bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term follow up ventricular tachycardia patients with preserved cardiac function -from Japanese cardiac sarcoidosis nationwide questionnaire survey-
Authors: Kamada H , Ishibashi K , Nakajima K , Ueda N , Kamakura T , Wada M , Yamagata K , Inoue Y , Miyamoto K , Nagase S , Noda T , Aiba T , Isobe M , Terasaki F , Kusano K 
Published: November 2020

Abstract

Background
Prior ventricular tachycardia (VT) and low left ventricular ejection fraction (LVEF) are the most important prognostic factors in cardiac sarcoidosis (CS). Recently diagnosis of CS was renewed according to Japanese new guidelines. Patients with preserved cardiac function often have VT events, thus new guidelines recommends to assess the implantable cardioverter defibrillator (ICD) implantation for CS patients with preserved LVEF (35%≤LVEF&lt;50%). However, the long-term prognosis of CS patients with preserved LVEF is unclear.

Objective
In CS patients with preserved LVEF, we evaluated the prognosis between VT manifestation and non-VT manifestation groups at CS diagnosis from Japanese nationwide questionnaire survey.

Methods
Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, who had LVEF≤35%, LVEF&gt;50%, or who underwent cardiac transplantations were excluded. 104 patients with LVEF 35–50% (67 females, mean age 60±15 years old, median follow-up periods 2134 days [interquartile range: 758–2935 days]) were analyzed. The prognosis between VT manifestation and non-VT manifestation groups at CS diagnosis were evaluated.

Results
30 patients had VT manifestation at CS diagnosis and 24 patients (80%) received ICDs. 74 patients had no VT manifestation at CS diagnosis and 19 patients (44%) received ICDs during follow up period. All-cause mortality was not different between two groups (Figure). Appropriate ICD therapy of non-VT manifestation group was significantly lower compared with that of VT manifestation group (log-rank p=0.001), however considerable number (n=7, 15%) of non-VT manifestation group had appropriate ICD therapy event during follow-up period. Cox hazard analysis revealed that concomitant non-sustained VT (NSVT) with atrioventricular block (AVB) was a predictor of appropriate ICD therapy in non-VT manifestation group.

Conclusion
This nationwide survey showed that considerable number of CS patients with preserved LVEF had VT events, independent of VT manifestation. Concomitant NSVT with AVB was a predictor of VT events, and ICD implantation should be assessed.

Funding Acknowledgement
Type of funding source: None
Cardiac function at diagnosis is important prognostic factor in patients with cardiac sarcoidosis -from Japanese nationwide questionnaire survey-
Authors: Kamada H , Ishibashi K , Nakajima K , Ueda N , Kamakura T , Wada M , Yamagata K , Inoue Y , Miyamoto K , Nagase S , Noda T , Aiba T , Isobe M , Terasaki F , Kusano K 
Published: November 2020

Abstract

Background
Sarcoidosis is a systemic non-caseating granulomatous disease of unknown etiology. Cardiac involvement (cardiac sarcoidosis, CS) has been reported to be an important prognostic factor in this disease because of heart failure and/or ventricular arrhythmia, and corticosteroid therapy is usually prescribed to prevent cardiac events. However, little is known about the relationship of cardiac function and concomitant corticosteroid therapy on later cardiac events in CS.

Objective
We evaluated the relationship between prognosis and left ventricular ejection fraction (LVEF) at the time of diagnosis in CS patients from the Japanese nationwide questionnaire survey.

Methods
Total of 757 Japanese patients from 57 hospitals who diagnosed CS were examined. Patients who unsatisfied the criteria of the Japanese new guidelines, or who underwent cardiac transplantations were excluded, and 420 patients (287 females, mean age 60±13 years old, median follow-up periods 1864 days [interquartile range: 845–3159 days]) were analyzed. The relationship of adverse events (all-cause death, cardiovascular death, and appropriate ICD [Implantable Cardioverter Defibrillator] discharge) and LVEF (with corticosteroid 84%) (low LVEF: LVEF≤35% n=98 [with corticosteroid in 78%], moderate LVEF: LVEF 35–50% n=104 [with corticosteroid in 93%], normal LVEF: 50≤LVEF n=218 [with corticosteroid in 83%]) were evaluated respectively.

Results
89 CS patients developed all-cause death (n=50), cardiovascular death (n=30) or appropriate ICD discharge (n=48). The frequency of corticosteroid therapy was not different in the each LVEF group, but Kaplan-Meier analysis revealed that all-cause death, cardiovascular death, and all cardiovascular adverse events were more observed in lower LVEF group (log-rank p&lt;0.0001). Furthermore, multivariate Cox hazard analysis revealed that LVEF was a most important independent prognostic factor in CS.

Conclusion
This Japanese nationwide questionnaire survey data showed that initial LVEF was an independent and strong prognostic predictor in CS, therefore primary prevention would be needed even after starting corticosteroid in patients with decreased cardiac function.

Funding Acknowledgement
Type of funding source: None
Recurrences of disease activity in patients with cardiac sarcoidosis under corticosteroid therapy: prevalence, clinical background and prognosis
Authors: Kaneta K , Takahama H , Tateishi E , Moriuchi K , Amano M , Okada A , Amaki M , Hasegawa T , Ohta Y , Kiso K , Kanzaki H , Kusano K , Yasuda S , Izumi C 
Published: November 2020

Abstract

Background
Corticosteroid therapy (CTx) has been widely accepted as first-line therapy for cardiac sarcoidosis (CS), but there are very limited data regarding recurrence of disease activity of CS. We retrospectively investigated the prevalence, patient characteristics and prognostic significance in patients with recurrences of CS.

Methods
We identified 102 consecutive patients who were clinically diagnosed CS (admission: 2012 and 2019) and whose disease activity was diminished clinically at least once. Recurrence of CS was defined as detection of increased uptake of 18F-fluoro-2-deoxyglucose or gallium-67 in the follow-up examination. Composite adverse events (events) were defined as all-cause of death or hospitalization for heart failure. Echocardiographic and laboratory data before initiation of CTx were obtained.

Results
During the follow-up term (median: 4.9 years), the recurrences of CS occurred in 28 patients at 30 months (median) after the initiation of CTx. In patients with recurrence, left ventricular (LV) ejection fraction before initiation of CTx was lower than in those without recurrences (median: 31% vs. 39%, p&lt;0.05). After the detection of CS recurrences, 17 patients were treated with only increases of PSL and remaining 11 patients were treated with adding other immunosuppressive therapies to CTx. The univariate analysis demonstrated that there were no parameters in echocardiographic and laboratory examinations to predict the recurrences of CS. The results of univariate analysis for event occurrences (n=12) are shown in the Table. Additionally, the Kaplan-Meier analysis showed that there were no differences in event free survival rate in the patients with and without CS recurrences (Figure).

Conclusion
This study showed that the recurrences of disease activity were observed in a substantial number of patients with CS even under the CTx. All patients received intensification of CTx or additional immunosuppressive therapy, and LV systolic function, rather than the recurrence itself, was associated with clinical outcomes in this study.
Kaplan-Meier analysis for events

Funding Acknowledgement
Type of funding source: None
Long-term monitoring of arrhythmias in cardiac sarcoidosis
Authors: Bakker A , Mathijssen H , Balt J , Van Dijk V.F , Veltkamp M , Swaans M , Akdim F , Grutters J.C , Post M.C 
Published: November 2020

Abstract

Introduction
Screening for cardiac sarcoidosis (CS) is recommended since it can manifest with ventricular arrhythmias (VA), atrioventricular conduction block (AVB) and sudden cardiac death (SCD). However, risk stratification for SCD is challenging, in particular in patients without overt cardiac symptoms.

Purpose
This study reports the practice-based risk stratification for SCD and the incidence of arrhythmias and mortality in CS patients by long-term monitoring of arrhythmias.

Methods
A retrospective, single center cohort study was performed in 537 patients with sarcoidosis screened for cardiac involvement with cardiac MRI and fluorodeoxyglucose PET in an hospital, a Dutch tertiary referral center. CS was diagnosed in 115 of 537 patients (21%), complete follow up was available in 108 patients (94%). After risk assessment for SCD (figure 1) an ICD was implanted in 16 high-risk patients. Within the92 low-risk patients, 80 had an internal loop recorder (ILR) implanted and 12 patients received no device. Chart review was performed to assess the occurrence of VA, AVB, death, ICD therapy and device related complications.

Results
During a mean follow-up of 31±15 months, 9 out of 80 ILR patients (11.3%) received an ICD of whom 7 (8.8%) based on recorded arrhythmias (VA in 5 and AVB in 2 patients).
Five out of the total 25 ICD patients (20%) experienced sustained VA successfully treated with anti-tachycardia pacing in 2 (8%) and terminated spontaneously in all other patients. Two ICD patients experienced a mild pocket infection, treated with antibiotics. Two deaths occurred in the low-risk patients: 1 non-cardiac death and 1 SCD due to asystole.

Conclusion
The practice-based risk stratification supported an ICD implantation in up to 5% of sarcoidosis patients screened for CS. Sustained VA occurred in 20% of ICD patients.Early detection of important arrhythmias with an ILR can optimize risk assessment for SCD in CS.
Practise-based risk stratification

Funding Acknowledgement
Type of funding source: None
Prominent involvement of basal left ventricular longitudinal strain in patients with monoclonal gammopathy of undetermined significance
Authors: Canonico M.E , Fiorillo L , Santoro C , Lembo M , Esposito L , Capone V , Casciano O , Esposito R , Galderisi M 
Published: November 2020

Abstract

Background
In cardiac amyloidosis the application of Speckle Tracking Echocardiography allows to identify a specific left ventricular (LV) longitudinal strain (LS) pattern characterized by “apical sparing”, with a prominent involvement of basal and middle segments and normal LS of apical cap. The pattern of regional LS has been never investigated in monoclonal gammopathy of undetermined significance (MGUS), a condition which can predispose to cardiac amyloidosis.

Purpose
To compare LV regional LS patterns and LS base-to-apex behaviour of patients affected by MGUS in comparison with healthy subjects.

Methods
We enrolled 40 patients affected by MGUS (M/F=20/20; age 62.6±13.8 years), asymptomatic for cardiac symptoms, and a control group of 40 healthy subjects, matched for sex and age. Nineteen (47%) MGUS patients showed prevalent free K light chain and 21 (53%) had prevalent free λ light chain. Participants underwent standard echo-Doppler exam, including Speckle Tracking of the three apical views. Global longitudinal strain (GLS), the average LS of six basal (BLS), six middle (MLS), and six apical (ALS) segments (considered in absolute values) and relative regional strain ratio RRSR [=ALS/(BLS+MLS)] were computed. Exclusion criteria were overt heart failure, LV ejection fraction &lt;53%, coronary artery and congenital heart disease, moderate to severe valvular disease, primary cardiomyopathies, atrial fibrillation and inadequate echo imaging.

Results
The two groups were comparable for body mass index, blood pressure and heart rate. LV mass index, relative wall thickness, left atrial volume index and Doppler-derived LV diastolic parameters did not differ significantly between the two groups. LV ejection fraction was also similar in MGUS and healthy controls. GLS resulted significantly lower in MGUS group than in controls (20.5±3.0 vs. 22.4±2.0%, p&lt;0.02). BLS (17.1±3.7 vs. 19.2±2.2%, p=0.004), MLS (24.9±3.8 vs. 27.1±3.6%, p&lt;0.01) and ALS (25.1±3.8 vs. 27.1±3.5%, p&lt;0.01) were significantly lower in MGUS than in controls. The intergroup difference of RRSR (0.60±0.05 vs. 0.58±0.04) did not achieve the statistical significance (p=0.26) and none of the MGUS patients had RRSR&gt;1. The figure depicts a LS bull'eye of a MGUS patient showing the prominent involvement of LV basal segments.

Conclusions
In presence of a normal LV ejection fraction, MGUS patients show a subclinical LV longitudinal systolic dysfunction. This is testified by a reduction of GLS and of regional LS which involves mainly LV basal segments, without substantial changes of relative regional strain ratio. LV regional longitudinal dysfunction could be useful to monitor LV myocardial mechanics during follow-up of MGUS patients.
LS bull's eye in a MGUS patient

Funding Acknowledgement
Type of funding source: None
Randall-type monoclonal immunoglobulin deposition disease: description of cardiac involvement
Authors: Ramonatxo A , Garcia R , Joly F , Degand B , Bidegain N , Bouleti C , Christiaens L , Levesque S , Desport E , Bridoux F 
Published: November 2020

Abstract

Background
Randall-type monoclonal immunoglobulin disease (MIDD) is a rare complication of a monoclonal plasma cell clone. MIDD differs from AL amyloidosis by the presence of Congo red negative non-organized immunoglobulin (Ig) deposits, most commonly light chains (LCDD) along basement membranes and sometimes heavy chains (HCDD) or light and heavy chains (LHCDD). As AL amyloidosis MIDD is a multi-systemic disease, and affects the heart. To date no study has focused on the clinical characteristics of heart disease in MIDD.

Purpose
The aim of this study was to describe the cardiologic features of patients with biopsy-proven MIDD and suspected cardiac involvement.

Methods
This multi-center, nation-wide retrospective study extracted from the database of the French reference center for AL amyloidosis and other Ig deposition diseases between 2012 to 2019. Diagnosis of cardiac involvement was assessed according to the International Society of Amyloidosis criteria for amyloid heart disease, as follows: left ventricular hypertrophy with a diastolic septum thickness ≥12mm, NTproBNP serum level ≥332 ng / L, histological evidence on cardiac or extra cardiac biopsy of typical linear non-organized Ig deposits along basement membranes. Severity was defined according to the Mayo Clinic classification for AL amyloidosis.

Results
Among 20 patients included (mean age was 70±9 years), 11 (55%) were males; 13 (65%) were LCDD, 3 (15%) HCDD and 4 (20%) LHCDD. At diagnosis, 19 (95%) had a history of hypertension, 3 (16%) had atrial fibrillation, 3 (15%) had NYHA grade 3 or 4 dyspnea. Mayo Clinic score was stage 3a in 4 patients (20%) and stage 3b in 6 patients (30%). The most frequent ECG changes were microvoltage (40%) and pseudo Q wave (40%); 64% of patients had altered sinus variability on 24-hour Holter monitoring, one patient had a high-grade conduction disorder and another had ventricular tachycardia. On echocardiography, all showed diastolic dysfunction; mean diastolic septum thickness was 13.5mm; only one patient had LVEF impairment but 38% had global longitudinal strain impairment. 10 patients had cardiac MRI, none showed contrast enhancement after gadolinium injection.
After median follow-up of 28 months, 4 patients were hospitalized for heart failure, including 2 with cardiogenic shock. Seven (35%) patients died within a median of 10 months from diagnosis. Among patients with Mayo clinic stage 3 (a or b), 67% died within a median of 8 months from the diagnosis.

Conclusions
To our knowledge, we present the first case series dedicated to the description of cardiac parameters in MIDD patients with cardiac involvement. Except for MRI appearance of cardiac infiltration, these patients showed features close to that of AL amyloid heart disease. Overall prognosis appears seemingly poor in MIDD patients with Mayo Clinic stage 3 cardiac disease.

Funding Acknowledgement
Type of funding source: None
The right heart in cardiac amyloidosis: a comparison between subtypes and with other genetic and non-genetic causes of left ventricular hypertrophy
Authors: Palmiero G , Rubino M , Monda E , Caiazza M , Trinchillo M.G , Ascione L , Caso P , Limongelli G 
Published: November 2020

Abstract

Background
Right chambers involvement is common in cardiac amyloidosis (CA) but has been ever compared to control groups.

Purpose
Aim of this study is to compare right heart involvement between CA subgroups (AL vs. ATTR amyloidosis) and between CA and other forms of genetic and non-genetic left ventricular hypertrophy.

Methods
We enrolled 25 patients with CA (10 pts with AL and 15 pts with wild type ATTR amyloidosis) and 75 patients with LVH (25 HCM pts; 25 HypCMP pts; 25 AS pts). Beside routine echocardiographic measurements, we analysed right chambers dimensions and classical and novel parameters for right ventricular (RV) function [TAPSE (Tricuspid Anulus Plane Systolic Excursion), St (S' wave at RV TDI), global and free-wall strain].

Results
ATTR group showed higher right dimensions compared to AL, without differences in RV systolic parameters (see table). CA patients, compared to LVH group, showed no differences in right dimensions. RV systolic parameters were significantly reduced while diastolic Doppler parameters were higher (E/E' 21.7±9.0 vs. 11.2±5.0; p&lt;0.0001). At ROC curve analysis TAPSE showed the best ability in discriminating CA among other forms of LVH (AUC 0.936; 95% CI: 0.879–0.993; p&lt;0.0001), with a sensibility of 94.7% and specificity of 87.3% for a cut-off value of 19.5 mm. At Kaplan-Meier estimation CA patients showed a significantly higher cardiovascular mortality compared to LVH group (9/25 deaths vs. none). At multivariate analysis TAPSE was the only independent prognostic factor (β 1.324; 95% IC: 1.086–1.614; p&lt;0.006).

Discussion
CA group showed a significantly impaired RV systolic function with higher pulmonary pressures compared to LVH group. TAPSE proved to be the only able to discriminate CA among genetic and non-genetic forms of LVH and also to have prognostic significance.

Funding Acknowledgement
Type of funding source: None
Response to fluid challenge in patients with atrial septal defect
Authors: D'Alto M , Chessa M , Gaio G , Santoro G , Giordano M , Romeo E , Argiento P , Wacker J , D'Aiello F , Sarubbi B , Russo M.G , Naeije R , Golino P 
Published: November 2020

Abstract

Background
A fluid challenge test (FCT) with a rapid infusion of saline allows for discrimination between pre- and post-capillary pulmonary hypertension (PH) and may unmask hidden post-capillary PH. Patients with atrial septal defect (ASD) may develop pre- or post-capillary PH after shunt closure respectively in case of pulmonary vascular disease or left ventricular disease.

Aim
To evaluate the haemodynamic changes of the pulmonary circulation in ASD patients undergoing percutaneous closure with indicated according to the current ESC guidelines.

Methods
Twenty-three patients (mean age 42.9±12.4 years; 15 female) underwent right heart catheterization in basal conditions and after FCT (volume loading with rapid saline infusion of 7 ml/kg in 10 min) before percutaneous closure of the ASD.
Right atrial pressure (RAP), systolic, mean and diastolic pulmonary arterial pressure (sPAP, mPAP and dPAP), pulmonary arterial wedge pressure (PAWP), cardiac output (CO), pulmonary vascular resistance (PVR), systemic vascular resistance (SVR) and the ratio between pulmonary and systemic flow (QP/QS) were calculated four times: before and after inflating the sizing balloon both at baseline and immediately after FCT (Fig. 1).

Results
The patients had an increase in pressures and flows after FCT with open ASD: mPAP (18.7±4.4 vs 16.7±4.6 mmHg, p&lt;0.001), PAWP (11.3±3.1 vs 9.2±3.0 mmHg, p&lt;0.001), QP (12.5±2.3 vs 10.3±2.0 l/min, p&lt;0.001), and QS (6.6±1.4 vs 5.9±1.2 l/min, p&lt;0.001) but RAP remained unchanged (8.7±3.0 vs 8.3±2.4 mmHg, p=0.35). PVR (0.2±0.4 vs 0.8±0.3 Wood Units, p&lt;0.001) and SVR (11.2±3.2 vs 12.5±3.2 Wood Units, p=0.02) decreased, and PVR/SVR (0.06±0.02 vs 0.06±0.3, p=0.25) remained unchanged. QP/QS increased in all patients after FCT (mean±SD: 2.0±0.4 vs 1.8±0.4, p&lt;0.001).
During a temporary ASD closure by sizing balloon, the patients had increases of RAP (9.0±2.6 vs 7.6±2.6 mmHg, p&lt;0.001) mPAP (19.5±4.0 vs 17.4±3.7 mmHg, p&lt;0.001), PAWP (13.2±2.1 vs 11.2±2.9 mmHg, p&lt;0.001), and CO (7.7±2.7 vs 6.8±2.3 l/min, p&lt;0.001) after FCT. PVR remained unchanged (0.9±0.4 vs 1.1±0.6, p=0.12) and SVR reduced (9.8±2.7 vs 11.3±2.9, p&lt;0.001) after FCT.

Conclusions
None of the reported ASD patients presented with FCT criteria of post-capillary PH (that is a PAWP &gt;18 mmHg). The FCT was associated with an increase in QP/QS suggesting that the patients still had a distensible pulmonary circulation. Further studies are needed to explore the relevance of a FCT in ASD patients, particularly those with higher PVR values.
Figure 1

Funding Acknowledgement
Type of funding source: None
Long-term outcome of adult patients with total anomalous pulmonary venous connection: data from the SACHER registry and a French center
Authors: Touray M , Bouchardy J , Schwerzmann M , Greutmann M , Tobler D , Engel R , Harald G , Pruvot E , Blanche C , Sekarski N , Ladouceur M , Rutz T 
Published: November 2020

Abstract

Introduction
Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease, where all pulmonary veins aberrantly connect to a systemic vein or the right atrium. The only curative treatment is surgery allowing the patients to reach adulthood. This study describes the long-term outcome of these individuals focusing on arrhythmias.

Methods
Clinical, surgical, imaging and invasive data were retrospectively reviewed from 7 centers participating in the Swiss Adult Congenital Heart disease Registry (SACHER) and one French center.

Results
A total of 57 patients were identified and analyzed 22±8 years after surgery.
At last follow-up, 21% of patients presented cardiac symptoms, mainly palpitations. No patient had pulmonary hypertension or a relevant valvulopathy. Echocardiography revealed in 5 (8.8%) patients a dilated right ventricle (RV) and in 3 (5.3%) patients a diminished RV systolic function. Exercise capacity was normal in most patients (table). Cardiac magnetic resonance imaging found in 2 (4%) had a residual shunt with an mean Qp:Qs of 1.25, due to a single anomalously connected pulmonary vein.
Holter recordings revealed arrhythmias in 23% of patient. Ten (17.5%) had atrial fibrillation, flutter or tachycardia. Three (5%) patients presented ventricular arrhythmias: two patients showed non-sustained ventricular tachycardia and one patient complex ventricular extrasystoles. Four patients (7%) were on antiarrhythmic medication for supraventricular arrhythmias. Three patients (5%) underwent an electrophysiological study with a mean time since surgery of 20 years. Three (5%) patients underwent pacemaker implantation within 3 weeks to 36 months after surgical correction, which were removed in 2 patients after 7 years.
Age and the presence of a valvulopathy at follow-up predicted tachyarrhythmia on binomial logistic regression analysis (p&lt;0.03).

Conclusions
In adult survivors after TAPVC repair, supraventricular but also ventricular arrhythmias are frequently observed which appears to be related to age and valvular lesions at follow-up. This study underlies the importance of long-term follow-up as some of the patients currently without arrhythmia will probably develop rhythm disorders in the future.

Funding Acknowledgement
Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant by Actelion SA, Switzerland