bluuebunny/crossref_metadata_2025 huggingface dataset:
How accurate is cardiac magnetic resonance in quantitation of primary mitral regurgitation?
Authors: Baron T , Kristensson J , Flachskampf F.A
Published: November 2020

Background

Since evaluation of mitral regurgitation (MR) in patients with mitral prolapse by echo may be challenging, cardiac MRI (CMR) has been used for identification of patients with severe valve disease. Little is known about potential factors influencing accuracy of this approach.

Purpose

To study the impact of left ventricular (LV) contouring methods and interobserver variability on quantitation of MR severity.

Methods

A total of 50 asymptomatic patients with echocardiographically moderate or severe MR were included to LV-REGURGE study and evaluated with CMR by two independent reviewers. LV volumes were calculated on SSFP short axis stack including or excluding intracavitary myocardium into/from the LV volume (“including” or “excluding” method, respectively). Aortic flow volumes were obtained using phase contrast method. Mitral regurgitant volume (RegVol) was calculated by subtracting forward aortic volume from LV stroke volume. Severe MR was diagnosed when RegVol >60mL and/or RegVol/LV stroke volume (RF) >40%. Agreement between MR grading into severe and non-severe, obtained by different tracing methods and different reviewers was assessed using kappa coefficient.

Results

Left ventricular and mitral regurgitant volumes obtained by “including” method were significantly larger as compared with “excluding” method, see table. “Including” method identified severe MR in 68% while “excluding” method in 57% of cases. Overall agreement between the methods was present in 84% of cases, meaning a substantial rate of agreement (κ 0.67). Within respective methods, interobserver agreement was present in 77% of cases, interpreted as a moderate rate of agreement (κ 0.48–0.54)

Conclusion

“Including” trabeculae and papillary muscles into the LV cavity volume substantially increases rate of recognizing severe MR, due to larger LV volumes, particularly LV ESV, comparing with the “excluding” method. Moreover, inter-observer variability in similar extent as tracing method seems to impact differentiation between severe and non-severe MR.

Funding Acknowledgement

Type of funding source: None
Intravascular ultrasound assessment of the actual transcatheter heart valve dimensions among patients treated for a right ventricular outflow tract insufficiency after surgical reconstruction
Authors: Kalinczuk L , Skotarczak W , Rynkiewicz K , Mintz G.S , Zielinski K , Swierczewski M , Michalowska I , Biernacka K , Ryzyllo W , Demkow M
Published: November 2020

Introduction

Transcatheter heart valve (THV) deployment can be used to treat right ventricular outflow tract (RVOT) insufficiency, but it is unknown whether manufacturers' predicted THV dimensions correspond with actualy measured.

Purpose

To compare valve frame nominal dimensions predicted by the manufacturers' charts (Melody or Edwards SAPIEN [ES] 3) versus actual dimensions assessed using intravascular ultrasound (IVUS) with Visions PV.035 Digital Catheter (Philips) offering an imaging field of 60mm.

Methods

IVUS recordings were made post-THV deployment in 6 pts (median age 33 [20–44] yrs, 3 ♀, all with Tetralogy of Fallot) who had undergone prior corrective surgery (4 transannular patch, 1 bioprosthetic valve or 1 pulmonary homograft), but who presented with significant RVOT insufficiency. IVUS-visualized homograft cross-sections were perpendicular to its long axis. Offline volumetric measurements included the inner-THV dimension for a total of 16 evenly spaced cross-sections along the THV length. Assessed were THV inner-frame diameters (minimal and maximal), cross-sectional areas, and cross-sectional area of the visual orifice area identified at the coaptation site (Fig. 1). Each THV (1 Melody [Ø 22mm, 28mm nominal length] and 5 ES3 [1Ø 23mm, 18mm height; 4 Ø 29mm, 22.5mm height]) was implanted after pre-stenting using stents of 36, 39, or 48mm length, deployed on a 24mm (n=1) or 30mm (n=4) balloon-in-balloon catheter.

Results

Overall, 96 paired cross-sections were analyzed. Actual THV inner area was 95%±20% of that predicted. However, minimum THV inner diameter was smaller, but maximal inner diameter exceeded those predicted (Table 1). The ratios of measured-to-predicted minimal and maximal THV inner diameters were 97%±11% and 112%±13%, respectively, with a measured max/min inner THV frame diameter ratio of 1.16±0.13. However, the ratio of max/min lumen diameter measured at the coaptation site was of 1.11±0.11 signifying low eccentricity. Visual orifice area measured 364.3±87.6 mm2, and was 65%±8% of the measured inner THV area.

Conclusions

After balloon-expandable THV implantation to treat RVOT insufficiency, there are differences in dimensions between actually measured vs predicated by manufacturers' charts. IVUS allows online tomographic insight into the actual THV frame dimensions and the substantially smaller, but circular orifice area.

Figure 1. IVUS visualization

Funding Acknowledgement

Type of funding source: None
Prognostic value of right ventricular systolic function by speckle tracking echocardiography beyond conventional echocardiography in significant tricuspid regurgitation
Authors: Hinojar Baydes R , De Angelis V , Garcia-Martin A , Gonzalez-Gomez A , Sanroman M , Pascual M , Lorente A , Monteagudo J.M , Jimenez-Nacher J.J , Zamorano J.L , Fernandez-Golfin C
Published: November 2020

Background

Right ventricular (RV) systolic function is determinant in the evaluation of patients with significant tricuspid regurgitation (TR). Timely detection of RV dysfunction with conventional 2D echocardiography is limited by the geometry and position of the RV. RV strain has emerged as an accurate and sensitive tool for evaluation of RV function with the capability of detect subclinical RV dysfunction

Purpose

This study was aimed to evaluate the prognostic value of RV strain in consecutive patients with significant TR, in comparison with conventional parameters of RV systolic function.

Methods

Consecutive patients in stable clinical status with significant TR (severe, massive or torrential TR) evaluated in the Heart Valve Clinic were included. RV systolic function was measured with conventional echocardiographic parameters (RV fractional area change [FAC], tricuspid annular plane systolic excursion [TAPSE]), DTI S wave ('S) and with STE derived automatic peak global and free wall longitudinal strain (GLS, FW-LS respectively) using the EPIQ system. A combined endpoint of hospital admission due to heart failure or cardiovascular mortality was defined.

Results

A total of 100 patients were included (mean age was 76±10 years, 65% females, 84% in NYHA I/II, 86% functional TR). Mean values of RV function parameters are shown in the table. During a mean follow up of 24±10 months, 24% of the patients reached the combined endpoint. Patients with events showed impaired RV GLS and FW-LS (p<0.01). Both parameters were predictive of the combined endpoint (table 1). Conventional parameters of RV systolic function were not associated with outcomes (p>0.05 for all).

Conclusion

In patients with severe TR, RV strain values are superior to conventional parameters to detect RV dysfunction. Among different measurements of RV function, RV GLS and FW-LS were the only predictors of poor prognosis. These parameters should be included in the serial evaluation of these patients.

Funding Acknowledgement

Type of funding source: None
TAVI durability
Authors: Ziviello F , Di Stefano D , Joannes F , Ancona M.B , Bellini B , Russo F , Ferri L , Carlino M , Montorfano M , Chieffo A
Published: November 2020

Background

TAVI (Transcatheter aortic valve implantation) is now established as the preferred treatment option for patients with symptomatic severe aortic stenosis at prohibitive or high surgical risk and its application is increasingly supported by an accumulating evidence base in patients at intermediate and low risk, but the issue of valve durability are still unsettled.

Purpose

To evaluate long-term clinical outcomes and bioprosthesis valve dysfunction after TAVI.

Methods

All patients treated with transfemoral TAVI, from November 2007 to December 2014 were analyzed. Baseline clinical, procedural and in-hospital outcomes were collected and revised. Clinical and echocardiographic follow-up was conducted either by clinic visits or telephone consultations. All definitions of the clinical endpoints and bioprosthesis valve failure used were in concordance with the Valve Academic Research Consortium 2 and the European Society of Cardiology consensus, respectively.

Results

Four hundred and eight patients were included in the analysis. Median follow-up was 2.733 days (7.5 years), with the longest follow-up at 4.096 days (11.2 years). At 7.5 years (IQR 5.4–9.3 years), all-cause mortality was 64.5% (n=263), of these cardiovascular death was adjudicated in 33.6% (n=137), non-cardiovascular death in 30.9% (n=126) and valve-related death was 1.9% (n=8). Structural valve deterioration occurred in 2.9% (n=12), non-structural valve deterioration in 0.2% (n=1), thrombosis in 2.2% (n=9) and endocarditis in 3.2% of patients (n=13). Fourteen patients (3.4%) required surgical (1.2%) or percutaneous (2.2%) aortic valve re-intervention.

Conclusions

Percutaneous aortic bioprosthesis demonstrated a low bioprosthesis valve failure at a median of 7.5 years of clinical follow-up after implantation, with low rate of degeneration or need of re-intervention. Overall mortality was high with acceptable rates of cardiovascular mortality and low rates of valve-related deaths.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): EAPCI Education and Training Grants
A novel image fusion technique to improve transseptal puncture during percutaneous mitral valve repair
Authors: Meijerink F , Wolsink I , Van Mourik M.S , Planken R.N , Boekholdt S.M , Bouma B.J , Baan J
Published: November 2020

Background

Percutaneous mitral valve repair (PMVR) using the MitraClip (Abbott Vascular), has gained popularity to treat mitral regurgitation (MR) in high risk patients. Accurate transseptal puncture (TSP) is a crucial step to achieve optimal guiding catheter position during the procedure.

Purpose

The aim of this study was to determine whether a planned optimal TSP site, using a novel computed tomography (CT) and echo image fusion methodology, corresponded with the actual TSP site, and if this was related to procedure time and outcome.

Methods

The mitral annulus and inter atrial septum were segmented in 26 patients (mean age 75 years, 50% female) undergoing PMVR. The optimal transseptal puncture (TSP) site was based on (1) perpendicular distance of 35–45mm from the TSP site to the mitral annulus, and (2) posterior position relative to the aorta. Optimal TSP was planned using a cardiac CT scan and 3Mensio software (Pie Medical imaging). The CT scan was exported to a Vivid E95 ultrasound system (GE Healthcare). After alignment with the three-dimensional echo image, the coordinates of the actual TSP site were determined. The distance between the optimal and actual TSP site was measured as (1) difference in height above the mitral annulus, and (2) absolute distance to the actual TSP site. Comparisons were made on procedure time, which was measured from the TSP to the achievement of optimal MitraClip position.

Results

The distance between the optimal and actual TSP site showed to be more than 5mm in 85% of the cases. The mean difference in height above the mitral annulus was 6.9mm. The mean procedure time was 21% longer when the absolute distance was more than 10mm. However, this did not reach statistical significance yet (p=0.4), likely due to small sample size.

Conclusion

CT-echo image fusion is an applicable method to plan TSP. This ongoing study suggests that image fusion based guiding may improve TSP site, reduce PMVR procedure time and potentially lead to more effective reduction of MR.

Bar chart of optimal and actual TSP site

Funding Acknowledgement

Type of funding source: Other. Main funding source(s): Abbott
Cause and risk factors for readmissions after transcatheter aortic valve replacement
Authors: Arora S , Strassle P.D , Hendrickson M.J , Sitammagari K , Qamar A , McRee C , Yeung M , Vavalle J.P
Published: November 2020

Background

Hospital readmissions following transcatheter aortic valve replacement (TAVR) are associated with higher costs and worse outcomes.

Purpose

Identify causes and risk factors for readmissions after TAVR

Methods

Hospitalizations of adults aged ≥50, with aortic stenosis and undergoing elective TAVR between 2012 and 2016 in the National Readmission Database were analyzed. Multivariable generalized logistic regression, adjusting for age, sex, Charleson Comorbidity Index, primary insurance type, median household income, hospital type and size, were used to assess the effect of inpatient complications, length of stay (LOS), discharge disposition, and TAVR hospital volume on 30-day cardiovascular (CV) and non-cardiovascular (non-CV) readmission.

Results

Between January 2012 and November 2016, 56,858 weighted TAVR hospitalizations were included. The most common causes of readmissions after TAVR were heart failure (23%), infection (17%), gastrointestinal (11%), respiratory (8%), and “other” non-CV causes (8%). The adjusted odds of both CV and non-CV readmissions were significantly higher in patients with acute kidney injury, inpatient LOS ≥5 days, those discharged to skilled nursing facility (SNF) and those treated at medium volume compared with high volume hospitals, Table 1.

Conclusion

Heart failure is the most common cause of readmissions after TAVR. Inpatient incidence of acute kidney injury, as well as longer LOS, SNF discharge and lower hospital TAVR volume were associated with higher odds of 30-day readmissions.

Funding Acknowledgement

Type of funding source: None
Adaptive development of concomitant secondary mitral and tricuspid regurgitation after TAVR
Authors: Winter M.P , Bartko P.E , Krickl A , Gatterer C , Nitsche C , Koschutnik M , Dona C , Spinka G , Siller-Matula J , Lang I , Mascherbauer J , Hengstenberg C , Goliasch G
Published: November 2020

Background

Concomitant secondary atrioventricular regurgitation is frequent in patients with severe aortic stenosis scheduled fortranscatheter aortic valve replacement (TAVR). The future implications of leaving associated valve lesions untreated after TAVR remain unknown. Aim of the present study wasto characterize the evolution of concomitant secondary atrioventricular regurgitations and to evaluate their impact on long-term prognosis.

Methods

We prospectively enrolled 429 consecutive TAVR patients. All patients underwent comprehensive clinical, laboratory, and echocardiographic assessments prior to TAVR, at discharge, and yearly thereafter. All-cause mortality was chosen as primary study endpoint.

Results

At baseline, severe concomitant secondary mitral regurgitation (sMR) was present in 54 (13%) and severe concomitant secondary tricuspid regurgitation (sTR) in 75 patients (17%). After TAVR 59% of patients with severe sMR at baseline experienced sMR regression, whereas analogously sTR regressed in 43% of patients with severe sTR at baseline. Persistence of sTR and sMR were associated with excess mortality after adjustment for our bootstrap-selected confounder model with an adjusted HR of 2.44 (95% CI 1.15–5.20, P=0.021) for sMR and of 2.09 (95% CI 1.20–3.66, P=0.01) for sTR (Figure 1). Furthermore patients showing regression of atrioventricular regurgitation exhibited survival rates indistinguishable to those seen in patients without concomitant atrioventricular regurgitation (sMR: P=0.83; sTR: P=0.74)

Conclusion

Concomitant secondary atrioventricular regurgitation in patients with severe AS is a highly dynamic process with up to half of all patients showing regression of associated valvular regurgitation after TAVR and subsequent favorable post-interventional outcome. Persistent atrioventricular regurgitation is a major determinant of TAVR futility and proposes a window of early sequel intervention.

Funding Acknowledgement

Type of funding source: None
A randomized surgical trial of mitral valve repair with leaflet resection versus leaflet preservation on functional mitral stenosis – primary results of the CAMRA CardioLink-2 trial
Authors: Chan V , Mazer C.D , Mesana T , De Varennes B.E , Gregory A.J , Bouchard D , Zuo F , Mohamad Ali F , Tsang W , Latter D.A , Juni P , Teoh H , Quan A , Leong-Poi H , Verma S
Published: November 2020

Background

The gold standard treatment for mitral valve regurgitation due to prolapse involves surgery with annuloplasty and either leaflet resection or leaflet preservation, with placement of artificial neochordae. It has been suggested that leaflet resection may be prone to functional mitral stenosis, whereby a patient may have a higher mitral gradient at peak exercise compared to a leaflet preservation strategy. Although both techniques are widely used, there has been no prospective randomized study conducted to compare these two techniques, particularly in regard to functional mitral stenosis.

Methods

A total of 104 patients with posterior leaflet prolapse were randomized to undergo mitral repair with either leaflet resection (N=54) or leaflet preservation (N=50) at 7 specialized Canadian cardiac centers. Patient age, proportion of female patients, and mean Society of Thoracic Surgeons risk score was 63.9±10.4 years, 19%, and 1.4% for those who underwent leaflet resection, and 66.3±10.8 years, 16%, and 1.9% for those who underwent leaflet preservation, respectively. The primary endpoint was the mean trans-mitral repair gradient at peak exercise 12-months after repair.

Results

Baseline characteristics were similar between the groups. At 12-months, the mean trans-mitral repair gradient at peak exercise in patients who underwent leaflet resection and preservation was 9.1±5.2 and 8.3±3.3 mmHg (P=0.4), respectively. The two groups had similar mean mitral valve gradient at rest (3.2±1.9 mmHg following resection and 3.1±1.1 mmHg following leaflet preservation, P=0.7). There was no between-group difference for the 6-minute walk distance (451±147 m and 481±95 m for the resection and preservation groups, respectively, P=0.3).

Conclusion

We report the first prospective surgical randomized trial to evaluate commonly used mitral valve repair strategies for posterior leaflet prolapse. Leaflet resection and leaflet preservation both yield acceptable results with no difference in postoperative valve gradient and functional status 12-months after surgical mitral valve repair.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): Heart and Stroke Foundation of Canada
The mitral annulus disjunction of mitral valve prolapse: presentation and outcome
Authors: Essayagh B , Sabbag A , Antoine C , Benfari G , Maalouf J , Michelena H , Sarano M.E
Published: November 2020

Background

Mitral-annulus-disjunction (MAD), often described in mitral-valve-prolapse (MVP) patients, remains incompletely defined and its clinical outcome consequences are unknown.

Objectives

To assess MAD prevalence, clinical and echocardiographic characteristics, and independent impact on outcome in patients with isolated-MVP.

Methods

A cohort of 595 (65±16 years, 278 female) consecutive patients with MVP and comprehensive clinical, rhythmic and Doppler-echocardiographic characterization, was identified. Primary-endpoint was overall-survival, or occurrence of ventricular-arrhythmia. To eliminate clinical differences, we matched MAD-patients 1:1 with no-MAD based on propensity score for overall survival analysis using a greedy 5-to-1 digit-matching algorithm.

Results

MAD was frequent, present in 186 (31%) of MVP-patients and independently associated to bileaflet-MVP, leaflet-redundancy, younger-age, female-sex, enlarged left-ventricle-endsystolic-diameter and higher left-ventricle-ejection-fraction (all P≤0.04). Outcome primary-endpoint of survival after MVP diagnosis in matched-cohort (10-year 95±1%) was not associated with MAD presence (10-year 93±2% for no-MAD and 97±1% for MAD, P=0.4). Mortality was comparable for MAD (univariate-hazard-ratio 0.64 [0.25–1.65], P=0.4 vs. no-MAD), even adjusted-comprehensively including for MVP-characteristics (adjusted-hazard-ratio 1.17 [0.39–3.50], P=0.8). However, 220 (51%) MVP-patients were diagnosed ventricular-arrhythmia (161 with ventricular-tachycardia only) and long-term, MAD was associated with lower arrhythmia- and ventricular-tachycardia- overall freedom (adjusted-hazard-ratio for MAD 2.35 [1.75–3.15], P<0.0001 vs. no-MAD and 2.71 [1.93–3.82], P<0.0001 respectively). Hence, 10-year freedom of significant ventricular-arrhythmia in overall-cohort was severely reduced with MAD (41±4% vs. 62±3% for no-MAD).

Conclusion

In this unique MVP cohort, with arrhythmia and MAD comprehensively-characterized, MAD presence was frequent and independently associated to bileaflet-MVP and leaflet-redundancy. Long-term, MAD compared to no-MAD is not independently associated with higher-mortality but to reduced arrhythmia-freedom occurrence.

Survival overall and by V arrhythmia

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): Mayo Foundation
The tricuspid annular peak systolic excursion to systolic pulmonary artery pressure index: association with all-cause mortality in patients with moderate or severe tricuspid regurgitation
Authors: Saeed S , Smith J , Grigoryan K , Lysne V , Rajani R , Chambers J.B
Published: November 2020

Background

In patients with significant tricuspid regurgitation (TR) the conventional markers for the assessment of right ventricular (RV) systolic function may be less accurate. Tricuspid annular plane systolic excursion (TAPSE) indexed to systolic pulmonary artery pressure (SPAP) (TAPSE/SPAP) may be prognostically useful in pulmonary hypertension and right ventricular (RV) dysfunction.

Objective

Our aim was to explore the prognostic value of TAPSE/SPAP index in patients with moderate or severe TR.

Methods

A total of 209 patients (72±14 years, 56% women) with moderate (n=123) or severe (n=86) TR (primary in 6% and secondary in 94%) were followed up for a median of 80 months (mean 70±33 months). The clinical correlates of TAPSE/SPAP ratio and association with all-cause mortality were assessed.

Results

The TAPSE/SPAP index was inversely correlated with all-cause mortality with an optimal threshold of 0.49 mm/mmHg. A low index was found in 139 (68%) patients and was associated with reduced survival (Figure). In a multivariate Cox regression analysis adjusted for age, smoking, coronary artery disease, left ventricular ejection fraction, right atrium area and mitral valve replacement, low TAPSE/SPAP index was associated with significantly higher hazard ratio of all-cause mortality (HR: 2.0; 95% CI 1.27–3.14, p=0.003). Age, coronary artery disease, left ventricular ejection fraction and right atrium area were other independent predictors of all-cause mortality.

Conclusions

The TAPSE/SPAP index, reflecting RV systolic function in the longitudinal axis corrected for force generating by the RV is a powerful predictor of all-cause mortality in patients with moderate or severe TR.

Figure 1

Funding Acknowledgement

Type of funding source: None
Inter and intraobserver variability in the echocardiographic measurement of vegetations in infective endocarditis
Authors: Lopez Diaz J , Garcia Granja P.E , Sevilla M.T , Revilla A , Vilacosta I , Olmos C , Ladron R , Gomez I , Cabezon G , San Roman J.A
Published: November 2020

Introduction and objectives

The indication for surgery to prevent embolism in infective endocarditis includes four clinical scenarios and three different echocardiographic measurements of the maximal vegetation diameter. These cut-off points are completely arbitrary and not evidence-based. Our hypothesis is that the vegetation diameter is not an appropriate surgical criterium. The goal of the study is to analyze the inter and intra-observer variability in this measurement and to compare the surgical indications agreement based on these parameters.

Methods

Two trained echocardiographers have measured the maximal vegetation diameter by transesophageal echocardiogram in 67 consecutive patients with definite infective endocarditis in an off-line workstation. The inter- and intra-observer variability was calculated by the interclass correlation coefficient and with the Bland-Altman analysis. The relationship between the strength of agreement for the cut-off points of 10 and 15 mm was also calculated.

Results

Intra and inter-observer interclass correlation coefficient in the measurement of the maximal longitudinal diameter of the vegetations were 0.872 (0.805–0.917) and 0.757 (0.642–0.839) respectively. The strength of agreement of the intra and inter-observer analysis for the cut-off point of 10 mm were 0.674 (0.485–0.862) and 0.533 (0.327–0.759). For the cut-off point of 15 mm they were 0.696 (0.530–0.862) and 0.475 (0.270–0.679).

Conclusions

The variability in the measurements of the maximal longitudinal diameter by transesophageal echocardiogram between two experimented echocardiographers is good. Nonetheless, surgical indications based on the cut-off points recommended in the European guidelines would have changed in an unacceptable high proportion of patients. Therefore, we suggest that these indications should be revised in the light of our results.

Funding Acknowledgement

Type of funding source: None
Long-term mortality in patients with infective endocarditis who undergo aortic root replacement versus isolated aortic valve replacement
Authors: Kyhl F , Jensen A.D , Oestergaard L , Smerup M , Dagnegaard H , Koeber L , Fosboel E.L
Published: November 2020

Background

Infective endocarditis (IE) with involvement of the aortic root (root abscess or aortic prosthetic valve endocarditis (PVE)) is associated with high mortality and morbidity, and it often requires root replacement. IE-guidelines recommend surgery in patients with aortic root involvement (class B recommendation), but the surgical technique is challenging, and the perioperative risk is high. Long-term data are sparse for these high-risk patients and may help better select appropriate patients for surgery.

Purpose

We set out to investigate the short- and long-term mortality for patients with destructive aortic valve endocarditis who underwent aortic root replacement. For comparison, we included those who underwent isolated aortic valve replacement for IE (isolated AVR).

Methods

We included patients with first-time IE from 2000 to 2016 who underwent aortic valve surgery identified from The Eastern Danish Thoracic surgery database. Patient characteristics were identified by cross-linking Danish nationwide databases. Patients who underwent aortic root replacement were compared with those who underwent isolated AVR. Kaplan-Meier plots and multivariable Cox regression analyses were used to estimate and compare the associated 30-days and 10-year mortality risks between groups.

Results

We included 368 patients with aortic valve IE who underwent AVR surgery; 126 patients underwent aortic root replacement and 242 underwent isolated AVR. Median age for root replacement patients was 65.4 years (interquartile range [IQR] 56.2–73.0) compared with 62.1 years (IQR 52.3–71.6) for isolated AVR patients. In the root replacement group, 40.5% had prosthetic valve endocarditis (PVE), whereas 6.6% had PVE in the isolated AVR group. 30-day mortality was 12.7% (CI95: 7.6%-19.2%) in the root replacement group and 7.0% (CI95: 4.3%-10.7%) in the isolated AVR group (P=0.06). Estimated 10-year mortality was 54.4% (CI95: 40.3%-67.6%) in the root replacement group and 45.3% (CI95: 35.7%-54.5%) (P=0.07) after isolated AVR (figure 1). At up to 10 years follow-up, there was no significant difference in adjusted mortality between the groups, adjusted HR=1.34 (CI 95: 0.90–2.00).

Conclusion

Patients with IE who underwent aortic root replacement surgery more often had a prosthetic heart valve, were older, and were more often male. There was no significant difference in long-term mortality between the groups. Nonetheless, long-term mortality was high – 50% of patients died by 10 years, and our results underline the need for stringent patient selection.

Figure 1

Funding Acknowledgement

Type of funding source: None
Genetic counselling and testing of adult patients with cardiomyopathies: insight from the EORP cardiomyopathy and myocarditis registry of the European Society of Cardiology
Authors: Helio T , Elliott P , Koskenvuo J.W , Gimeno J.G , Tavazzi L , Tendera M , Kaski P , Maggioni A , Laroche C , Caforio A.L.P , Charron P
Published: November 2020

Introduction

Cardiomyopathies comprise a heterogeneous group of diseases, often of genetic origin.

Purpose

We assessed the current practice of genetic counselling and testing of adult cardiomyopathy patients in the prospective ESC EORP cardiomyopathy registry.

Methods

3 208 adult patients from sixty-nine centres in 18 countries were enrolled. Clinical data on genetic counselling and testing and on the presentation of cardiomyopathies were gathered.

Results

Genetic counselling was performed in 60.8% of all patients (75.4% in hypertrophic (HCM), 39.2% in dilated (DCM), 70.8% in arrhythmogenic right ventricular (ARVC) and 49.2% in restrictive cardiomyopathy (RCM), p<0.001). Comparing European geographical areas, genetic counselling was performed from 42.4% to 83.3% (p<0.001). It was provided by a cardiologist (85.3%), geneticist (15.1%), genetic counsellor (11.3%), or a nurse (7.5%), (p<0.001). Genetic testing was performed in 37.3% of all patients (48.8% in HCM, 18.6% in DCM, 55.6% % in ARVC and 43.6% in RCM, p<0.001). Index patients with genetic testing were younger at diagnosis, had more familial disease, family history of sudden cardiac death or implanted cardioverter defibrillators but less comorbidities than those not tested (p<0.001 for each comparison). At least 1 disease causing variant was found in 41.7% of index patients with genetic testing (43.3% in HCM, 33.3% in DCM, 51.4% in ARVC and 42.9% in RCM, p=0.13).

Conclusion

We report on the practice of genetic counselling and testing in cardiomyopathies in Europe. Genetic counselling and testing were performed in a substantial proportion of patients but less often than recommended by European guidelines, and much less in DCM than in HCM and ARVC, despite evidence for genetic background.

Funding Acknowledgement

Type of funding source: None
Eosinophilic granulomatosis with polyangiitis, a new recurrent feature in an extremely rare disease
Authors: Zampieri M , Beltrami M , Fumagalli C , Dei L , Urban L , Emmi G , Marchi A , Carrassa G , Chiriatti C , Tomberli A , Baldini K , Olivotto I
Published: November 2020

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare necrotizing vasculitis affecting small- and medium-sized vessels. EGPA may affect the heart leading to myocardial inflammation and necrosis. Although, only a few cardiological based studies have been conducted.

Purpose

This study aimed to investigate the prevalence and clinical impact of cardiac-specific involvement (CSI+) and to give an update on EGPA cardiological manifestations.

Methods

This is a single-centre study. Cardiological evaluation included ECG, blood test, echocardiography, global longitudinal strain (GLS), cardiac magnetic resonance (CMR).

Results

We prospectively enrolled 52 consecutive EGPA patients, between October 2018 and October 2019, mean age 59±3 years, 30 (57%) female. We identified 13 (25%) CSI+ patients: 6 myocarditis, 2 pericarditis, 1 coronaritis, 1 Prinzmetal angina, 2 LV apical thrombosis, 1 unexplained wall motions abnormalities (WMA) in the absence of coronary artery disease.

Twelve-leads ECG revealed abnormalities in 11 (85%) CSI+ vs 9 (23%) CSI−, p=0.0001; ECG abnormalities identified CSI+ with 85% sensitivity, 77% specificity, 94% negative predictive value.

Median troponin level in CSI+ 9 ng/L (IQR 6–11) vs CSI− 11 ng/L (IQR 6–25), p=0.2548; NT-pro-BNP value in CSI+ 210 pg/L (IQR 175–484) vs CSI− 159 ng/L (IQR 66–299), p=0.0576.

Echocardiographic left ventricular end diastolic volume in CSI+ 62±4 ml/m2 vs CSI− 52±1 ml/m2, p=0.0116; LV ejection fraction in CSI+ 57%±2 vs CSI− 66%±1, p=0.0002. In CSI+ patients GLS was −15±1 vs CSI− GLS −21±0.4, p<0.0001. Echocardiography identified WMA in 8 (61%) CSI+ vs 1 (3%) CSI−. In 7 (54%) CSI+ patients, apical segments showed WMA and among them 5 demonstrated the presence of apical aneurysm.

Twelve patients underwent CMR, it showed non ischaemic late gadolinium enhancement in 7 patients; in 5 patients we identified akinetic or dyskinetic segments without associated LGE. Two patients showed LV apical thrombus.

Conclusions

EGPA showed a high prevalence of CSI. We repeatedly found a tropism for apical involvement in WMA that often manifested themselves as apical aneurysm. These may be a new clinical feature, previously not described, in such a rare condition.

Cardiac involvement EGPA

Funding Acknowledgement

Type of funding source: None
Long term outcomes in left ventricular non-compaction
Authors: Casas G , Oristrell G , Limeres J , Gutierrez Garcia-Moreno L , Barriales R , Garcia-Pavia P , Zorio E , Gimeno J.R , Villacorta E , Jimenez-Jaimez J , Ripoll T , Bayes A , Diez C , Ferreira I , Rodriguez-Palomares J.F
Published: November 2020

Background

Left ventricular non-compaction (LVNC) is a highly heterogeneous entity with a wide phenotypic expression. Risk factors have not been well established and prognostic stratification remains challenging.

Objectives

Describe long term outcomes of LVNC patients and determine predictors of cardiovascular events.

Methods

Prospective multicentric study of consecutive patients fulfilling imaging criteria for LVNC. Demographic, ECG, imaging and genetic variables were collected. End points were heart failure (HF), ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause death. Major adverse cardiovascular event (MACE) was described as the combination of the four previous end points.

Results

592 patients from 13 referral centres were included from 2000 to 2018. Mean age at diagnosis was 45 years, 252 (43%) were female and mean LVEF was 48% (Table 1). During a median follow-up of 55 months (IQR 24–90), 144 (25%) patients presented HF, 101 (18%) VA, 27 (5%) SE and 33 (6%) died. MACE occurred in 223 (39%) patients.

In multivariate analysis, independent predictors of HF were LVEF (OR 0.9), PSAP (OR 1.17) and late gadolinium enhancement (LGE) (OR 1.3). VA were independently associated with LVEF (OR 0.97) and LGE (OR 2.51). Independent predictors of SE were LVEF (OR 0.96) and LA diameter (OR 1.07). No independent predictors of all-cause death could be described. MACE were independently associated with LVEF (OR 1.04) and PSAP (OR 1.08) (Table 1).

Among patients who underwent genetic testing (340, 57%), genotype was associated with outcomes: MYH7 and ACTC1 variants were protective while multiple mutations, TTN and MYBPC3 variants exhibited worse prognosis.

Conclusions

In a large prospective multicentric cohort of LVNC patients, there was a moderate long term incidence of cardiovascular events. LVEF and fibrosis were the main predictors and genotype was a modifier of outcomes. These factors might be used to risk stratify LVNC patients.

Funding Acknowledgement

Type of funding source: None
Clinical characteristics and natural history of PRKAG2 syndrome
Authors: Dominguez F , Lopez-Sainz A , Rocha-Lopes L , Barriales-Villa R , Climent V , Tiron C , Marques N , Rasmussen T.B , Espinosa M.A , Quarta G , Arad M , Asselbergs F , Olivotto I , Elliott P , Garcia-Pavia P
Published: November 2020

Purpose

Mutations in the PRKAG2 gene cause a syndrome characterized by hypertrophic cardiomyopathy, conduction disease and ventricular preexcitation. Only a small number of cases have been reported, and the natural history of the disease is poorly understood. The aim of this study is to describe phenotype and natural history of PRKAG2 mutation in a large multicenter international cohort.

Methods

We retrospectively studied clinical, electrocardiographic and echocardiographic data from 90 individuals with PRKAG2 mutations (53% males, 33±21 years) from 27 centers.

Results

At baseline evaluation, 93% of patients were in NYHA functional class I-II. Maximum left ventricular (LV) wall thickness was 18±8 mm and LV hypertrophy (LVH) was present in 60 (67%) subjects at baseline. LV ejection fraction was 61±12%. Seventeen pactients (19%) had a pacemaker (mean age at implantation 37±15and 16 (18%) had atrial fibrillation (AF) (mean age 41±23 years) and 33% had ventricular preexcitation or had undergone an accessory pathway ablation. After a median follow-up of 6 years (IQR:2.3–13.9), 71% of individuals had LVH, 29% had AF, 21% a de novo pacemaker (mean age at implantation 38±18 years), 14% required admission for heart failure (HF), 8% experienced sudden cardiac death or equivalent, 4% required a heart transplant and 13% died.

Conclusions

PRKAG2 syndrome is a severe, progressive cardiomyopathy characterized by high rates of AF, conduction disease, advanced HF and life-threatening arrhythmias. Outcome is not clearly related to the classical features of preexcitation and severe LVH, which are not always present.

Natural history of PRKAG2 syndrome

Funding Acknowledgement

Type of funding source: Public Institution(s). Main funding source(s): Instituto de Salud Carlos III (ISCIII)
Gender variability in the clinical phenotype of hypertrophic cardiomyopathy
Authors: Wasserstrum Y , Larranaga-Moreira J.M , Lotan D , Fernandez-Fernandez X , Klempfner R , Sabbag A , Freimark D , Monserrat L , Arad M , Barriales-Villa R
Published: November 2020

Introduction

Hypertrophic cardiomyopathy (HCM) is a structural heart disease defined by an increase in left-ventricular (LV) wall thickness, that may be complicated with a clinical syndrome of heart failure, arrhythmia and death.

Purpose

To characterize the clinically significant factors for female HCM patients and bring forward the awareness to gender-based differences in HCM.

Methods

We reviewed the patient records of 1297 patients age ≥18 years, diagnosed with HCM, from two tertiary medical centers in Spain and Israel, and analyzed 748 male and female patients matched for age ≥60 years at admission, BMI, hypertension, diabetes and obstructive HCM.

Results

Female patients were older at original diagnosis [median 54 years (38–66) vs. 50 years (35–60), p=0.007], and had higher rates of family history of both HCM (46% vs. 37%, p=0.01) and sudden cardiac death (23% vs. 15%, p=0.02). Female patients had lower rates of active smoking (11% vs. 25%, p<0.001) and coronary disease (3% vs. 8%, p=0.007). In echocardiographic studies, females had a smaller LV cavity both during diastole [median 42mm (39–46) vs. 46 (42–50), p<0.001] and during systole [median 24 (21–29) vs. 27 (23–31), p<0.001]. Female patients had higher rates of significant mitral insufficiency (37% vs. 19%, p<0.001), and despite no significant differences in the rates of obstructive HCM, diastolic dysfunction, left-atrial enlargement, atrial fibrillation, ventricular tachyarrhythmia or pulmonary disease, they also had more significant pulmonary hypertension (≥45mmHg, 29% vs. 14%, p<0.001). Women had a lower functional capacity (p<0.001 for differences in NYHA, see fig.), and lower exercise capacity [median 7.0 METs (5–10) vs. 10.0 (7–12), p<0.001) and more abnormal blood pressure response to exercise (35% vs. 26%, p=0.03). Electrocardiographic features were similar across genders, except for a non-clinically significant shorter QRS duration in females. No significant differences were seen in 5-year mortality follow-up, which was ∼3% in the entire cohort.

Conclusions

Female patients suffering from HCM have a clinical phenotype defined by slightly later presentation (and shorter time until admission to a tertiary-center cardiomyopathy clinic), smaller LV cavity size, higher severity of mitral insufficiency and pulmonary hypertension and worse functional and exercise capacities. Genders did not differ in 5-year mortality, which was relatively low.

Functional and exercise capacity

Funding Acknowledgement

Type of funding source: None
Ruling in or out the presence of cardiac amyloidosis based on cut-off values using T1-mapping – caution is required regarding the control group
Authors: Korthals D , Chatzantonis G , Bietenbeck M , Meier C , Florian A , Yilmaz A
Published: November 2020

Background

Cardiac amyloidosis (CA) is an infiltrative disease that is characterized by accumulation of amyloid deposits in the interstitium of the myocardium. In contrast, hypertrophic cardiomyopathy (HCM) is caused by a disorganized arrangement of myocyte hypertrophy as well as expanded extracellular matrix, composed of interstitial and replacement fibrosis.

Purpose

A diagnostic algorithm based on (native) T1-mapping using cardiovascular magnetic resonance (CMR) was suggested in a recent study for the diagnosis of CA: A native T1 <1,036ms was mentioned to have a 98% negative predictive value (NPV) for ruling out CA whereas a native T1 >1,164ms showed a 98% positive predictive value (PPV) for the presence of CA. In the present study, we critically addressed the calculation of such cut-off values considering possible differences in the composition of the control group.

Methods

N=30 patients with CA, N=20 patients with HCM and N=15 healthy controls without relevant cardiac disease underwent dedicated CMR studies on a 1.5-T MR scanner. The CMR protocol comprised standard sequences for cine-imaging, native and post-contrast MOLLI-based T1-mapping and late-gadolinium-enhancement (LGE). ECV measurements were based on pre- and post-contrast T1-mapping images.

Results

Native T1 and ECV were significantly increased in CA compared to HCM and receiver operating characteristic (ROC) analyses revealed an area-under-the-curve (AUC) = 0.984 for native T1 (p<0.001) and AUC = 0.985 for ECV (p<0.001) regarding the diagnosis of CA). When CA patients were compared to HCM patients (excluding healthy controls), a native T1 <1,036ms or an ECV <33% were associated with a 99% NPV for ruling out CA whereas a native T1 ≥1,082ms or an ECV ≥41% were associated with a 99% PPV for diagnosis of CA. However, when CA patients were compared to healthy controls (excluding HCM patients), a native T1 <1,025ms or an ECV <34% were associated with a 99% NPV for ruling out CA whereas a native T1 ≥1,025ms or an ECV ≥34% were associated with a 99% PPV for diagnosis of CA since there was no overlap in native T1 and ECV values between CA patients and healthy controls.

Conclusion

Cut-off values for native T1 or ECV derived from ROC analyses (in a specific group of study patients) for ruling in or out the presence of CA are – amongst others - determined by the native T1 and ECV values of the respective “control group”. A different composition of the control group (e.g. HCM patients vs. healthy volunteers) will result in different cut-off values. Hence, previously suggested cut-off values obtained in single center studies need to be considered carefully – with a special attention to the control group of the underlying study – and should not be transferred to other centers carelessly.

Funding Acknowledgement

Type of funding source: None
Long-term integrated safety of patisiran in patients with hereditary transthyretin-mediated amyloidosis with polyneuropathy
Authors: Gillmore J , Berk J , Dispenzieri A , Polydefkis M , Gonzalez-Duarte A , Sekijima Y , Sweetser M.T , Arum S , Wang J.J , White M.T , Maurer M
Published: November 2020

Background/Introduction

Hereditary transthyretin-mediated (hATTR) amyloidosis is a progressive, life-threatening disease; the majority of patients develop a mixed phenotype of polyneuropathy and cardiomyopathy. Patisiran halted or reversed polyneuropathy and improved quality of life in the Phase 3 (APOLLO) study. In a prespecified cardiac subpopulation of APOLLO, patisiran also improved cardiac structure and function versus placebo.

Purpose

To describe the long-term comprehensive, integrated safety data from the patisiran clinical development program in patients with hATTR amyloidosis with polyneuropathy.

Methods

Safety data as of October 7, 2019 from the Phase 2 Open-Label Extension (OLE) (NCT01961921), Phase 3 APOLLO (NCT01960348), and ongoing Global OLE (NCT02510261) studies were analysed.

Results

Across the three studies, 224 patients received patisiran for a mean (range) of 43.6 (0.7–71.7) months, with a cumulative 813.9 patient-years of exposure; 105 (46.9%) patients received patisiran for ≥4 years and 35 (15.6%) patients received patisiran for ≥5 years. In this cohort, 149 (66.5%) had medical histories of cardiac disorders per MedDRA System Organ Class (SOC), which may be reflective of a mixed phenotype in some patients. A total of 222 (99.1%) patients experienced at least one adverse event (AE) and 132 (58.9%) patients experienced at least one serious AE. AEs considered to be related to patisiran and occurring in >5% of patients included infusion-related reactions (IRRs) (25.9%) and diarrhoea (6.3%). Cardiac AEs occurring in >5% of patients included atrial fibrillation (10.7%) and cardiac failure (7.6%). Amongst all patients, the exposure-adjusted mortality rate was 4.3 deaths per 100 patient-years.

Conclusions

Patients with hATTR amyloidosis with polyneuropathy in the patisiran clinical development program represent those with the longest treatment with an RNAi therapeutic, including more than 15% of patients receiving patisiran for ≥5 years. Patisiran continues to demonstrate a positive benefit:risk profile in patients with hATTR amyloidosis with polyneuropathy.

Funding Acknowledgement

Type of funding source: Private company. Main funding source(s): Alnylam Pharmaceuticals