bluuebunny/crossref_metadata_2025_split huggingface dataset:
Percutaneous transluminal septal myocardial ablation markedly reduces energy loss in hypertrophic obstructive cardiomyopathy: a four-dimensional flow magnetic resonance imaging study
Authors: Dai Z , Iguchi N , Takamisawa I , Takayama M , Nanasato M , Kanisawa M , Mizuno N , Isobe M 
Published: November 2020

Abstract

Background
Functional follow-up modalities of hypertrophic obstructive cardiomyopathy (HOCM) subjected to percutaneous transluminal septal myocardial ablation (PTSMA) are limited mainly to echocardiography and catheterization. Recent advancements in four-dimensional (4D) flow magnetic resonance imaging (MRI) have enabled us to assess patients from the perspective of fluid dynamics by visualising blood flow and calculating quantitative parameters such as wall shear stress and energy loss within cardiac chambers or blood vessels. Several reports have demonstrated that the intra-cardiac energy loss decreased along with improvement of cardiac function achieved by treatment of cardiac diseases. Whether changes in energy loss occur along with PTSMA in HOCM patients and the underlying mechanism remain unknown.

Purpose
This study sought to investigate the influence of PTSMA in patients with HOCM on energy loss in the left ventricle (LV) and aortic root measured by 4D flow MRI.

Methods
We retrospectively recruited HOCM patients who underwent PTSMA at a referral centre from May to November 2019. Patients who underwent 4D flow MRI both before and after PTSMA were included. We collected demographic and clinical data from electronic health records. MRI scans implemented two-dimensional phase-contrast imaging of the three-chamber plane with three-directional velocity, using a 1.5 T scanner. Furthermore, 4D blood flow analysis was performed on off-line saved data, using iTFlow version 1.9. We assessed energy loss in one cardiac cycle within the three-chamber plane of the LV and aortic root (area surrounded by the LV endocardium, sinotubular junction, and mitral annulus).

Results
This study finally included 12 patients, whose mean age was 66±12 years, and 5 (42%) of whom were men. The pressure gradient between the LV apex and ascending aorta was 81±32 mmHg before and 20±22 mmHg immediately after PTSMA (P&lt;0.005, paired). Before PTSMA, 6 patients were in New York Heart Association functional class III and the other 6 in class II. However, after PTSMA, 10 patients improved to class I and 2 to class II. PTSMA reduced energy loss in one cardiac cycle within the three-chamber plane of the LV and aortic root, from 79±36 mJ/m to 55±19 mJ/m (P=0.001, paired).

Conclusions
PTSMA in patients with HOCM reduced energy loss within the LV and aortic root, indicating significant decrease with cardiac workload. Four-dimensional flow MRI of the three-chamber plane to assess energy loss within the LV and aortic root is a time-efficient and reproducible quantitative method to evaluate the effects of PTSMA. Given its non-invasive nature, it also enables to sequentially follow-up HOCM patients who underwent PTSMA.
Periprocedural changes of energy loss

Funding Acknowledgement
Type of funding source: None
Benefits of tafamidis in patients with advanced transthyretin amyloid cardiomyopathy
Authors: Rapezzi C , Kristen A.V , Gundapaneni B , Sultan M.B , Hanna M 
Published: November 2020

Abstract

Background
In the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT), tafamidis was shown to be an effective treatment for patients with transthyretin amyloid cardiomyopathy (ATTR-CM). Further assessment of the efficacy of tafamidis in patients with more advanced ATTR-CM would aid treatment decisions.

Purpose
To characterize the benefits of tafamidis in patients with advanced ATTR-CM.

Methods
In ATTR-ACT, ATTR-CM patients were randomized to tafamidis (n=264) or placebo (n=177) for 30 months. Efficacy outcomes included all-cause mortality and frequency of cardiovascular (CV)-related hospitalisations. Key secondary endpoints were change from baseline to Month 30 in 6MWT distance and KCCQ-OS score. Efficacy assessments in NYHA Class III patients at baseline (n=141) were a pre-specified analysis. In a post-hoc analysis, mortality and CV-related hospitalizations were assessed in all patients grouped into quartiles of increasing disease severity based on 6MWT distance at baseline. Longer-term all-cause mortality (as of 1 Aug 2019) was assessed in NYHA Class III patients utilizing data from ATTR-ACT patients who enrolled in a long-term, extension study (LTE) and continued treatment with higher dose tafamidis (n=55; median treatment duration 51.6 months); or, if previously treated with placebo, started tafamidis treatment (placebo/tafamidis; n=63 [50.1 months]).

Results
In advanced ATTR-CM patients (NYHA Class III), tafamidis reduced the risk of death (HR [95% CI] 0.837, [0.541, 1.295], P=0.4253), and the decline in 6MWT distance (LS mean [SE], 31.6 (22.1) m; P=0.1526) and KCCQ-OS score (LS mean [SE], 13.1 (5.0); P=0.0090), vs placebo. Paradoxically, there was a higher frequency of CV-related hospitalizations with tafamidis (RR [95% CI] vs placebo, 1.411 [1.048, 1.900]). In all patients by 6MWT quartile, CV-related hospitalizations/year with tafamidis and placebo increased with disease severity, with the exception that placebo-treated patients in the highest severity quartile had fewer CV-related hospitalisations (0.73) than those in the third quartile (0.92). Mortality with tafamidis and placebo increased, and was greater with placebo, in every quartile (Figure). Survival (NYHA Class III patients in ATTR-ACT and LTE) was improved with high dose tafamidis with longer term follow-up (HR vs placebo/tafamidis [95% CI], 0.6569 [0.4175, 1.0336]; P=0.0692).

Conclusions
These analyses, including longer-term follow-up, demonstrate that patients with advanced ATTR-CM benefit from tafamidis. The decrease in CV-related hospitalisations in more severe patients treated with placebo suggests that the comparatively greater hospitalisation frequency in NYHA Class III patients treated with tafamidis is a consequence of their lower mortality rate.
Figure 1

Funding Acknowledgement
Type of funding source: Private company. Main funding source(s): This study was sponsored by Pfizer
Prevalence of hereditary transthyretin cardiac amyloidosis (mATTR) in Western Denmark
Authors: Rasmussen T.B , Vase H , Ladefoged B , Dybro A.M , Clemmensen T , Terkelsen A.J , Molgaard H , Petersen L.N , Poulsen S.H 
Published: November 2020

Abstract

Purpose
To investigate the prevalence of mATTR in a cohort of consecutive ATTR patients in a tertiary Danish Amyloidosis Center.

Methods
Consecutive patients in the period from 2001 to 2019 with a definite diagnosis of ATTR-CA or ATTR-FAP based on either 99-Tc-DPD-scintigraphy, or transthyretin deposition in the myocardium or fatty tissue proved by immunohistology or protein mass spectrometry underwent testing for exonic genetic TTR variants. The distributions of age at time of diagnosis of mATTR and wildtype ATTR (wtATTR) are described by mean age, standard deviations, and range.

Results
Seventy-four caucasian patients of Danish ancestry except one (Swedish) with ATTR were included (72 ATTR-CA, 2 ATTR-FAP). TTR missense variants were present in 13.5% (10/74) of ATTR patients concluded to have mATTR: c.148G&gt;A/p.Val50Met, n=1; c.187C&gt;T/p.Pro63Ser, n=1; c.193G&gt;T/p.Ala65Ser, n=2; c.218G&gt;C; p.Gly73Ala, n=1; c.391C&gt;A/p.Leu131Met, n=3; c.424G&gt;A/p.Val142Ile, n=2.
The mean age of mATTR patients was 57.6 years ±13.0, range 37.1–71.3 years, and was significantly lower than mean age for wtATTR patients: 79.4 years ±5.5, range 65.7–90.2 years, p=0.0004 (Welch's two-sample t-test with unequal variances). All patients with mATTR had familial disease proven by clinical phenotyping of family members or had a positive family history with heart failure and pacemaker use.

Conclusion
A minority of Danish patients (10–15%) with ATTR have mATTR. These mATTR patients are characterised by a significantly younger age at time of diagnosis than wtATTR patients. This observation indicates that TTR gene test may not be necessary in ATTR in patients older than 75 years. For the first time, the p.Val142Ile TTR variant, associated with mATTR and prevalent in 4% of African-Americans, was identified in a Danish family with mATTR-CA – a finding that may be explained by a common founder effect.

Funding Acknowledgement
Type of funding source: None
High sensitivity cardiac troponin T is a useful biomarker for predicting prognosis of the patients with sarcoidosis
Authors: Baba Y , Kubo T , Kawaguchi J , Ochi Y , Hirota T , Yamasaki N , Kitaoka H 
Published: November 2020

Abstract

Background
Cardiac involvement is one of the most significant prognostic factors for the patients with sarcoidosis. In spite of the advancement of diagnostic tools, such as cardiac magnetic resonance imaging or 18F-fluoro-2-deoxyglucose positron emission tomography, there is still significant room for improvement in cardiac screening and prognostic prediction.

Purpose
To evaluate the prognostic factors for the patients with sarcoidosis.

Methods and results
We prospectively studied 133 patients with sarcoidosis and evaluated clinical data including biomarkers. The mean age at diagnosis was 62.1±12.8 years. During a mean follow up period of 5.6±4.1 years, nine patients died and 27 patients suffered from cardiac events (cardiac death, heart failure admission, arrhythmic event). We divided patients into two groups according to cardiac events, event group had high serum high-sensitivity cardiac troponin T (hs-cTnT) (0.028±0.017 vs. 0.015±0.011 ng/ml, p&lt;0.001), high BNP (409.2±634.0 vs. 122.4±195.8 pg/ml, p&lt;0.001), low EF (43.1±16.4 vs. 59.2±15.5%, p&lt;0.001). This was observed even if those patients were not diagnosed with cardiac involvement at the enrollment. On the other hand, there were no significant differences in the values of angiotensin-converting enzyme, lysozyme, soluble interleukin-2 receptor, or calcium in both groups. Multivariate analysis revealed that hs-cTnT was an independent biomarker to predict cardiac events (hs-cTnT &gt;0.014 ng/ml: HR 3.2, 95% CI 1.02 to 10.19, p=0.046).

Conclusion
Hs-cTnT was a useful biomarker for predicting cardiac events for the patients with sarcoidosis even if cardiac involvement was not detected at initial evaluation.
Figure 1

Funding Acknowledgement
Type of funding source: None
Cardiac sarcoidosis: frequency, diagnostic approach and follow-up in a tertiary center
Authors: Van Der Velde N , Poleij A , Hassing H.C , Lenzen M.J , Budde R.P.J , Kirschbaum S.W.M , Schinkel A.F.L , Michels M , Hirsch A 
Published: November 2020

Abstract

Background
Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. The aim of this study is to evaluate our diagnostic results and follow-up for the diagnosis of CS in a tertiary center.

Methods
We studied 188 patients with proven extra-cardiac sarcoidosis referred to our outpatient clinic for evaluation of cardiac involvement. Eight patients were excluded because electrocardiogram (ECG) and/or transthoracic echocardiography (TTE) was missing. Cardiac magnetic resonance (CMR) and/or positron emission tomography (PET) was performed in 66% and 37% of the patients, respectively. Median follow-up duration was 2.9 [1.2–5.3] years. The diagnosis of CS was based on the Heart Rhythm Society criteria.

Results
Cardiac symptoms defined as palpitations, angina, dyspnea and (near)-syncope were present in 156 of 180 (87%) patients. Any abnormality on ECG (bundle branch blocks, atrioventricular blocks, sinus tachycardia or atrial fibrillation) and/or TTE (left ventricular ejection fraction &lt;55%, presence of regional wall abnormalities or myocardial hypertrophy) was found in 92/180 (51%) patients. CS was diagnosed in 42 of 180 (23%) patients, of whom 31 (74%) had any ECG and/or TTE abnormalities. However, ECG and/or TTE abnormalities were also present in 44% of the patients without cardiac involvement. Patients with CS showed a second type II or third degree AV-blocks in 3/42 (7%), a left ventricular ejection fraction &lt;35% on TTE in 9/42 (21%), late gadolinium enhancement by CMR consistent with CS in 28/34 (82%), and myocardial FDG uptake by PET in 19/31 (61%). In 84 of the 138 patients without cardiac involvement, CMR was performed. In 15 patients an alternative diagnosis was found (i.e. myocardial infarction or other non-ischemic cardiomyopathy). The estimated 8-year cumulative event rate composite endpoint of sustained ventricular tachycardia, ventricular fibrillation, aborted sudden cardiac death, heart transplantation and all-cause mortality was 41% in the CS patients and 12% in the patients without CS (Figure 1, p&lt;0.001).

Conclusions
In our study, 23% of the patients with proven extra-cardiac sarcoidosis was diagnosed with CS. Cardiac symptoms, ECG and TTE were of limited diagnostic value for screening for CS. CMR provided a good diagnostic yield and identified other cardiac diseases in a substantial number of patients.
Figure 1. KMCurve_CompositeEndpoint

Funding Acknowledgement
Type of funding source: None
Long-term survival with tafamidis in patients with transthyretin amyloid cardiomyopathy
Authors: Damy T , Elliott P , Gundapaneni B , See Tai S , Sultan M.B , Drachman B.M 
Published: November 2020

Abstract

Background
The Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT), demonstrated that tafamidis was an effective treatment for patients with transthyretin amyloid cardiomyopathy (ATTR-CM). Tafamidis, at an 80 mg daily dose, was subsequently approved in several countries for the treatment of ATTR-CM. Patients who completed ATTR-ACT were eligible to enroll in an ongoing long-term extension study (LTE) which provides additional data on the long-term efficacy of tafamidis.

Purpose
To assess the long-term benefit of tafamidis in patients with ATTR-CM and to determine median survival times with treatment.

Methods
Patients with ATTR-CM who completed ATTR-ACT (in which they were randomized to tafamidis meglumine 80 mg, 20 mg or placebo for 30 months) could enroll in the ongoing LTE in which patients continued to be treated with the same dose of tafamidis or, if previously treated with placebo, were randomized to tafamidis meglumine 80 mg or 20 mg. Tafamidis free acid 61 mg is a new formulation (bioequivalent to tafamidis meglumine 80 mg) developed for patient convenience and all patients in the LTE transitioned to tafamidis free acid 61 mg as of 1 Aug, 2018. All-cause mortality (with heart transplant or cardiac mechanical assist device [CMAD] implantation counted as death) was assessed using a Cox proportional hazards model (as of 1 Aug, 2019). Patients treated with tafamidis meglumine 80 mg in ATTR-ACT and the LTE who transitioned to tafamidis free acid 61 mg (tafamidis 80/61 mg) were compared with patients treated with placebo in ATTR-ACT who transitioned to tafamidis in the LTE (placebo/tafamidis).

Results
There were a total of 176 tafamidis 80/61 mg patients and 177 placebo/tafamidis patients. Median treatment duration was 51.9 months with tafamidis 80/61 mg and 51.4 months with placebo/tafamidis. With tafamidis 80/61 mg, there were 75 (42.6%) all-cause deaths; consisting of 67 (38.1%) deaths, 6 (3.4%) heart transplants, and 2 (1.1%) CMAD implantations. With placebo/tafamidis, there were 108 (61.0%) all-cause deaths; consisting of 102 (57.6%) deaths and 6 (3.4%) heart transplants. There was a significant reduction of 41.1% in the risk of all-cause mortality with tafamidis 80/61 mg compared with placebo/tafamidis (hazard ratio [95% CI], 0.5888 [0.4370, 0.7931]; P=0.0004). Median survival time with placebo/tafamidis was 35.8 months but was not reached with tafamidis 80/61 mg.

Conclusions
Treatment with tafamidis significantly improves long-term survival in patients with ATTR-CM. The comparatively poorer survival in patients treated with placebo in ATTR-ACT who transitioned to tafamidis in the LTE highlights the importance of early diagnosis and treatment.

Funding Acknowledgement
Type of funding source: Private company. Main funding source(s): This study was sponsored by Pfizer.
Risk of stroke in patients with Chagas cardiomyopathy is associated with depressed LV function but not with LV myocardial fibrosis or apical aneurysm: a study using cardiovascular magnetic resonance
Authors: Schmidt A , Turin Moreira H , Volpe G.J , Braggion Santos M.F , Marin Neto J.A 
Published: November 2020

Abstract

Background
Chronic Chagas cardiomyopathy (CCC) is classically related to higher occurrence of thromboembolic events, especially stroke, particularly in patients with atrial fibrillation (AF). However, risk factors for stroke in patients with CCC but no atrial fibrillation have not been well established.

Purpose
We aimed to assess the relationship between left ventricular (LV) structure and function, as evaluated with cardiac magnetic resonance (CMR), with stroke in individuals with CCC.

Methods
We prospectively collected data from 141 patients with CCC who underwent CMR from October 2009 to December 2013 and who were thereafter followed for a median period of 6.8 years. The outcome was the occurrence of stroke during the follow-up period. The only exclusion criteria were history of previous stroke, AF or use of oral anticoagulant (OAC) at the time of CMR. CMR-derived LV parameters were: LV ejection fraction (LVEF), extension of LV myocardial fibrosis, assessed by late gadolinium enhancement, and presence of LV apical aneurysm. LVEF was classified as follows: (I) normal ≥55%; (II) mid-range &lt;55% and ≥40%; and (III) reduced &lt;40%. Age and gender were covariates in the adjusted Cox proportional hazard model.

Results
Of the initial 141 CCC participants, 25 subjects were excluded due to previous stroke (n=2), history of AF (n=11), or use of OAC (n=12). The remaining 116 individuals had a mean age of 56±14 years, 51% women. Normal, mid-range and reduced LVEF were found in 48 (41%), 43 (37%), and 25 (22%) patients, respectively. Myocardial fibrosis was detected in 79 of 111 (71%) subjects in whom the detection method was feasible. The extension of LV fibrosis was evaluated as a median of 4.6% [interquartile range: 0–10]. LV apical aneurysm was identified in 36 (31%) patients. During the follow-up (median 6.8 years), stroke was reported in 9 participants. In the univariate analysis, reduced LVEF was significantly associated with higher risk of stroke compared to normal LVEF (HR: 9.2, 95% CI: 1.8–48.4), while there was no significant difference between mid-range and normal LVEF (HR: 1.2, 95% CI: 0.2–8.2). The association of reduced LVEF and stroke remained significant in the multivariable model, adjusted for age and gender (HR: 12.6, 95% CI: 1.9–83.2). Kaplan-Meier curves are presented in figure 1 (logrank p-value &lt;0.001). Neither the extension of LV myocardial fibrosis (HR: 1.0, 95% CI: 0.9–1.1) nor the presence of LV apical aneurysm (HR: 1.9, 95% CI: 0.5–7.2) was related to higher risk of stroke.

Conclusion
In patients with CCC, reduced LVEF assessed by CMR, but not LV fibrosis or presence of apical aneurysm, was a significant predictor of stroke.

Funding Acknowledgement
Type of funding source: None
Management and outcome of acute and recurrent pericarditis during pregnancy
Authors: Lotan D , Wasserstrum Y , Itelman E , Nir-Simchen M , Arad M , Kuperstein R 
Published: November 2020

Abstract

Background
Pericarditis in pregnancy is uncommon. Treatment options, including NSAIDs and long term gluco-corticosteroids (CS) have extensive side-effects, while data on the use of Colchicine in pregnant women who suffer from pericarditis is limited.

Objective
To evaluate the management and outcome of active pericarditis during pregnancy in both acute (AP) and recurrent pericarditis (RP) patients.

Methods and results
Twelve pregnant women (14 pregnancies) with active pericarditis were followed prospectively in our cardiology-pregnancy clinic; 6 with AP and 8 with RP. Etiology: 11 idiopathic, 2 post-pericardiotomy syndrome and 1 Q fever. Maternal age on presentation was 27±4y vs. 33±3y in RP vs AP respectively. Average gestational age upon diagnosis was lower in RP than in AP (18 weeks, range 6–30 vs 26 weeks, range 5–35). In the RP group, 7/8 women (87%) were treated with CS compared with 2/6 women (33%) in the AP group; 3 women in RP group (37%) failed to respond to subsequent treatment with azathioprine and 1 patient was treated successfully with Anakinra. Colchicine was prescribed in 13/14 (93%) of pregnancies, average colchicine exposure during pregnancy was 25±15 weeks. Recurrence during pregnancy: 6/8 (75%) of RP; 2/6 (33%) of the AP, all of them on CS. All pregnancies culminated in a live birth with a mean gestational age on delivery of 37±1.4 weeks. Average birthweight was 3267±507 grams. There were no fetal anomalies or developmental delays after a mean follow-up of 2.7 years. All newborns and maternal outcomes were normal. On long term follow-up after delivery RP patients treated with colchicine developed less recurrences.

Conclusion
Active pericarditis is associated with a high recurrence rate during pregnancy despite treatment with CS. Colchicine use in pregnant women with active pericarditis appears to be safe.

Funding Acknowledgement
Type of funding source: None