bluuebunny/crossref_metadata_2025 huggingface dataset:
What happens >10 years after percutaneous septal ablation for hypertrophic obstructive cardiomyopathy? Ultra long-term follow-up of 120 patients
Authors: Faber L , Scholtz S , Batzner A , Seggewiss H
Published: November 2020

Introduction and methods

Recent registry analyses including the European multicenter registry (Euro-ASA) documented a favourable long-term outcome of septal ablation (ASA/PTSMA) competitive to surgical myectomy. There is no information about the period beyond 10 years. We therefore analyzed the outcome in 120 HOCM patients (pts., mean age @ index intervention: 62±13 years) treated with ASA who survived >10 years.

Results

Mean CK rise during the index intervention was 537±258 U/l (reference: <80). A DDD-pacemaker (DDD-PM) had to be implanted in 26 pts. (22%) for procedure-related AV conduction problems. A re-intervention for residual or recurrent outflow obstruction (LVOTO) had to be performed in 12 pts. (10%; re-PTSMA: 10 pts.; myectomy: 2 pts). These cases included, at their last follow-up visit 107 pts. (89%) were in functional class I or II. During follow-up (159±33 [range: 122–264] months), 12 pts. (8%) died, of these 6 (4%) from non-cardiac, and 6 (4%) from cardiovascular causes including one sudden death. Furthermore, 4 (3%) ICDs were implanted (1 for secondary, 3 for primary prevention of sudden cardiac death). The most frequent clinical problem was atrial fibrillation which occurred in 27 pts. (23%), and which was refractory to rhythm control attempts in 21 pts. (18%).

Conclusions

The procedure-related DDD-PM rate in this septal ablation cohort including the early learning curve more than doubled the current one. Nevertheless, during ultra long-term follow up a durable clinical improvement was observed with mortality rates around 1%/year, nearly equivalent to that of 0.8% observed in normal populations. Atrial fibrillation is a frequent problem in this elderly, formerly obstructive HCM population.

Funding Acknowledgement

Type of funding source: None
The influence of age on the diagnostic yield of genetic testing in dilated cardiomyopathy
Authors: Cannie D.E , Protonotarios A , Lorenzini M , Akhtar M , Syrris P , Lopes L , Elliott P
Published: November 2020

Background

Dilated cardiomyopathy (DCM) has an estimated population prevalence of 1/250 and is the underlying diagnosis in a third of heart failure patients. A substantial proportion of patients have familial disease caused by dominant mutations in one of more than 50 genes, but clinical practice guidelines recommend genetic testing in young patients with idiopathic DCM. There is an absence of robust data on the influence of age on the diagnostic yield of genetic testing.

Methods

The study cohort comprised 825 consecutive and unrelated patients (524 male (63.5%)) with DCM who underwent genetic testing from 2015 to 2019. Genetic variants were classified using American College of Medical Genetics (ACMG) criteria. Analyses were stratified by age and sex.

Results

173 (20.1%) patients had a positive genetic test (“pathogenic” or “likely pathogenic” variant); 292 (34.4%) had a variant of unknown significance. Mean age at genetic testing was 49.9±14.4 years. Mean age of patients with a positive test was 47.6±13.6 years. 99 (18.9%) men and 67 (22.3%) women had a positive test (p=0.246).

Mutations in the TTN gene, encoding for titin, accounted for 46.1% of positive results. 13.8% of mutations were in DSP, 8.4% in RBM20, 6% in FLNC, 4.2% in LMNA, 3.6% in BAG3 and 3.6% in MYH7.

There was a trend to declining yield with age (likelihood ratio chi-square p value = 0.047). The yield was 17.2% in the 56–65 year age group and 11.5% above 66 years of age (figure 1).

Conclusions

Approximately 1 in 5 patients with DCM had a positive genetic test. The yield declined in patients over 66 years but remained above 11%, suggesting that genetic testing should not be confined to younger patients with DCM.

Figure 1. Yield of Genetic Testing by Age

Funding Acknowledgement

Type of funding source: None
Prognostic impact of suspected cardiac amyloidosis in aortic stenosis patients referred for transcatheter aortic valve implantation
Authors: Ferreira V , Aguiar Rosa S , Rodrigues I , Moura Branco L , Galrinho A , Rio P , Patricio L , Cacela D , Ramos R , Mendonca T , Castelo A , Garcia Bras P , Mano T , Viegas J , Cruz Ferreira R
Published: November 2020

Background

The prevalence of cardiac amyloidosis (CA) and aortic stenosis (AS) both increase with age. Transcatheter aortic valve implantation (TAVI) expands the number of patients (P) eligible for treatment of AS, emphasizing the need to understand the prevalence of CA in AS and its prognostic associations. Echocardiography with speckle tracking has emerged as a useful method to enhance the clinical suspicion and to provide prognostic information.

Purpose

To estimate the prevalence of CA in P with severe AS referred for TAVI and to evaluate the impact of concomitant CA in prognosis.

Methods

94 consecutive AS P who underwent TAVI with maximum left ventricular wall thickness (LVWT)>12 mm were retrospectively identified. Clinical data, pre TAVI echocardiographic parameters and follow up (FU) data regarding all-cause mortality and MACE (including all-cause mortality, admission for heart failure, pacemaker implantation and stroke) were analysed. We registered apical sparing pattern in bull's eye plots (ASPB), calculated relative apical longitudinal strain formula (RALS) [average apical LS/(average basal LS + mid-LS)] and ejection fraction/global longitudinal strain (EF/GLS) ratio.

Results

Mean age was 82.2±5.8 years (Y), with 43 men (45.7%). 27.7% were in NYHA functional class II, 64.9% in functional class III and 7.4% in functional class IV. Median EF was 57±15% and 26.6% presented EF<50%. Suspected CA evaluated by ASPB was found in 39 P (41.5%) and RALS >1 was identified in 22 P (23.4%). An EF/GLS ratio >4.1 was obtained in 53 P (56.4%). Over a median follow-up of 13.4±25.8 months, 28 deaths (29.8%) and 31 MACEs (33.0%) occurred.

The presence of ASPB was associated with increased all-cause mortality (33.3% vs. 5.6%, p=0.002) and MACE (48.7% vs 22.2%, p=0.01). RALS>1 correlated also with all-cause mortality (31.8% vs. 12.5%, p=0.04) and with new bundle branch block and indication for pacemaker implantation (46.2% vs 37.0%, p=0.05). P with GLS>−14.8% and ASPB had significantly worse prognosis regarding all-cause mortality (p=0.003) and MACE (p=0.007). Kaplan–Meier survival analysis showed that survival was significantly worse for P with ASPB (log-rank 0.002). With multivariate Cox regression analysis, ASPB was independently associated with all-cause mortality (HR=4.49, p=0.039).

Conclusions

Suspected CA appears prevalent among patients with AS and associates with all-cause mortality. The importance of screening for CA in older AS patients and optimal treatment strategies in those with CA warrant further investigation, especially in the era of transcatheter aortic valve implantation.

Funding Acknowledgement

Type of funding source: None
Iatrogenic transthyretin cardiac amyloidosis after sequential liver transplantation
Authors: Costa R , Rodrigues P , Felix R , Oliveira M , Frias A , Campinas A , Santos M , Reis H , Torres S
Published: November 2020

Introduction

Sequential liver transplantation (SLT) uses livers excised from patients with hereditary transthyretin-related amyloidosis during liver transplantation as grafts to other patients with severe hepatic pathologies and a reserved prognosis. We intended to investigate the development of cardiac manifestations consistent with iatrogenic transthyretin amyloidosis (iATTR).

Methods

We retrospectively analyzed the medical records of 72 consecutive patients submitted to SLT between 2007 and 2010, who received livers with V30M mutation.

Results

Our sample had 79% male patients and a mean age at transplantation of 55±6 years. Median follow-up time was 80 months, were 44% of the patients died. One-year mortality rate after SLT was 7%. Clinical manifestations of iATTR occurred in 29% of individuals, on average 6 years after SLT, and amyloid was seen in 76% of those who underwent a biopsy. Left ventricular hypertrophy (LVH) was identified in 42 (58%) patients at baseline. Considering 39 patients that had an echocardiography at baseline and during follow-up, 22 (61%) presented de novo LVH or basal LVH worsening during follow-up, with a significant increase of wall thickness (11±1 to 13±3 mm; p<0.001). They had similar age at presentation (55±5 vs 58±5, p=0.249) and incidence of hypertension (52% vs 64%, p=0.365) but higher incidence of chronic kidney disease (CKD; 68% vs 29%, p=0.023). Mortality during follow-up was higher in patients with de novo LVH or worsening LVH but not significantly, probably due to the sample size (23% vs 7%, p=0.221, log rank test p=0.262). Considering the global sample, significant conduction changes were rarely seen (1 patient); however, there was a trend towards an increase in PR interval and atrial fibrillation was reported in 8% of cases.

Conclusions

In our sample, probable iATTR was often seen within a decade after SLT. Further investigation of LVH needs to be made in these patients, as it can represent amyloid cardiomyopathy, but other contributing factors such as hypertension, CKD and age need to be taken into consideration. In our sample, development of a possible infiltrative pattern was relatively more common and conduction disorders were rarer than one would extrapolate from hereditary early onset ATTR V30M patients. Further studies may help us clarify if indeed these patients behave like late onset ATTR V30M. Our data suggests that these patients should probably undergo periodic cardiac imaging during follow-up.

Funding Acknowledgement

Type of funding source: None
Sarcoid heart disease: rates of arrhythmias, implantable cardiac devices and endomyocardial biopsy
Authors: Gonuguntla K , Patil S.P , Rojulpote C , Borja Z.E , Bravo P.E
Published: November 2020

Introduction/Background

Sarcoidosis is a granulomatous disease with various cardiac manifestations such as arrhythmias, heart block, heart failure and sudden cardiac death.

Objective

We sought to determine and compare the rates of arrhythmias, implantable cardiac devices in patient with sarcoid heart disease (SHD) after excluding those with a history of coronary artery disease

Methods

The United States Nationwide Inpatient Sample was queried from 2010 to 2014 using ICD-9 diagnosis code (135) for sarcoidosis among patients >18 years. We combined it with code (425.8) for cardiomyopathy in others disease including sarcoid and sarcoid heart muscle disease. We excluded patients with a history of prior myocardial infarction, percutaneous coronary intervention and coronary artery bypass graft.

Results

From 2010 to 2014, we identified 9,063 patients with SHD (Mage = 53.11±11.28 years; men 51.8% and black 52.5%). The overall in-hospital mortality rate was 227 (2.5%). Average Length of stay (LOS) (M ± SD) was 6.15±8.964. Various arrhythmias include atrial fibrillation 1,659 (18.4%), ventricular fibrillation 223 (2.5%), ventricular tachycardia 2,248 (24.7%), complete heart block 506 (1.5%) and second degree Mobitz type II 45 (0.5%). Rates of implantable cardiac devices included implantable cardioverter-defibrillator (ICD) 881 (9.7%), Cardiac Resynchronization Therapy Defibrillator (CRT-D) 83 (3.6%), PPM 172 (1.9%). Rates of Endomyocardial biopsy were 257 (2.8%). Overall rates of sudden cardiac arrest was 161 (1.8%). On comparing the two groups, sarcoidosis without cardiac involvement and sarcoid heart disease the rates of arrhythmias, implantable cardiac devices, in-hospital mortality and sudden cardiac arrest were as follows: atrial fibrillation (9.4 vs 18.3; p<0.001), ventricular fibrillation (0.1 vs 2.5; p<0.001), ventricular tachycardia (1.3 vs 24.8; p<0.001), complete heart block were (0.3 vs 5.6; p<0.001), and second degree Mobitz type II (0.1 vs 0.5; p<0.001), Pacemaker (0.4 vs 1.9; p<0.001), ICD (0.2 vs 9.7; p<0.001), CRT-D (0.1 vs 3.6; p<0.001), in-hospital mortality (2.5 vs 2.5; p=0.8), Sudden cardiac arrest (0.6 vs 1.8; p<0.001).

Conclusions

Based on the results of our study, we conclude that SHD is associated with a variety of cardiac arrhythmias and conduction defects. Sarcoidosis with cardiac involvement is associated with increased rates of atrial fibrillation, ventricular fibrillation, ventricular tachycardia, complete heart block, and second degree Mobitz type II. They also had a higher rate of pacemaker implantation, ICD, CRT-D and had a overall higher rate of sudden cardiac arrest. Based on the results of our study we conclude that sarcoidosis with cardiac involvement has worse outcomes and hence, early diagnosis and prompt treatment can prevent the progression of the disease.

Funding Acknowledgement

Type of funding source: None
Utility of updated diagnositc criteria to detect isolated cardiac sarcoidosis
Authors: Sato K , Yamamoto M , Ishizu T , Ieda M
Published: November 2020

Background

Prior study reported around one-third of cardiac sarcoidosis (CS) are considered as isolated CS. Detection of CS is challenging due to the limited sensitivity of endomyocardial biopsy and applicability of guidelines, especially in patients without extra-cardiac involvement. Existing diagnostic criteria by Japanese Ministry of health and Welfare (JMHW) or Heart Rhythm Society (HRS) require the presence of extra-cardiac sarcoidosis for clinical diagnosis, isolated CS is not diagnosable in the absence of a positive histological finding. Recently, Japanese Society of Cardiology (JCS) updated diagnostic criteria for CS, which provides the pathway to diagnose isolated CS.

Purpose

We aimed to assess the reliability of the updated CS guideline in diagnosing CS compared to the prior guidelines.

Methods

We retrospectively identified 162 consecutive patients who underwent FDG-PET for suspected CS from 2012 through 2019. According to the updated JCS diagnostic criteria, patients were classified as histologic diagnosis of CS, clinical diagnosis of CS, or isolated CS (Figure A). We compared the association between diagnostic criteria and response with anti-inflammatory therapy.

Results

The JCS criteria classified 24 patients (15%) as having clinical CS, 4 (3%) as histological diagnosis of CS, and 21 (13%) as isolated CS. The JMHW criteria defined 22 patients (14%) as having CS (clinical 11%, histological 3%) and HRS criteria classified 11 patients (7%) as having CS (clinical 4%, histological 3%). Extra-cardiac involvement was detected in 36 patients (22%) with 8% of histological confirmation. Among the 126 patients without extra-cardiac involvement, prevalence of cardiac involvement was higher in isolated CS (P<0.05 for all). Compared with clinical diagnosis group, patients with isolated CS showed higher incident of regional wall motion abnormality (WMA) or left ventricular (LV) dysfunction (p=0.023). In the subgroup of 45 patients with serial FDG-PET evaluation, only updated CS criteria was associate with improvement in myocardial inflammation by FDG-PET (p<0.001).

Conclusions

Updated JCS diagnostic criteria detects CS patients with active myocardial inflammation which require anti-inflammatory therapy regardless of extra-cardiac involvement better than the prior guidelines.

Diagnostic criteria for CS

Funding Acknowledgement

Type of funding source: None
Association between the elevation of biomarkers and incipient alterations in ventricular function in patients with Chagas disease without evidence of specific organ damage
Authors: Chirino D , Dizeo C , Volverg V , Toledo G , Vecchio V , Leonardi M.S , Calderon G
Published: November 2020

Background

Chagas disease (ChD) has a long asymptomatic period, where there is no evidence of myocardial damage. However, incipient alterations in ventricular systo-diastolic function have been described using echocardiography with Tissue Doppler and Strain. On the other hand, the presence of elevated higth-sensitivity T troponin (HS-TnT) and N-terminal pro B-type natriuretic peptide (NT-pro BNP) has been documented in the early stages of the ChD. The association between the elevation of both markers and incipient alterations in ventricular function in patients with ChD without evidence of heart damage has been poorly studied.

Purpose

The aim of this study is to evaluate the prevalence of elevation of both biomarkers (HS-TnT and NT-proBNP) and their association with insipients alterations in systo-diastolic function in patients with ChD without evidence of structural heart affection.

Materials and methods

Outpatients with stage 0 of ChD (positive serology, with normal electrocardiogram, holter and chest teleradiography) were included prospectively. They were divided in three groups according to the dosage of HS-TnT and NT-proBNP. Group 1: patients with both markers within normal values; group 2: patients with elevation of one of the markers (HS-TnT>13 ng/L OR NT-proBNP>125 pg/ml) and group 3: patients with elevation of both markers (HS-TnT>13 ng/L AND NT-proBNP>125 pg/ml). All of them underwent Doppler echocardiography with tissue Doppler.

Results

Two hundred and sixty-one patients were included, with 47±9 years old of age, 44% woman, 86.6% (n=226) in group 1, 9.6% (n=25) in group 2 and 3.8% (n=10) in group 3. Table 1 shows the main results.

Conclusions

Elevation of both markers was found in almost 4% of patients. This elevation was associated with a higher E/e' ratio, E/A ratio, Letf Atrial Area and a lower S'-wave lateral.

Funding Acknowledgement

Type of funding source: None
Hydroxychloroquine for colchicine-resistant glucocorticoid-dependent idiopathic recurrent pericarditis: an observational prospective study
Authors: Lazaros G , Antonopoulos A , Azzu A , Antonatou A , Skendros P , Ritis K , Hadziyiannis E , Lazarou E , Leontsinis I , Simantiris S , Vlachopoulos C , Tousoulis D , Vassilopoulos D
Published: November 2020

Background

Glucocorticoid (GC)-dependent, colchicine-resistant idiopathic recurrent pericarditis (IRP) remains a clinical challenge. We assessed for the first time the efficacy and safety of hydroxychloroquine (HCQ) in IRP.

Methods

This is a single center, post hoc analysis of prospectively collected data of 15 patients with refractory to standard therapy (colchicine plus either GC or anakinra) IRP (≥3 recurrences, disease duration ≥12 months and inability to wean off treatment) treated with HCQ (400 mg/day). These patients were matched 1:1 for age, sex, and treatment type to IRP patients receiving standard-of-care treatment (control group, n=15). Pericarditis recurrence, the time and C-reactive protein (CRP) levels at 1st flare, the % of patients able to achieve a ≥50% reduction of baseline GC dose and the % reduction of GC dose were compared between groups.

Results

Almost all patients (n=29) but one in the HCQ group (14/15) relapsed during follow-up. However, HCQ treatment was associated with an increased median time of flare-free survival (increase by 4 weeks compared to controls) and reduced hazard ratio for flare in survival analysis (HR=0.36, 95% CI 0.16–0.77, p=0.009). HCQ was also associated with a higher proportion of patients obtaining a ≥50% dose reduction of GCs (33.3% vs. 0% in the control group, p=0.037) and reduced GC dose (HCQ: −43.5% vs. control: −4.5%, p<0.001). There were no signficant differences in CRP levels at flare between groups (p=0.615).

Conclusions

In this prospective study, HCQ use was associated with a GC-sparing effect and an increased flare-free survival period in patients with colchicine resistant GC-dependent IRP.

Funding Acknowledgement

Type of funding source: None
Long-term natural history of transient constrictive pericarditis
Authors: Ramchand J , Chahine J , Alnajjar H , Chetrit M , Cremer P , Johnston D , Kwon D , Jellis C , Klein A
Published: November 2020

Background

In recent years, there has been increasing recognition of a potentially-reversible, transient/ subacute form of constrictive pericarditis (CP). To date, studies have been small with lack of long-term longitudinal follow-up.

Purpose

We aimed to elucidate the causes and natural history of subacute CP.

Methods

Patients were included if (1) they had a diagnosis of CP, (2) had cardiac magnetic resonance (CMR) within 12 months of symptom onset with evidence of pericardial delayed enhancement/ inflammation (Figure) (3) received anti-inflammatory medications.

Results

A total of 78 individuals were included, comprising 61 men (78%) with a mean age of 59±14 years. Causes of subacute CP included idiopathic/ viral pericarditis (58%), post-pericardiotomy (29%), autoimmune (6%), radiation therapy (3%) and others (4%). After median follow-up of 4.4 years, 31 (40%) required pericardiectomy. There were no deaths.

Patients who underwent pericardiectomy had longer duration of symptoms at presentation [6 (4–9) vs. 3 (2–5) months, P<0.01], were more likely to be on diuretic therapy (87 vs. 45%, P<0.001), had lower ultra-sensitive C-reactive protein [4.4 (2.6–13.1) vs. 11.95 (1.8–61.55) mg/dl, P<0.001] and lower erythrocyte sedimentation rate [5 (2–10) vs. 25 (6–43 mm/hr), P=0.031] compared to those who were managed medically. There were no other significant differences in clinical characteristics or baseline anti-inflammatory therapy. The presence of elevated inflammatory markers (HR: 0.18; 95% CI: 0.06–0.58, P<0.01) was an independent predictor of freedom from pericardiectomy after adjustment for relevant clinical and imaging parameters.

Conclusions

We present the original observations of the largest cohort of patients with transient CP to date and demonstrate that increased inflammatory markers were independently associated with long-term freedom from pericardiectomy. Our results suggest that a trial of anti-inflammatory therapy in the setting of elevated inflammatory markers may be appropriate prior to referral for surgery given the possible reversibility.

Pericardial delayed enhancement

Funding Acknowledgement

Type of funding source: None
Pretriscuspid shunt and Eisenmenger syndrome: a deadly combination
Authors: Playan Escribano J , Segura De La Cal T , Dos Subira L , Lazaro Salvador M , Barbera Mir J.A , Garrido-Lestache E , Otero Gonzalez I , Martinez-Quintana E , Lopez Ramon M , Martinez Menaca A , Molina Ferragut L , Suberviola V , Lopez Reyes R , Barrios Garrido-Lestache E , Escribano Subias P
Published: November 2020

Introduction

Pulmonary arterial hypertension (PAH) is a common comorbidity in congenital heart disease (CHD) and significantly affects prognosis. Our aim was to investigate how the location of the shunt influences the outcome of these patients.

Methods

The REHAP registry is a Spanish multicentre voluntary registry of patients over 14 years of age, which includes patients with congenital heart disease and PAH, starting in 2007. Baseline characteristics, functional class, right catheterization data, treatment and survival were compared. We performed a cox regression model for assessing the impact of the type of shunt on prognosis, after looking for confusing and effect modifying variables.

Results

664 patients were analyzed, their characteristics are detailed in the table. Mean follow up time was 10 years. We performed a survival analysis adjusted for the confounding variables: age (Hazard ratio (HR) 1.02 p<0.001), functional class (HR 2.45 p 0.001) and clinical group. The clinical group was also an effect-modifying variable, which means there is no unique effect (HR) of the type of shunt, instead it depends on the clinical group. We did a multiple regression analysis, adjusting for the confounding variables, to calculate the HR for each clinical group: in patients with Eisenmenger syndrome, the HR is 0.27 (p<0.001) for simple posttricuspid shunt and HR 0.38 (p<0.001) for complex posttricuspid shunt (reference group: pretricuspid shunt). In the others clinical groups, the type of shunt did not reach statistical significance.

Conclusion

The type of shunt significantly affects prognosis in Eisenmenger patients, with a much worse outcome for those with pretricuspid shunts. Functional class and clinical group are also closely related to outcome.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): MSD, GSK
Transcatheter intervention of coarctation of the aorta (CoA): a multinational population-based registry – effect on hypertension
Authors: Eriksson P , Pihkala J , Jensen A.S , Dohlen G , Liuba P , Wahlander H , Sjoberg G , Hlebowicz J , Furenas E , Dellborg M , Settergren M , Kudsk-Nielsen J.E , Sondergaard L , Sinisalo J , Larsen S.H
Published: November 2020

Background

CoA is associated with hypertension caused by reduced wind kessel function in the aortic arch, general hypoplasia of the arch and/or essential hypertension. In patients with a native or recurrent/rest CoA, a gradient >20 mmHg by non-invasive meassurement if associated with hypertension is an ESCguideline indication for intervention. We studied the persistence and presence of hypertension after transcatheter intervention of a CoA

Methods

All consecutive patients undergoing catheter interventions for CoA from 1st of January 2000 to 31st of December 2016 were identified by each of the particpating nine centers. The nine centers perform all catheter interventions for CoA for a complete population coverage of 25 millions inhabitants. A common protocoll was filled out from medical records. Hypertension was defined as a pre-intervention blood pressure above 140/80 or pharmacological treatment of hypertension. Exclusion criteria were weight less than 20 kg at the time of intervention or Norwood surgery

Results

590 interventions were performed on 520 patients: two interventions n=76, three: n=11, four n=2 and one patient underwent five interventions. Before intervention, 437 (74%) of the patients were hypertensive and 285 were on pharmacologocal treatment; 134 (48%) were treated with one drug, 79 patients (28%) with two drugs, 41 patients (15%) with three drugs and 14 (5%) with four drugs. After the intervention during follow up hypertension was present in 294 patients (50%, p<0.001 vs pre) of whom 270 (46%) were on pharmacological treatment; with one drug, n=128 (48%), two drugs n=93 (34%), three drugs n=34 (13%) or 4 drugs n=7 (3%).

Conclusions

Catheter intervention of CoA reduced the presence of hypertension significantly from 74% down to 50% but many patients will remain hypertensive and in need for treatment. Life time follow up also after transcatheter CoA intervention seems warranted.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): ALF-LUA, Heart and Lung Foundation
The benefit of atrial septal defect closure in the elderly patients
Authors: Prochownik P.J , Przewlocki T , Sobien B , Gancarczyk U , Olszowska M , Podolec P , Komar M
Published: November 2020

Objective

Closure of the atrial septal defect in the elderly patients is controversial. The aim of the study was to evaluate the outcomes of transcatheter closure of secundum atrial septal defect (ASD) in elderly patients.

Methods

From a total of 650 pts with ASD who underwent transcatheter closure 120 pts over 60 years (70 F, 50 M) with a mean age of 66.7±18.1 (60–78) were analyzed. All patients had an isolated secundum ASD with a mean Qp:Qs: 2.79±1.8 (1.5–3.9). A symptom-limited treadmill exercise tests with respiratory gas exchange analysis and transthoracic color Doppler echocardiographic study as well as Quality of life (QoL) measured using the SF36 questionnaire (SF36q) were repeated in all pts before procedure and after 12 months of follow-up.

Results

The device was successfully implanted in all pts (procedure time 30.1±9.9 (10–59) minutes, fluoroscopy time 10.1±7.2 (6–40) minutes). There were no major complications. The defect echo diameter was 19.5±15.4 (12 - 34) mm. The mean balloon stretched diameter of ASD was 22.0±7.8 (14 - 36) mm. The diameter of the implanted devices ranged 16 - 38 mm. After 12 months of ASD closure, all the pts showed a significant improvement of exercise capacity parameters. Seven QoL parameters (except mental health) improved at 12 months follow up compared to their baseline data. The mean SF36q scale increased significantly in 96 (80.2%) pts of mean 41.6±26.1 (4–71). The right ventricular dimension decreased in 101 pts (84.2%) (Table 1).

Conclusions

Closure of ASD in elderly patients caused a significant clinical and hemodynamic improvement after percutaneous treatment, which is maintained to long-term follow-up what justified this procedure in old age.

Funding Acknowledgement

Type of funding source: None
Mechanical-electrical interaction of the right ventricle in patients with repaired tetralogy of fallot: the next step towards resynchronization in congenital heart disease
Authors: Rodriguez Garcia J , Pijuan Domenech A , Perez Rodon J , Benito Villabriga B , Francisco Pascual J , Santos Ortega A , Dos Subira L , Subirana Domenech M , Miranda Barrio B , Gordon Ramirez B , Ferreira Gonzalez I , Rivas Gandara N
Published: November 2020

Introduction

Patients with repaired tetralogy of Fallot (rTF) and severe pulmonary regurgitation frequently progress to dilation and dysfunction of the right ventricle (RV). It has been documented in the literature that there is a correlation between the duration of the QRS in the surface electrocardiogram and the hemodynamic parameters of the RV of these patients, suggesting the presence of a mechanical-electrical interaction.

Purpose

To determine if there is an association between the contraction delay in certain areas of the RV measured in M-mode echocardiography and the delay in electrical activation measured in the electroanatomic map (EAM) of RV in patients with rTF.

Methods

Unicentric and observational study of all patients with rTF undergoing EAM, echocardiography with study of RV asynchrony and cardiac magnetic resonance imaging (MRI). Activation delay in the antero-basal area and in the RV outflow tract (RVOT) in the EAM were both analysed (Figure 1A). The shortening delay in the same areas in M-mode echocardiography was also evaluated (Figure 1B, C). MRI data regarding volume and ejection fraction was also collected.

Results

64 patients were included (36.7±10.6 years, 65% men). The mean total activation time of the RV (RV-TAT) was 127.3±42.4 ms. Activation mapping showed a recurrent pattern with beginning in the interventricular septum and ending in RV antero-basal region and/or RVOT. A linear positive correlation was observed between RV-TAT and the activation delay in both regions analysed (ρ=0.60 and ρ=0.52, respectively; p<0.001) and also between the electrical and mechanical delay in the anterior wall (ρ=0.41; p=0.001). On the other hand, it was observed a negative correlation between RV ejection fraction (RVEF), measured on MRI, and the RV-TAT (ρ=−0.41, p=0.002) and also between RVEF and the activation delay in the RV antero-basal region and in the RVOT (ρ=−0.32, p=0.016 and ρ=−0.36, p=0.007, respectively).

Conclusions

There is a mechanical-electrical interaction in the RV of patients with rTF, with a negative correlation between the activation delay and RVEF and between mechanical and electrical activation delay in specific anatomical regions (regional mechanical-electrical interaction). These results may guide future studies on resynchronization in this heart disease.

Figure 1. EAM and echocardiographic measures

Funding Acknowledgement

Type of funding source: None
Contribution of peptidyl prolyl isomerase (Pin1) to development of pulmonary hypertension via pulmonary vascular endothelial cell dysfunction
Authors: Sakai S , Maruyama H , Ieda M
Published: November 2020

Background

Endothelial dysfunction is thought to be a major contributor to overall pathogenesis of vasculopathy seen in pulmonary hypertension (PH), which is manifested by the impaired release of nitric oxide (NO) generated through endothelial nitric oxide synthase (eNOS) in endothelial cells. Activation of human eNOS is regulated by phosphorylation at multiple sites including Thr33 and Ser114, which residues are followed by Pro. The peptidyl isomerase Pin1 specifically isomerizes the phospho-protein having Ser/Thr-Pro bond and regulates their activity. Pin1 is involved in proliferation, cell cycle, and apoptosis in cancer, by isomerizing some functional molecules such as JNK, JUN, cyclin D, BAX, etc. However, it is controversial whether direct interaction of Pin1 with eNOS and how eNOS activity is altered by Pin1, especially in PH.

Purpose

We aimed to clarify whether Pin1 contributes to the PH development using Pin1 knockout mice and Pin1 affects the expression of phosphorylated eNOS (p-eNOS) molecule and pulmonary arterial endothelial cell (PAEC) apoptosis.

Methods and results

Wild (WT) and Pin1-deficient mice (KO) were exposed to hypoxia (10% O2) or normoxia for 3 weeks to generate hypoxia-induced PH. Hypoxia-inducible factor (HIF1α) expression in lungs was significantly enhanced in WT-hypoxia (WH, n=6) and KO-hypoxia (KH, n=6), suggesting that hypoxic response was certainly occurred in these mice. Pulmonary arterial pressure did not elevate in KH compared with KO-normoxia (KN, n=6) and WT-normoxia (WN, n=6), it was significantly increased only in WH (P<0.01), indicating that KO did not develop PH by hypoxia. The gain of RV weight was parallel to the increase of pulmonary arterial pressure. Western blot showed that p-eNOS expression in lungs was significantly decreased in WH compared to WN, however, the expression was not different between KH and KN. It suggests that Pin1 plays a regulatory role in p-eNOS expression in hypoxic response. In cultured PAECs, the expression of p-eNOS and eNOS was markedly increased by siRNA-mediated Pin1 knockdown. Immunoprecipitation study showed the possibility of Pin1 binding to p-eNOS molecule. Apoptosis evaluated by caspase-3/7 activity by fluorescent assay and cleaved caspase-3 expression by Western blot was significantly increased by Pin1 overexpression in PAECs; however, it was significantly decreased by Pin1 knockdown. Moreover, the exaggeration of apoptosis induced by doxorubicin was markedly increased by Pin1 overexpression compared with control in PAECs; however, it was clearly suppressed by Pin1 knockdown.

Conclusion

This study suggests that endogenous Pin1 contributes to the development of PH partly via the dysfunction of PAECs, that is, by the interference with p-eNOS expression and by the increase of apoptosis inducibility to external stimuli.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): JSPS KAKENHI
Prognostic value of pulmonary artery diameter in patients with pulmonary arterial hypertension
Authors: Zuffa E , Dardi F , Saia F , Niro F , Palazzini M , Russo V , Taglieri N , Lovato L , De Lorenzis A , Pasca F , Guarino D , Magnani I , Marrozzini C , Manes A , Galie N
Published: November 2020

Background

Pulmonary artery (PA) dilation is common in pulmonary arterial hypertension (PAH) and may cause left main coronary artery (LMCA) compression. This complication have been sistematically evaluated in patients with angina but no evidence is available for patients asymptomatic for angina.

Purpose

To evaluate the prognostic value of PA diameter and the prevalence of LMCA compression in PAH patients with and without angina.

Methods

All patients with PAH and a baseline angio-CT scan were included. The last angio-CT scan performed was considered for PA diameter increase velocity calculation. Patients with angina or radiological signs of suspected LMCA compression underwent a coronary (cor)-CT scan. Patients with a not negative cor-CT underwent invasive coronary angiography. If clinically indicated invasive coronary angiography was performed without a preliminary cor-CT. To evaluate the predictivity for compression at coronary angiography the PA diameter at cor-CT or at the nearest angio-CT scan was considered.

Results

958 patients were included. PA diameter at baseline is higher in patients with congenital heart disease (CHD) and is not predictive of death. The velocity of PA diameter increase is lower in patients with connective tissue disease and is a risk factor for death in both overall population and different etiological subgroups except CHD and pulmonary veno-occlusive disease. 180 patients had angina and 50 had LMCA compression. 120 patients without angina underwent cor-CT and/or coronary angiography and 15 patients had LMCA compression. The best PA diameter cut-offs predicting LMCA compression in symptomatic/asymptomatic patients were, respectively, 40/42 mm (sensitivity and specificity, respectively, 80 and 72%/87 and 77%).

Conclusion

28% of patients with angina and 13% of patients without angina had LMCA compression. We found a similar PA diameter predicting LMCA compression in patients with or without angina. PA diameter, instead, was not predictive of prognosis (maybe because most deaths were related to heart failure and also because patients with LMCA compression were treated with stenting, possibly preventing sudden cardiac death). Eventually, PA diameter velocity increase is associated with prognosis but this may only reflect a severe disease refractory to medical therapy.

Funding Acknowledgement

Type of funding source: None
Identification of the optimal hsTnI cut-off value for risk stratification of normotensive patients with pulmonary embolism
Authors: Ebner M , Guddat N , Keller K , Merten M.C , Lerchbaumer M.H , Hasenfuss G , Konstantinides S.V , Lankeit M
Published: November 2020

Background/Introduction

While numerous studies confirmed the prognostic role of high-sensitivity troponin T (hsTnT) in pulmonary embolism (PE), the prognostic relevance of high-sensitivity troponin I (hsTnI) is inappropriately studied and disease specific cut-off values remain undefined.

Purpose

To investigate the prognostic relevance of hsTnI in normotensive PE patients, establish the optimal cut-off value for risk stratification and compare the prognostic performances of hsTnI and hsTnT.

Methods

Consecutive PE patients enrolled in a prospective single-centre registry between 09/2008 and 04/2018 were studied. Using receiver operating curve analysis, an optimised hsTnI cut-off concentration was identified and the prognostic value for the prediction of in-hospital adverse outcomes (PE-related death, cardiopulmonary resuscitation or vasopressor treatment) and all-cause mortality analysed.

Results

We analysed data from 459 PE patients (age 69 [interquartile range (IQR) 57–77] years, 52% female). Patients who suffered an in-hospital adverse outcome (4.8%) had higher median hsTnI concentrations compared to those with a favorable clinical course (57 [IQR 22–197] vs. 15 [IQR 10–86] pg/ml, p=0.03). A hsTnI cut-off value of 16 ng/ml provided the best prognostic performance and predicted an in-hospital adverse outcome (Odds ratio [OR] 6.5, 95% confidence interval [CI] 1.9–22.4) and all-cause mortality (OR 3.7, 95% CI 1.0–13.3). Between female and male patients, no relevant differences in hsTnI concentrations (17 [IQR 10–97] vs. 17 [IQR 10–92] pg/ml, p=0.79) or optimized cut-off values (17 pg/ml and 19 pg/ml, respectively) were observed. Stratification of patients to risk classes according to the 2019 European Society of Cardiology (ESC) algorithm revealed no differences if calculated based on either hsTnI or hsTnT (Table).

Conclusions

Our findings confirm the prognostic relevance of hsTnI in normotensive PE. An optimal hsTnI cut-off value of 16 pg/ml predicted in-hospital adverse outcome and all-cause mortality. The use of sex specific cut-off values does not appear necessary. Importantly, our results suggest that hsTnI and hsTnT can be used interchangeably for risk stratification.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study was supported by the German Federal Ministry of Education and Research (BMBF 01EO1503). BRAHMS GmbH, part of Thermo Fisher Scientific, Hennigsdorf/Berlin, Germany provided financial support for biomarker measurements. The sponsor was neither involved in biomarker measurements, statistical analyses, writing of the abstract nor had any influence on the scientific contents.
Deep learning for screening of pulmonary hypertension using standard chest X-ray
Authors: Hirata Y , Kusunose K , Yamaguchi N , Morita S , Nishio S , Okushi Y , Takahashi T , Yamada H , Tsuji T , Kotoku J , Sata M
Published: November 2020

Background

Early detection of pulmonary hypertension (PH) is crucial to ensure that patients receive timely treatment for the progressive clinical course. The chest X-ray (CXR), a routine method at hospitals, has recommended in order to reveal features supportive of a diagnosis of PH. However, it is well known that the sensitivity and specificity are low.

Purpose

We tested the hypothesis that application of artificial intelligence (AI) to the CXR could identify PH.

Methods

We retrospectively enrolled 900 data with paired CXR and right heart catheter (RHC), including the pulmonary artery pressure, from October 2009 to December 2018. We trained a convolutional neural network to identify patients with PH as actual value of pulmonary artery pressure, using the CXR alone (Figure). The diagnosis of PH was performed using hemodynamic measurements according to the most recent World Symposium standards: mean PAP ≥20 mmHg. We have compared the area under the curve (AUC) by human observers, measurements of CXR images, and AI for detection of PH.

Results

Subjects were divided into two groups with PH (439 patients; mean age, 66±14 years; 233 male) and without PH (461 patients; mean age, 68±12 years; 278 male). In an independent set, AI was the highest diagnostic ability for detection of PH (AUC: 0.71). The AUC by the AI algorithm was significantly higher than the AUC by measurements of CXR images and human observers (0.71 vs. 0.60 and vs. 0.63, all compared p<0.05).

Conclusion

Applying AI to the CXR (a classical, universal, low-cost test) permits the CXR images to serve as a powerful tool to screen for PH.

Neural network

Funding Acknowledgement

Type of funding source: None
Clinical and imaging predictors of recovery in patient with pulmonary emboli
Authors: Naghavi B , Alizadehasl A , Sadeghipour P , Moosavi J , Shafe O , Pouraliakbar H.R , Rezaei Kalantari K , Naghshbandi M , Farrashi M , Khansari N , Maleki M , Bakhshandeh H , Amin A , Talakoob H , Mohseni Salehi M
Published: November 2020

Background

Post-pulmonary emboli impairment (PPEI), comprised significant morbidity and mortality. A single center registry of patients with PE was established in order to estimate patients' risk, monitor clinical management and develop a simple model to assess the risk for PPEI for the entire spectrum of patients treated in general clinical practice.

Methods

For developing the mentioned model, apart from approved imaging parameters, potential novel imaging parameters were also evaluated. Recovery were assessed according to Pulmonary Embolism Thrombolysis (PEITHO) trial definition (Figure 1).

Results

140 patients have been registered with at least 6-month imaging follow up. PPEI was confirmed in 75 (53.6%) patients with PE. The prognostic value of traditional and novel imaging parameters on 6-month complete recovery were summarized in Table. A multivariable logistic regression model was developed using 140 patients. Previous PE, HRfollowing five in-hospital-parameters accounted for simple prognostic model for incomplete recovery: history of PE, heart rate≥110, serum blood urea nitrogen level, RV/LV ratio and right atrium area, The overall prediction ability was excellent with 80.6 overall percentage for PPEI.

Conclusions

PPPEI rate was considerable in our study. Our proposed simple prediction model might have a role on identification of patients at risk for PPEI.

Figure 1

Funding Acknowledgement

Type of funding source: None
Statins for venous thromboembolism prevention: old dog, new tricks
Authors: Sousa J.P , Puga L , Ribeiro J , Lopes J , Saleiro C , Gomes R , Campos D , Lourenco C , Goncalves L
Published: November 2020

Background

Statins are highly effective in preventing major acute cardiovascular events in the setting of atherosclerotic arterial disease. On the other hand, given their antithrombotic and anti-inflammatory properties, statins may also attenuate patients' odds of developing venous thromboembolism (VTE). However, clinical studies have yielded variable estimates of this effect.

Purpose

To perform a meta-analysis designed to evaluate the extent to which statin use influences the rate of subsequent VTE events.

Methods

We systematically searched MEDLINE, Embase, Web of Science, Cochrane Library and Google Scholar for both randomized controlled trials (RCTs) and observational studies addressing the association between statins and VTE risk, published up until December 1, 2019. Manually reviewed references and key investigators interaction via e-mail correspondence were also data sources. RCTs comparing the effects of statin therapy with those of a placebo or no treatment were included, while interventional studies appraising different lipid-lowering pharmacological strategies were not. Observational studies encompassed both cohort and case-control designs. The primary endpoints were general VTE, deep vein thrombosis or pulmonary embolism. Patients with cancer, heart failure and chronic kidney disease (CKD) were further investigated separately. Study-specific relative risks (RRs) were pooled using generic inverse variance outcome meta-analytic technique with a random-effects model.

Results

23 RCTs comprising 118.464 participants, 12 cohort studies encompassing 2.881.184 patients and 9 case-control studies including 354.367 patients were regarded as eligible for quantitative evaluation. Specifically, 5 observational studies comprising 9.656 cancer patients, 3 studies encompassing 9.693 heart failure patients and 4 studies including 4.353 CKD patients were gathered. In RCTs, statin therapy was proven slightly superior to placebo or no treatment in lowering VTE incidence (RR 0.85, 95% CI 0.73–0.99, p=0.04, i2=14%). Observational studies were found to corroborate this effect, with statin treatment resulting in VTE risk reduction overall (RR 0.72, 95% CI 0.64–0.81, p<0.001, i2=84%) and in both cohort (RR 0.86, 95% CI 0.83–0.90, p<0.001, i2=85%) and case-control (RR 0.68, 95% CI 0.57–0.82, p<0.001, i2=80%) designs. This positive effect held true in cancer patients (RR 0.56, 95% CI 0.33–0.95, p=0.03, i2=78%), but not in those with heart failure (RR 0.7, 95% CI 0.42–1.16, p=0.17, i2=2%) and CKD (RR 1.04, 95% CI 0.67–1.60, p=0.87, i2=0%).

Conclusion

Currently available evidence suggests that statins significantly reduce patients' odds of developing VTE. Given their favorable safety profile and low cost, statin treatment should now be considered in high-risk individuals, particularly in those with cancer.

Funding Acknowledgement

Type of funding source: None