also reveal the severity of dilatation and the potential necessity of surgery.

Conclusions

Our data show that the various z-scores differ significantly. No z-score was superior regarding above-mentioned aspects, their differences in monitoring were small. However, it seems important to consistently use one same z-score. Expectedly, z-score values of each score were higher in the operated subgroup than in patients without surgery. However, there is no z-score value that determines a clear-cut indication for surgery.

Funding Acknowledgement

Type of funding sources: None.
Reperfusion in high-risk acute pulmonary embolism: can the PESI score predict outcomes?
Authors: Martinho M , Cale R , Alegria S , Ferreira F , Loureiro M J , Judas T , Ferreira M , Gomes A , Delerue F , Pereira H
Published: October 2021

Introduction

Acute pulmonary embolism (PE) is one of the leading causes of cardiovascular death worldwide. Haemodynamic (HD) instability defines high risk (HR) of early mortality and reperfusion treatment is the standard of care for rapid relieve of right ventricle (RV) overload in these situations. The impact of reperfusion in long-term outcomes is not well established. The PE Severity Index (PESI) score is used to stratify the risk of early death in HD stable patients (pts) and was not validated to predict outcomes in HR-PE.

Purpose

Estimate the prognostic performance of the PESI score in HR-PE and study its possible interaction in acute and long-term outcomes of reperfusion in HR-PE pts.

Methods

Retrospective single-centre study of consecutive HR-PE pts, defined by the 2019 ESC guidelines criteria, between 2008–2018. Logistic regression analysis was performed to test for an interaction between tertiles of the PESI score and reperfusion in early-mortality (during hospitalization and at 30 days) as well as 1-year MACE (a composite of cardiovascular mortality, PE recurrence or chronic thromboembolic pulmonary hypertension).

Results

Of a total of 1955 PE pts, 102 fulfilled the inclusion criteria (72.5% pts initially presented with HD instability with the remaining developing HR-PE after hospital admission). Mean age was 68±15 years and 60% were females. In-hospital and 30-day mortality were 39.6% and 43.0%, respectively. At one-year follow-up, MACE was 55.0%. Mean PESI at the time of HR-PE diagnosis was 200±39 and showed significant differences for in-hospital mortality (189±38 vs 217±34; OR 1.02, 95% CI 1.00–1.03, p<0.001), 30-day mortality (191±38 vs 214±36; OR 1.02, 95% CI 1.00–1.03, p=0.004) and 1y-MACE (186±41 vs 214±32; OR 1.02, 95% CI 1.01–1.03, p<0.001). Total reperfusion rate was 57.8% and was also associated with lower in-hospital mortality (OR 0.45, 95% CI 0.20–1.02; p=0.057), 30-day mortality (OR 0.35, 95% CI 0.15–0.80; p=0.012) and 1y-MACE (OR 0.35, 95% CI 0.15–0.80; p=0.014). The benefit of reperfusion was significantly influenced by the PESI score categorized by tertiles (figure 1).

Conclusions

Although the PESI score stratifies HD stable pts, in this population it was able to predict cardiovascular outcomes in HR-PE pts. Furthermore, it showed a significant interaction with the prognostic impact of reperfusion in early and late cardiovascular outcomes.

Funding Acknowledgement

Type of funding sources: None. Figure 1. Interaction between PESI and reperfusion
Case fatality rate and fatal bleeding complication in patients with pulmonary embolism and patent foramen ovale
Authors: Hobohm L , Schmitt V H , Munzel T , Konstantinides S V , Keller K
Published: October 2021

Objectives

In patients with acute pulmonary embolism (PE), right atrial pressure is elevated, which increases risk for right-to-left shunt when patent foramen ovale (PFO) is present and thus potentially increases risk for paradoxical embolism. Little is known about the clinical outcome of patients with PE and concomitant PFO.

Methods

We analysed data on patient characteristics, treatments and in-hospital outcomes for all PE patients (ICD-code I26) with concomitant presence of PFO in Germany 2005–2018 (source: Research Data Center (RDC) of the Federal Statistical Office and the Statistical Offices of the federal states, DRG Statistics 2005–2018, and own calculations).

Results

Between January 2005 and December 2018, 1,174,235 patients with acute PE (53.5% females) were included in this analysis; of those, 5,486 (0.5%) had a concomitant diagnosis of PFO. Trends analysis demonstrating an increasing frequency of diagnosed PE with additional PFO from 2005 (n=299) to 2018 (n=556; p<0.001). While patients with PE and PFO presented more often with signs of haemodynamic compromise such RV dysfunction (37.6% vs. 28.5%) or shock (7.1% vs. 3.9%) as well as paradox arterial emboli (47.8% vs. 3.2%) or intracerebral bleeding (3.3% vs. 0.6%), PE patients with PFO died less often compared to PE patients without PFO (11.1% vs. 15.8%). Patients with PE and PFO were younger (65 [IQR 52–75] vs. 72 [60–80]; P<0.001) and were more often treated invasively with a reperfusion treatment approach like embolectomy (10.2% vs. 4.2%) or systemic thrombolysis (5.0% vs 0.1%). A multivariate logistic regression analysis revealed a 27.6-fold increased risk for paradox arterial emboli (OR, 27.6 [95% CI 26.1–29.1]; p<0.001) and a 3.9-fold increased risk for intracerebral bleeding events (OR, 3.9 [95% CI 3.3–4.54]; p<0.001) for patients with PE and concomitant PFO. In normotensive patients with RVD and PFO, embolectomy were not associated to affect the rate of intracerebral bleeding events (OR, 0.8 [95% CI 0.2–2.6]; p=0.720) compared to conventional non-reperfusion treatment; instead of systemic thrombolysis, which is associated with a higher risk of intracerebral bleeding (OR, 3.5 [95% CI 1.8–6.59]; p<0.001) compared to conventional non-reperfusion treatment.

Conclusion

Patients with acute PE and the concomitant presence of PFO are associated with a high risk for paradox arterial emboli and intracranial bleeding events. Especially in normotensive patients, the use of systemic thrombolysis should be considered with cautious. Thus, our findings may improve the clinical management of patients with PE and PFO.

Funding Acknowledgement

Type of funding sources: None.
Outcome and clinical profile of patients with newly detected atrial fibrillation and cardioembolic stroke
Authors: Garkina S , Tatarskiy R , Lebedev D , Efimova O , Pavlova T , Duplyakov D
Published: October 2021

Background

Atrial fibrillation (AF) is a global health care problem with evidence suggesting an increasing prevalence and incidence worldwide. Undiagnosed AF represents the most common cause of thromboembolic events. The aim of the study was to analyze the clinical profile and outcome in patients with cardioembolic stroke and newly detected AF.

Methods

We enrolled 139 consecutive patients with atrial fibrillation and confirmed diagnosis of ischemic stroke (mean age 72.25±6.33 years, 59 male). Follow-up period was 1 year since the episode of acute stroke.

Results

All patients with verified cardioembolic stroke were first diagnosed with AF on admission. Patients with AF were characterized by polymorbidity (hypertension was diagnosed in 96 patients, a concomitant chronic renal failure was observed in 60 cases while a complicated course of coronary heart disease – in 35 patients, 22 patients were diagnosed with diabetes mellitus, while 9 people had a long smoking history). Mean value of CHA2DS2-VASc score was 4.51±1.2 and after acute stroke patients were recommended permanent anticoaugulation (12% – warfarin, 45% – rivaroxaban, 24% – apixaban and 19% – dabigatran). At the end of the year of follow-up patients were taking oral anticoagulants only in 16.2% of cases. In the same time only 9.9% of patients had a history of mild or moderate nasal or gingival bleeding (8.2%) while severe hemorrages were not reported. On multivariable analysis, lack of antithrombotic treatment guideline adherence was associated with increased risk of recurrent stroke (hazard ratio, 4.45; 95% confidence interval, 1.25–6.87; P=0.012 for undertreatment). For 3 (2.2%) patients the recurrent sroke was fatal and 2 (1.4%) patients had lethal outcome due to heart failure deterioration after 6 months of follow up. During one year follow up 27 (19.4%) patients had spontaneous AF conversion into sinus rhythm and 19 (13.7%) patients underwent successful catheter ablation. The adjusted risk of reccurent stroke for those in chronic AF was higher than in sinus rhythm (hazard ratio, 1.70; 95% CI, 1.37–2.12).

Conclusions

This study showed that patients with newly dignosed AF and cardioembolic stroke are characterized with polymorbidity and high thromboembolic risk but after sinus rhythm restoration the adjusted risk of reccurent stroke is much lower. The results demonstrate a low quality care of patients with AF and cardioembolic stroke at the outpatient stage. Appropriate medication compliance is crucial for positive outcomes as well as effectivesecondary stroke prevention in AF patients.

Funding Acknowledgement

Type of funding sources: None.
Effectiveness and prognostic value of intravscular imaging in patients with left main coronary artery stenosis and heart failure
Authors: Maximkin D , Shugushev Z , Chepurnoy A , Bolivougui J M , Faybushevich A , Gitelzon E , Volkov S , Korobkov A
Published: October 2021

Aims

A comparative analysis and evaluate the effectiveness and prognostic value of optical coherence tomography (OCT) and fractional flow reserve (FFR) guiding measurement in patients with stenosis of the terminal part of the left main coronary artery (LMCA).

Methods

222 patients were selected in the study. Inclusion criteria: true bifurcation stenosis of the LMCA according to quantitative coronary angiography (QCA) and classification by A. Medina. Criteria for determining the hemodynamic significance of stenosis: according OCT - minimal lumen area (MLA) in the terminal part of LMCA <6 mm2; according FFR guiding – <0.8 (in LCx or LAD or both). Patients, who have not been diagnosed hemodynamically significant stenosis, were further subjected to the dynamic observation. All received optimal medical therapy. The study continued to participate patients whose compliance to receive drugs was not lower than 80%. Primary endpoints: frequency of MACE (death, myocardial infarction, revascularizations). The follow-up were 12, 24, 36 months.

Results

The OCT was performed in 110 patients and FFR guiding measurement – in 112 patients. According to the OCT, were hemodynamically significant stenoses are determined in 36 (32.7%) patients and after FFR-guiding measurement – in 32 (28.6%) of patients (χ2=2.184 p>0.05). Patients without hemodynamically significant stenoses distributed into 2 groups: group 1 (n=74) – according to the OCT and group 2 (n=80) – according to the FFR. The long-term results were monitored in all patients. The frequency of myocardial infarction in group 1 were 1.4% and in group 2 – 7.5% (p<0.05).The frequency of revascularization in group 1 were 5.4% and in group 2 – 15% (p<0.05). The total frequency of major cardiac events were 6.75% in group I and 22.5% in group II (χ2=6.435; p<0.001). The survival without major cardiac events (Kaplan-Maier analysis) were significantly differ between the groups, of 93.25% in group 1 and 77.5% – in group 2 (χ2=7.162 p<0.001).

Conclusions

The effectiveness of the OCT imaging and the FFR-guiding measurement in determining the hemodynamic significance of the bifurcation stenosis of the LMCA, not different. However, in the long term period, patients with insignificant stenosis identified by the FFR, have a worse prognosis and are distinguished by a major cardiac events, compared with the OCT, which does not allow us to recommend the FFR method as the main one for determining the hemodynamic significance of LMCA.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Russian academic excellence project 5-100
Impact of cusp-overlap view for TAVR with self-expandable valves on 30-day conduction disturbances
Authors: Mendiz O , Noc M , Fava C M , Gutierrez Jaikel L A , Sztejfman M , Pleskovic A , Lev G , Gada H , Tang G H L
Published: October 2021

Background and aim

Conduction disturbances leading to permanent pacemaker implantation (PPMI) remains a common complication for TAVR procedures, particularly when self-expanding valves are used. We compared the 30-day incidence of new onset Left Bundle Branch Block (LBBB) and permanent pacemaker implantation (PPMI) rate between two consecutive groups using either conventional 3-cusp coplanar view (CON) and right/left cusp-overlap view (COVL) for implantation.

Methods and results

We retrospectively compared 257 consecutive patients undergoing TAVR with self-expandable valves using either CON (n=101) or COVL (n=156) in four intermediate/low volume centers. There were no significant differences in baseline characteristics between the groups. The 30-day incidence of new onset LBBB (12.9% vs 5.8%; p=0.05) and PPMI rate (17.8% vs 6.4%; p=0.004) were significantly lower when using the COVL implantation view. There was no difference between the CON and COVL groups in 30-day incidence of death (4.9% vs. 2.6%), any stroke (0% vs. 0.6%) and the need for surgical aortic valve replacement (0% for both groups).

Conclusion

Using the COVL view for implantation we achieved a significant reduction of the LBBB and PPMI rate after TAVR in comparison with the traditional CON view, without compromising the TAVR outcomes when using self-expandable prostheses.

Funding Acknowledgement

Type of funding sources: None.