bluuebunny/crossref_metadata_2025 huggingface dataset:
Outcomes of infective endocarditis in patients with end-stage renal disease and dialysis in Spain: a population-based study
Authors: Gomez-Ramirez D , Olmos C , Fernandez-Perez C , Del Prado N , Rosillo N , Bernal J L , Zulet P , Vilacosta I , Elola F J
Published: October 2024

Background

Patients with end-stage renal disease (ESRD), particularly those undergoing dialysis, have a significantly higher risk of developing infective endocarditis (IE) compared to the general population. ESRD is not only related to a higher risk of infection, but also to a worse prognosis in IE. However, most data regarding the epidemiology and outcomes of patients with ESRD and IE is derived from noncontemporary population-based studies with limited clinical variables and lack of complete microbiological information, or from retrospective single-center cohorts.

Purpose

The aim is to describe the clinical characteristics and outcome of patients with IE and ESRD in Spain, compared to patients without ESRD, and to investigate potential differences between patients undergoing dialysis and the rest of patients with ESRD.

Methods

Retrospective observational population-based study. A total of 9,008 episodes of IE between 2016 and 2019 were analysed using information from the Spanish Minimum Basic DataSet. 428 patients had ESRD, of whom 332 were undergoing dialysis. A multivariable and multilevel logistic model was developed to evaluate the association of different variables with in-hospital mortality in ESRD patients.

Results

Compared to patients without ESRD, those with ESRD were younger, had more comorbidities, and had a higher incidence of Staphylococcus aureus (31.8% vs. 18.4%; p <0.001) and coagulase-negative staphylococci infections (19.2% vs. 14%; p =0.006). In terms of in-hospital complications, septic shock was more frequent in patients with ESRD (12.1% vs. 8.9%; p=0.007). Furthermore, ESRD patients underwent cardiac surgery less frequently than patients without ESRD (12.6% vs. 19.6%; p <0.001) and had significantly higher in-hospital mortality (33.4% vs. 26.9%; p =0.003).

When comparing patients undergoing dialysis with the rest of ESRD patients, it was observed that those on dialysis had more comorbidities and a higher proportion of Staphylococcus aureus infections (36.1% vs. 16.7%; p <0.001).

No differences were found in observed in-hospital mortality between patients on dialysis and the rest of ESRD patients. The multilevel analysis revealed that dialysis was not an independent risk factor for in-hospital mortality in patients with ESRD. Cardiac surgery was also not independently associated with higher in-mortality in ESRD patients.

Conclusions

Patients with ESRD and IE have a different clinical and microbiological profile than other patients with IE. Moreover, they undergo cardiac surgery less frequently and have significantly higher in-hospital mortality. Dialysis treatment and cardiac surgery were not independent risk factors for mortality in ESRD patients with IE.
Long-term follow-up after infective endocarditis: comparison of patients who have undergone cardiac surgery versus a conservative therapy with and without an indication for cardiac surgery
Authors: Taner E , Zahn R , Kilkowski C , Karcher A K , Winkler R , Kleemann T , Ouarrak T , Schneider S , Sutor D , Weisse U , Sack F U
Published: October 2024

Background

Infective endocarditis (IE) is still a serious disease, with a high mortality despite cardiac surgery and modern conservative therapy. However, data on long-term follow-up are sparse.

Methods

Retrospective single centre registry on the hospital course and long-term follow-up of patients with IE who have undergone cardiac surgery or a conservative therapy with and without an indication for cardiac surgery.

Results

Between 1/2013 and 12/2016, 171 IE patients were treated at our hospital. A follow-up with either patient contact, review of hospital charts or physician contact was performed in 2022. Due to lost to follow-up (n=4) or refusal to participate after discharge (n=1), the final evaluation was performed with 166 patients (97.1%). They were followed-up for a median 2385.0 (between 2156 and 2773) days. 139 (81.3%) of the patients were operated (surgery) and 32 (18.7%) were treated conservatively. Amongst the conservatively treated group, 34.4% (11/32) of the patients had an indication for surgery, but it was decided not to perform it (cons-with) and 65.6% (21/32) had no indication for surgery (cons-without).

Operated patients were younger compared to cons treated patients (65.1 ± 13.0 vs 72.8 ± 15.7, p = 0.00016). Whereas hospital mortality was not different between both groups (21.6 % vs 21.9 %, p = 0.97112), calculated 5y mortality was higher in der cons treated group (71.0 % vs 49.6 %, p=0.03168).

Conclusions

These data on long-term follow-up after IE show a high total mortality amongst all patients regardless of type of therapy, but higher mortality rate after 5 years amongst conservative treated patients (71%) compared toa surgical treated (49.6% p= 0.03168). The highest mortality rate after 5 years (100%) was detected in the cons-with compared to 55% in the cons-without group. More than 2/3 of deaths were due to IE. However, operated patients were younger and had less co-morbidities. Furthermore, rehospitalisation rate for recurrent IE was also high.
Mild intracerebral bleeding does not adversely affect survival and neurological outcome of surgical therapy for left-sided infective endocarditis
Authors: Schach C , Fuessl E , Wiesinger I , Wendl C , Heller A , Schmid C , Maier L S , Schlachetzki F , Debl K
Published: October 2024

Background

Intracerebral bleeding (IB) is a serious condition that requires increased attention and has implications for treatment options. For patients with infective endocarditis (IE), current guidelines recommend postponing surgery if IB is present. IB can be classified into four relevant groups (following European Cooperative Acute Stroke Study and Heidelberger Bleeding classification): hemorrhagic transformation (HT) 1 and 2, and parenchymal hemorrhage (PH) 1 and 2. PH2 is the only IB with neurological deterioration. These classifications were originally introduced for stroke patients following thrombolysis but without IE. The hypothesis tested in our study is that IB may influence IE treatment and thus outcome.

Method

Patients were retrospectively identifyied who were operated for left-sided IE, neuroradiologists re-evaluated and classified preoperative cerebral imaging for any IB. Outcome was quantified using the modified Rankin score (mRS), and mRS were extracted from the patients’ hospital chart at admission and by follow-up telephone call (FU), also including mortality. To ensure that the influence of IB was comparable, patients with IB (IB+) were propensity matched (EuroScore 2, Charlson Comorbidity Index, C-reactive protein; nearest neighbor match) to patients without IB (IB-) who also received surgical IE therapy. All IB+ patients underwent a careful risk-benefit analysis before therapy.

Results

Of 286 consecutive patients (January 2015 - November 2022) operated for left-sided IE, 36 had IB (65±13 years, 26% female): 11 with HT1, 9 with HT2, 8 with PH1, and 8 with PH2. These patients were matched with 36 operated IB- patients. Survival analysis showed no overall effect of IB+ vs. IB- (p = 0.112, 95% CI of hazard ratio was 0.246-1.243 vs. 0.709-3.572). Although there was no effect of IB on survival in the mild IB subgroup (HT1/2, Figure A), there was a difference in patients with severe IB (PH1/2) with less survival in IB+ (Figure B). Neurological outcome (ΔmRS) showed an effect of IB grade in the two-way ANOVA analysis (p = 0.029). Intergroup comparison showed a significantly better outcome for IB- (vs. IB+) in the PH2 subgroup (p = 0.034), but not in other subgroups. For all groups, the mean ΔmRS for IB+ was 1.1±1.6 vs. 0.70±1.4 for IB- (p = 0.256). The mean FU time was 2.6±2.1 years.

Conclusion

In patients operated for left-sided IE, mild IB did not affect survival or neurological outcome. In the more severe forms of IB, i.e. PH1 and PH2, survival was less favorable compared to matched controls. Furthermore, we observed a difference in neurological outcome in patients with severe IB. Thus, surgical therapy of left-sided IE should be avoided in patients with severe IB, whereas we can add evidence to the safety of surgical therapy in mild IB. Therefore, we recommend regular assessment of the degree of IB in such patients.Figure
Acute renal failure in patients with infective endocarditis
Authors: Rodriguez Esteban M T , Miranda Montero S , Godoy R , Quijada Fumero A , Alvarez Gonzalez L , Hernandez Afonso J
Published: October 2024

Background/Introduction

Acute kidney failure (AKF) appears in a high percentage of patients with infective endocarditis (IE), becoming a complication related to the mortality of the disease and delayed recovery of patients.

Purpose

To investigate the factors that are related to the development of AKF in patients with IE.

Methods

Study of all cases of IE consecutively diagnosed in an university hospital between 2005 and 2021. A disease extension protocol was carried out using imaging tests and the levels of potentially nephrotoxic antibiotics were monitored. Furthermore, starting in 2010, a multidisciplinary committee (MC) was created to follow patients with EI.

Factors potentially related to the development of AKF during hospital admission according to RIFLE criteria were analyzed using a binary logistic regression analysis.

Results

305 cases were included, 96.7% of them meeting definitive IE criteria. The median age was 66 (54-74.5) years, 75.4% male. The most frequent microorganism was S. aureus (66; 21.6%), 172 (56.4%) underwent surgery and 107 patients (35.1%) died. 149 cases (45.3%) developed AKF, with no differences before (45.3%) and after the creation of the CM.

The variables related in the multivariate analysis to the appearance of AKF were age (OR 1.01; 95% CI: 1.01-1.05; p= 0.006), baseline creatinine (OR= 1.53; 95% CI = 1.22-1.68; p<0.001). ), peripheral embolism (OR=1.86; 95% CI = 1.07-3.22; p= 0.027), the development of heart failure (OR=1.98; 95% CI = 1.12-3.22; p= 0.017) and left ventricular systolic dysfunction (OR= 4.02; 95% CI = 1.09-14.85; p= 0.037). None of the other variables analyzed, including the type of microorganism and the use of nephrotoxic antibiotics, were related to the development of AKF.

Conclusions

In a hospital center with a protocol for the study and treatment of infective endocarditis, the development of AKF is related to age, baseline creatinine, peripheral embolism, and the development of heart failure.
Temporal dynamics of cardiac remodelling in pressure overload: the key role of visceral adipose tissue
Authors: Tarraf A , Freitas Nabuco Leva Ferreira J A , Delmont T , Altintas D , Radu C , Pini M , Czibik G , Folliguet T , Zhang Y , Bischof O , Derumeaux G
Published: October 2024

Background

Myocardial fibrosis is a prominent pathological feature of cardiac remodelling in pressure overload. The underlying mechanisms of cardiac remodelling are complex including mechanical, inflammatory and neurohormonal factors. We have recently uncovered a pathological crosstalk between visceral adipose tissue (VAT) and heart in biological aging characterized by profibrotic proteins in both the VAT secretome and the plasma contributing to myocardial interstitial fibrosis. We hypothesize that a model of pressure overload induced by transverse aortic constriction (TAC) is responsible for VAT remodelling and a VAT profibrotic secretome, which in turn worsens cardiac fibrosis and alters cardiac function.

Purpose

To explore the effect of pressure overload on VAT and identify a crosstalk between heart and VAT.

Methods

We divided wild-type male mice (5-month-old, n=10/group) into two groups: a visceral Lipectomy group and a Sham group two weeks before a moderate TAC (26G; i.e. gradient between left ventricle and aorta of 30mmHg) or a sham surgery. We sacrificed the mice at 1- and 8-week after TAC. We performed in vivo metabolic tests, echocardiography, and invasive hemodynamics, followed by ex vivo tissue analysis. We then analysed the time-course of the cardiac transcriptomic signature in TAC vs control and the impact of visceral lipectomy in both groups.

Results

Our results show that TAC induced not only cardiac remodelling affecting the structure and function of the heart, but also VAT fibrosis and inflammation together with activation of adrenergic receptors (Adrb3). VAT became a major source of profibrotic (TGFb and osteopontin) and proinflammatory (TNFa, IL6 and PAI1) factors as early as 1 week after TAC. Interestingly, lipectomy prevented cardiac remodelling and dysfunction. After 1 week of TAC, cardiac transcriptomic profile showed a similar upregulation of profibrotic and prohypertrophic genes in TAC groups in presence or absence of lipectomy. After 8 weeks of TAC, lipectomy prevented the upregulation of those genes and transcriptomic profile of TAC+lipectomy was similar to sham group (Figure). PCR analysis confirmed the lower expression of profibrotic (TGFb, Osteopontin), prohypertrophic (Nppa) and contractility genes (Adr1b) in TAC+Lipectomy vs TAC group after 8 weeks of TAC.

Conclusion

Our results suggest that VAT is detrimental to cardiac structure and function by releasing a profibrotic and a proinflammatory secretome that aggravates the cardiac remodelling induced by TAC. Conversely, lipectomy prevents the early development of TAC-induced cardiac fibrosis.
Nexilin-related cardiomyopathy (NEXN-CMP) - an island's perspective
Authors: Abela M , Cassar M , Mifsud J , Scerri J , Debattista J , Gialanze' Y , Farrugia Wismayer M , Scerri C , Felice T
Published: October 2024

Introduction

The phenotypic features of Nexilin related cardiomyopathies (NEXN-CMP) are poorly understood and under-reported. A better understanding of the genotype-phenotype association is paramount.

Objectives

The main objective of this study was to investigate the causative role and phenotypic expression of the NEXN gene in the Maltese Islands.

Methodology

Probands and relatives referred for a cardiac gene panel were evaluated. Subjects harbouring NEXN variants were identified. Those finally classified as having NEXN-CMP were evaluated for the purpose of this study.

Results

Out of 444 probands (confirmed or suspected cardiomyopathy), 12 probands were labelled with NEXN-CMP. This equates to a prevalence of 2.7% in the local cardiomyopathy pool, substantially higher to what has been previously reported.

Genetic testing identified 19 individuals (probands [n=15] and relatives [n=4]) all harbouring the same novel frameshift variant: NEXN c.1589_1590del p.(Arg530LysfsTer3), classified as likely pathogenic according to ACMG criteria.

Three (15.8%) harboured a second pathogenic variant (n=2 MYH7, n=1 TTN). Another 3 were not clinically affected (n=1 FH of SCD, n=2 first degree relatives of NEXN-CMP probands). These individuals were excluded from further analysis.

The NEXN-CMP cohort (n=13) consisted of a dominant male (68.8%) population (54±22 years at presentation, 7.7% athletes, 12 families). Most were probands (n=12/13). DCM was the dominant phenotype (n=8, 61.5%) followed by HNDLVC (n=3, 23.1%) and HCM (n=2, 15.4%). One infantile DCM was homozygous for the NEXN variant.

Symptoms were the most frequent method of presentation (n=6, 46.2%), followed by a FH of SCD and/or cardiomyopathy (n=3, 23.1%), abnormal echocardiogram (n=2 15.4%), OHCA (n=1, 7.7%) and inutero diagnosis (n=1, 7.7%). A FH of SCD was present in a third (n=4/12, 33.3%).

The ECG was abnormal in the majority (n=10/11, 90.9%). Most patients had a LBBB (6/11, 54.5%) followed by inferolateral TWI (n=3, 27.3%), ventricular arrhythmias/high-grade AVB (n=1, 9.1% each).

NEXN-CMP appears to be a left dominant cardiomyopathy. LV dilation and reduced LVEF were present in 84.6%. Non-ischaemic scar was present in 3/8 cases (37.5%), 2 had a ring-like pattern. RV morphology and function was normal in all cases.

Tachyarrhythmias were frequently observed (n=4/6, 66.7%) during holter monitoring or exercise testing (n=2 NSVTs, n=1 VEs, n=1 SVEs). A fourth (n=3/11, 27.3%) were implanted with an electrophysiological device. No significant predictors for device implantation were identified at univariate analysis. One patient is on the transplant list (infantile DCM).

Conclusion

The prevalence of NEXN-CMP in Malta appears higher than previously reported (2.7%), possibly because of a founder mutation. DCM and HCM are the dominant presenting phenotypes, frequently associated with symptoms, an abnormal ECG, a FH of SCD and LV dilatation/dysfunction.
Novel risk scoring model to assess the need for venoarterial-extracorporeal membrane oxygenation in patients with acute myocarditis
Authors: Hong D , Yang J H
Published: October 2024

Background

Some patients with acute myocarditis unpredictably deteriorate into a fulminant form and their prognosis is poor. However, it is uncertain which acute myocarditis patients are likely to exacerbate and even require venoarterial-extracorporeal membrane oxygenation (VA-ECMO).

Objectives

This study aimed to identify the risk factors associated with the need for VA-ECMO in patients with acute myocarditis and to develop a predictive model.

Methods

This retrospective study included 841 patients from seven hospitals in Korea with biopsy-proven or clinically suspected acute myocarditis. Logistic regression was performed to identify clinical characteristics of patients who required VA-ECMO and to construct a scoring system that can predict the need for VA-ECMO.

Results

Among the study population, 217 (25.8%) patients underwent VA-ECMO. The study population was divided into training (N=621) and testing (N=220) cohorts according to participating center. The final predictive model of VA-ECMO insertion derived from the training cohort included the following: initial mean blood pressure <65mmHg, cardiac arrest, Glasgow Coma Scale ≤12, platelet <100×103/mL, pulmonary congestion on chest X-ray, QRS interval ≥120ms, left or right bundle branch block, and left ventricular ejection fraction <40%. Using this predictive model, a β-coefficient-weighted Korean Acute Myocarditis (KAM) score was developed. The VA-ECMO insertion rate increased with increasing KAM score (odds ratio 1.683, 95% confidence interval 1.529-1.853, P<0.001). External validation of the predictive model and KAM score using the testing cohort showed excellent discriminant ability (areas under the curve of 0.945 and 0.921, respectively). Furthermore, at a KAM score of 8 or higher, which was the cut-off KAM score to predict the need for VA-ECMO, patients without VA-ECMO tended to have higher in-hospital mortality than patients who needed VA-ECMO.

Conclusions

A risk scoring system based on simple clinical and laboratory parameters at initial presentation could predict the need for VA-ECMO and clinical course in patients with acute myocarditis.Graphic Abstract
Is unsatisfactory outcome of alcohol septal ablation in patients with hypertrophic obstructive cardiomyopathy predictable?
Authors: Lawin D , Stellbrink C , Poudel M R , Marx K , Lawrenz T
Published: October 2024

Background

Alcohol septal ablation (ASA) has been introduced for left ventricular outflow tract gradient (LVOTG) reduction in patients with hypertrophic obstructive cardiomyopathy (HOCM). However, some patients may not profit from ASA. Thus, we identified characteristics of patients with unsatisfactory outcome of ASA in a large single-center cohort.

Methods

Consecutive ASAs between 1997 and 2023, performed at our institution, were retrospectively enrolled. Those ASAs performed in patients with missing follow-up (FU), previous septal reduction therapy (SRT), and atypical HOCM were excluded. Patients with unsatisfactory outcome (PUO) were defined as continuing to meet the guideline criteria for SRT (NYHA ≥III or NYHA ≥II with exertional syncope and resting/exercise-induced LVOTG ≥50mmHg). PUO were compared to patients with good outcome (PGO). Results are expressed in % or median and interquartile range (IQR). Pre-procedural predictors of poor hemodynamic outcome were identified using logistic regression analysis.

Results

65 of 1003 patients were PUO (6.5%). Those were younger (48.0 years [40.5-61.8] vs. 57.0 years [IQR 47.0-67.0] in PGO; p=0.0030), more often reported syncope at baseline (35.4% vs. 22.2% in PGO; p=0.0214), and had lower body-mass-index (26.4kg/m2 [23.8-29.2] vs. 27.7kg/m2 [25.2-23.1] in PGO; p=0.0160). In PUO, resting LVOTG was higher at baseline (53.0mmHg [24.5-82.0]) than in PGO (40.0mmHg [22.0-75.0]; p=0.0292; Table 1). Differences in sex, family history of sudden cardiac death, dyspnoea, medication, and interventricular septum diameter (IVSD) were not observed at baseline (all p>0.05; Table 1).

Age (OR 1.023; CI: 1.005-1.042; p=0.0129), syncope at baseline (OR 1.890; CI: 1.049-3.324; p=0.0296), and BMI (OR 1.072; CI: 1.007-1.145; p=0.0355) were found to independently influence poor hemodynamic response to ASA using multiple logistic regression controlling for confounders. However, sex, baseline IVSD, and LVOTG were not found to influence poor hemodynamic outcome (p>0.05).

There were no differences with regard to the number of embolized branches (1 in both groups), the size of the balloon used to cover the septal branch (1.5mm in both groups), the volume of ethanol (PUO: 1.4ml [1.0-2.0]; PGO: 1.5ml [1.2-2.0]), or creatine kinase release (PUO: 879.0U/l [375.0-1187.0]; PGO: 842.0U/l [501.5 - 1234]) (all p>0.05).

At 6-months-FU, PUO had significantly higher IVSD (18.0mm [13.5-22.0] vs. 16.0mm [12.0-19.0] in PGO; p=0.008), higher resting LVOTG (31.0mmHg [20.0-55.5] vs. 14.0mmHg [10.0-22.0] in PGO; p<0.0001) and higher exercise-induced LVOTG (105.0mmHg [84.5-143.0] vs. 35.0mmHg [22.0-64.0] in PGO; p<0.0001).

Conclusion

ASA was overall effective with only few patients experiencing unsatisfactory outcome. Syncope, age, and BMI were found predicting poor hemodynamic outcome.Baseline characteristics
Characterizing disease burden and health care resource utilization in patients with hypertrophic cardiomyopathy: a nationwide real-world study in Finland
Authors: Laine M , Holsa O , Simin J , Laatikainen O , Vilen S , Kuusisto J
Published: October 2024

Background

Hypertrophic cardiomyopathy (HCM) is an often hereditary myocardial disease with an estimated prevalence of 1:500 in the general population.

Purpose

This study aimed to investigate healthcare resource utilization (HCRU) and associated costs in patients diagnosed with HCM (and the associated subtypes: obstructive or non-obstructive) in a nationwide cohort in Finland.

Methods

This retrospective, registry-based cohort study included all Finnish patients diagnosed with HCM between 2000 and 2021, who were 12+ years old at the time of diagnosis, as identified from the national care registers for healthcare. HCRU data were collected between 2014 and 2021 from the Finnish national care registers for health care and the social insurance institution. Data pertaining to New York Heart Association (NYHA) classification were collected from three Finnish university hospitals.

Results

The study cohort comprised 5,834 patients with an HCM diagnosis; obstructive HCM accounted for 34% of cases. Mean per patient-year costs were €6638, 95% confidence interval (CI) €6472-€7415, and €6112, 95% CI €5957-€6560 for those with obstructive and non-obstructive HCM respectively. All-cause hospitalizations at specialized care were the most prominent cost associated with patients with obstructive and non-obstructive HCM equating to 46.3% and 46.1% of the total cost respectively (Fig 1.). Comorbidity burden, as measured by Charlson comorbidity index (CCI) score, was greater in patients with the obstructive than non-obstructive phenotype of the disease (CCI ≥4: 2.2 and 1.8, p<0.01, respectively). The highest utilization of invasive procedures was found for patients with NYHA class III-IV.

Conclusion

This population-based study, utilizing real-world data, provides novel insights into HCRU and related costs among Finnish patients with HCM (and associated subtypes). HCRU appears to be primarily driven by hospitalizations at specialized care. Furthermore, the data indicate that patients with obstructive HCM are likely to have a more severe disease, with higher comorbidity burden and requiring more invasive procedures than patients with the non-obstructive form of the disease. However, there were no statistically significant differences in health care costs between those with obstructive and non-obstructive HCM.
Effectiveness of contemporary risk stratification in patients with hypertrophic cardiomyopathy: a pilot study
Authors: Bonaventura J , Jensovsky M , Puchnerova V , Gleta E , Hansvenclova E , Ostadal P
Published: October 2024

Introduction

Risk stratification and primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) is a challenging discipline. When using current stratification systems, there is always a compromise between sensitivity and specificity.

Objective

In our pilot study, we sought to determine the incidence of SCD and the effectiveness of stratification systems, i.e., the ESC HCM Risk-SCD score and the system of individual major risk factors (RF) according to ACC/AHA in patients followed in an expert center.

Methods

Patients with HCM were risk-stratified and followed for 10 ± 8 years at the referral HCM center.

Results

Five hundred and five patients were stratified, and 71 (14%) were implanted with a cardioverter-defibrillator (ICD) based on risk stratification. ICD patients were stratified at age 39 ± 17 years, of which 26 (35%) were women, maximal left ventricular wall thickness (MLWT) was 23 ± 7 mm, and left atrial dimension (LA) 46 ± 7 mm. During follow-up, 12 (17%) patients had ≥ 1 appropriate ICD therapy at age 46 ± 19 years. Patients with ≥ 1 appropriate ICD therapy had an average 5-year ESC score of 1.8 ± 0.4% and an average ACC/AHA major RF of 1.4 ± 0.8, MLWT 25 ± 6 mm, LA 47 ± 7 mm. None of the ICD patients died of SCD. Five (7%) patients suffered from ICD complications, including 4 (6%) inappropriate shock, and one patient experienced infective endocarditis requiring system extraction.

During follow-up, 118 (23%) patients died, of which 28 (6%) from HCM-related causes, 12 (2%) from stroke, 11 (2%) from advanced heart failure, one perioperatively (myectomy) and 4 (1%) patients died suddenly (SCD). The mean age of SCD patients at the time of stratification was 59 ± 14 years; all were male, MLWT 18 ± 5 mm, LA 49 ± 2 mm, mean ESC score 2.0 ± 0.9%, and three (75%) had no RF present according to ACC/AHA. The ESC HCM Risk-SCD score was markedly less sensitive (sensitivity 56%; 95% CI 21-86) but more specific (specificity 98%; 95% CI 96-99) than the ACC/AHA approach (sensitivity 77%; 95% CI 46-95; and specificity 71%; 95% CI 67-75) (Figure).

Conclusion

With current risk stratification, albeit imperfect, SCD is rare in HCM patients managed at an expert center.
Effect of alcohol septal ablation on exercise hemodynamics, symptoms and quality of life in patients with obstructive hypertrophic cardiomyopathy
Authors: Dybro A M , Rasmussen T B , Ladefoged B T , Pedersen A L , Andersen M J , Nielsen B R , Terkelsen C J , Jensen M K , Poulsen S H
Published: October 2024

Background

The comprehensive impact of septal reduction therapy in patients with obstructive hypertrophic cardiomyopathy (HCM), including exercise hemodynamics and quality of life (QoL), remains poorly understood.

Purpose

The aim of this study was to evaluate the effect of alcohol septal ablation (ASA) in mitigating left ventricular outflow tract (LVOT) obstruction, reducing filling pressures during exercise, and the effect on QoL in patients with obstructive HCM.

Methods

This prospective study enrolled 24 patients with obstructive HCM referred for ASA. Evaluations included right-heart catheterization and echocardiography performed at rest and during exercise, before and approximately six months post-ASA. The primary outcome was the difference in filling pressures assessed by pulmonary capillary wedge pressure (PCWP) at 75 Watt (W). Secondary outcome assessed LVOT gradients, New York Heart Association (NYHA) functional class, Kansas City Cardiomyopathy Questionnaire Overall Summary Score (KCCQ-OSS), and exercise capacity.

Results

ASA significantly reduced PCWP at 75 W (24 ± 10 mmHg vs 33 ± 9 mmHg; P = 0.0001) (Figure 1), decreased LVOT gradients at rest (49 mmHg (24-90) vs 17 mmHg (14-27); P=0.002), at peak exercise (45 mmHg (29-90) vs 32 mmHg (21-61); P = 0.02), and post-exercise (96 mmHg (34-161) vs 28 mmHg (21-62); P=0.004). The KCCQ-OSS score was also significantly reduced (75.6 ± 18.8 vs 64.7 ± 16; P = 0.006), without affecting peak oxygen consumption (19.9 ± 4.8 ml/kg/min vs 18.7 ± 3.6 ml/kg/min; P = 0.26). The improvement in the KCCQ score was confirmed by changes in NYHA functional class. Before ASA treatment, 54% of patients were in NYHA functional class III compared to 13% after ASA (P < 0.0005).

Conclusion

ASA substantially reduces exercise-induced filling pressures, reduces LVOT obstruction, and improves symptoms and QoL in obstructive HCM, without impacting maximal exercise capacity.
Real-life experience with rilonacept for the treatment of recurrent pericarditis
Authors: Thomas G , Mbualungu J , Golino M , Abbate A
Published: October 2024

Background

Rilonacept, a soluble Interleukin-1 (IL-1) receptor fusion protein that neutralizes IL-1α and IL-1β, has shown efficacy in symptom resolution, weaning of standard-of-care therapies, and reducing the frequency of recurrent pericarditis (RP) episodes in clinical trials.

Purpose

We aimed to review the efficacy, safety, and duration of treatment with Rilonacept reliance in managing RP in our practice.

Methods

We analyzed 22 patients with RP treated with Rilonacept, 55 [43-69] years, 11 (50%) females. We reviewed clinical characteristics and outcomes, including the time since initial diagnosis of pericarditis, the presence of pericardial effusion/thickening, the duration of Rilonacept treatment, the ability to wean off steroids, and the recurrence of pericarditis symptoms attempting discontinuation.

Results

The length of time from the initial diagnosis of pericarditis to Rilonacept initiation was 9 [4-32] months. Fifteen patients out of 22 (68%) had pericardial effusion, 3 (20%) with tamponade requiring pericardiocentesis. Median length of treatment with Rilonacept was 14 [6-23] months, with 17/22 (77%) of patients being treated for ≥ 6 months. All patients who were on steroids (14/22, 64%) were able to discontinue their usage 1 [1-9] months after starting Rilonacept. Of the seventeen who were treated for 6 months or more, 12 (70%) remained on Rilonacept 160 mg weekly at the most recent assessment, 3 (18%) continued treatment with Rilonacept 160 mg every 2 or 4 weeks, and 2 (12%) discontinued Rilonacept treatment after 6 and 50 months. Of the 12 patients who remained on weekly treatment after a period of 6 months, 8 (67%) required Rilonacept for symptom control, 1 (8%) had recurrent symptoms after treatment every 2 weeks, and the remaining 3 (25%) preferred to continue despite a lack of symptoms (Figure 1).

Conclusions

The efficacy of Rilonacept in treating patients with RP and the ability to stop steroid therapy in the real world is in line with the efficacy data from clinical trials.Figure 1
Prognostic factors and etiological insights in constrictive pericarditis
Authors: Admella Severiano L , Sonora S , Rodenas E , Oristrell G , Soriano T , Ferreira I , Rello P
Published: October 2024

Constrictive pericarditis (CP) is an infrequent disease that poses a diagnostic and therapeutic challenge, with mortality often linked to underlying etiological factors. Our unicentric retrospective research aims to elucidate prognostic indicators and unravel the diverse etiological spectrum of this condition. The study is accepted by the Ethical Committee of our Center. Between January 2010 and December 2023, we have identified a total of 112 patients diagnosed with CP, with an average follow-up time of 6.42 years (IQR 1.98 - 11.45). We present the baseline variables of the patients, etiology and prescribed treatments in the table 1, comparing patients who survive with those who do not survive. 66.1% of patients were males and median age was 60.8 years old.

We present two Kaplan-Meier curves (Figures 1 and 2) comparing the survival rates between CP etiologies. In Figure 1, all causes are represented separately. In Figure 2, CP related to cardiac surgery, radiotherapy, purulent pericarditis, tuberculous pericarditis, mixed and other causes are combined. Our results suggest a better prognosis in patients with idiopathic CP and worse prognosis in patients with oncological related CP.

The significant predictors in the univariate Cox analysis were age, cachexia, ascites, oncology etiology, hemoglobin, sodium, Euroscore II and surgical pericardiectomy. The best predictive model (selected with a lower AIC) to predict mortality throughout follow-up, with a Harrell's C of 0.835, included the variables age, cachexia, oncological cause and hyponatremia as risk factors for mortality and surgical treatment by pericardiectomy as a protective factor (Figure 3).

In conclusion, our study suggests that CP etiology has an impact on survival with oncological cause having the worst prognosis. Furthermore, other factors as hyponatremia, cachexia, shock and age at diagnosis have also resulted in indicators of poor outcome.Figure 1.Kaplan meierFigure 3.Predictive model
Arrhythmia burden in patients with congenital heart disease
Authors: Eckerstrom F , Dellborg M , Kok W G , Eriksson P , Mandalenakis Z
Published: October 2024

Background

The prevalence of congenital heart defects (CHD) is rising. Many patients experience arrhythmias either due to the defect itself or necessary intervention. Sudden onset of arrhythmia poses grave risks such as heart failure, stroke, or cardiac death. Research gaps exist regarding high-risk defects, age susceptibility, and prognostic implications of arrhythmia.

Purpose

Delineate the burden of arrhythmias in patients with CHD.

Methods

Patients born in the period 1970-2017 and diagnosed with CHD were identified in Swedish nationwide medical registries. Each patient was matched by birthyear and sex with ten controls from the general population. Cox proportional hazard regression model and Fine-Gray regression model with death as competing risk were used to compute risk (hazard ratio [HR]) and cumulative incidence of arrhythmias (supraventricular and ventricular arrhythmias). Estimates were compared with the matched controls. Follow-up started at time of birth.

Results

We included 63,559 patients and 583,205 matched controls with median follow-up of 12.8 years. Almost two-thirds of the total CHD population were born after year 2000 and 89% (n=56,564) had a simple lesion (defined as atrial or ventricular septal defect, coarctation of the aorta or malformation of the heart valves). During follow-up, 1,632 events of arrhythmia (90% of supraventricular origin) were registered in the population of patients with CHD, yielding an incidence rate of 16.4 per 10,000 person-years (versus 1.6 in the control population). The cumulative incidence of arrhythmias in the total CHD population was slightly increased during childhood compared with the control population, but rose significantly hereafter, illustrated in Figure 1, yielding an overall risk of 10.5 (CI 95%: 9.8-11.2). Stratified by lesion severity, the risk for arrhythmias was 8.7 (CI 95%: 7.6-8.7) and 30.4 (CI 95%: 26.2-35.2) in patients with simple and complex lesions, respectively. Patients with simple CHD lesions displayed a risk of 7.7 (95% CI: 7.1-8.4) for supraventricular arrhythmias and 11.5 (95% CI: 9.2-14.5) for ventricular arrhythmias. Correspondingly, patients with complex CHD lesions displayed risks of 66.7 (95% CI: 42.7-104.2) and 28.9 (95% CI: 24.8-33.6).

Conclusion

Arrhythmias, irrespective of origin, are common in patients with CHD and correlate with lesion severity. The overall burden of arrhythmias increased significantly after childhood. Meticulous follow-up is vital in order to prevent, detect and treat arrhythmias as it might trigger clinical deterioration and even sudden cardiac death in patients with CHD.
The impact of tricuspid regurgitation on the timing of pulmonary valve replacement in adult patients after Tetralogy of Fallot repair
Authors: Brancaccio G , Fugallo D , Trezzi M , Bordonaro V , Secinaro A , Piccinelli E , Miraldi F , Butera G , Chinali M , Lisignoli V , Iacobelli R , Leonardi B , Iodice F , Galletti L
Published: October 2024

Background

Tricuspid regurgitation (TR) in patients with repaired tetralogy of Fallot (rTOF) is an important finding with a wide spectrum of etiologies. TR represents an important risk factor in adult patients with rTOF, influencing clinical outcomes and the requirement for additional surgical interventions. In this study, we reviewed the long-term outcomes of TR in ACHD patients with rTOF as well as its impact on the timing of surgical pulmonary valve replacement (PVR).

Methods

This was a retrospective analysis of a single institutional cohort which included 149 ACHD patients with a history of rTOF (transannular patch only) with CMR data, complete follow-up, and a recent (< 1 year) outpatient clinic visit.

Results

Overall follow-up time was 25.5 years (95% CI 24.5,26.4). Between 2002 and 2021, 93 patients required isolated PVR at a mean time from TOF repair of 16.6 years (95% CI 15.5,17.7) and at a mean age of 17.9 years (95% CI 16.8,19.0). In the whole cohort, significant TR was associated with a two-fold increase in the likelihood of requiring PVR (log-rank p = 0-.0023, HR 2.02; 1.09-4-47). Moderate to severe TR was more frequent in those who underwent VSD closure through a right atriotomy (17 versus 0, p = .0002) and affected time to PVR (13.9 versus 16.3 years, p = .0071), with equal preoperative RVEDVi at CMR. After PVR, RVEDVi normalized in all patients, regardless of the preoperative amount of TR (median 115.3 ml/m2 versus 112.3 ml/m2, p = .7). At a median time of 6.5 years from PVR, 13 patients (13/93, 14%) underwent redo PVR.

Conclusions

The preoperative severity of tricuspid regurgitation (TR) may have a substantial role on the likelihood of patients needing PVR at a younger age. This, in turn, could result in a reduced duration of freedom from valve failure. Replacing the pulmonary valve too early can effectively increase the lifetime number of procedures, preserving the function of the tricuspid valve at the time of TOF repair is of paramount importance.
Interaction between right atrial function reserve and liver stiffness after complete repair of tetralogy of fallot: a pilot study
Authors: Chik B W K , Chan K L , Chow P C , Yu C K M , Ho S C S , Cheung Y F
Published: October 2024

Background

Impaired right atrial mechanics is reported in patients with repaired tetralogy of Fallot (TOF) and is associated with liver stiffness. However, whether right atrial functional reserve correlates with liver stiffness is unknown.

Purpose

This study tested the hypothesis that right atrial reserve capacity is limited in patients with repaired TOF and explore its relationships with right ventricular function and liver stiffness.

Methods

10 patients (45% male) aged 19.04 ± 3.76 at 16.70 ± 4.30 years after repair and 15 controls (60% male, aged 20.31 ± 1.25 years) were studied. RA mechanics was assessed by speckle-tracking echocardiography (STE) at rest and during bicycle exercise, with quantification of positive, negative, and total strain, and strain rates at ventricular systole (aSRs), early diastole (aSRed), and atrial contraction (aSRac). Right atrial function reserve (RAFR) is calculated as (∆RA total strain x [1-1/ RA total strain at baseline]). RV systolic and diastolic function was quantified using Doppler interrogation and STE. Hepatic shear wave velocity (c) and tissue elasticity (E) were measured using two-dimensional shear wave elastography.

Results

At rest, patients had significantly lower RA positive, negative and total strain, aSRs and aSRed than control subjects (all P<0.02). Shear wave velocity and hepatic E value were significantly higher in patients than controls (both P<0.02). Compared with controls, patients had significantly lower RA positive, negative and total strain, aSRs, aSRed, and aSRac at exercise (all P<0.05). No significant difference in hepatic shear wave velocity and tissue elasticity were found between patients and control subjects at exercise (all P>0.05). Both baseline liver stiffness indices correlated negatively with RAFR, RA positive and total strain and aSRed at exercise (p<0.05 for all). RV function parameters, including baseline trans-tricuspid early and late diastolic inflow velocity (E, A), systolic, early and late diastolic tricuspid annular velocity (s, e, a), isovolumetric acceleration (IVA), RV systolic and diastolic reserve correlated significantly with RA positive and total strain at exercise (P<0.05 for all).

Conclusions

In young adults with repaired TOF, right atrial function reserve is impaired, and is associated with increased liver stiffness and RV dysfunction.RA mechanics from rest to exerciseStress RA mechanics and liver stiffness
Outcome of pregnancy in women with unrepaired atrial septal defect - study of a single tertiary centre
Authors: Kecskemeti D , Nagy Z S , Szabo B , Pataki S Z , Szegedi M , Temesvari A , Balint O H
Published: October 2024

Background

Patients with unrepaired atrial septal defect (ASD) are prone to develop arrhythmia, right heart failure and to suffer paradoxical embolism. The risk might be even bigger during pregnancy due to the hemodynamic changes combined with the hypercoagulable state.

Purpose

To evaluate maternal, foetal, and neonatal outcome of pregnancy in women with unrepaired ASD.

Methods

A retrospective study of 43 pregnancies in 24 women with unrepaired ASD was performed in our tertiary centre from 2004. Previous maternal cardiovascular events and events during pregnancy (maternal, neonatal, and obstetric) were documented.

Results

24 women with unrepaired ASD (median age: 27.5 ± 6.2) had 43 pregnancies resulting in one termination, 4 miscarriages and 38 live births. 55% were primigravida and 2 twin pregnancies were documented. At baseline 2 women (10.5%) were in NYHA functional class II and signs of right ventricle (RV) volume overload were detected on echocardiography in 18 cases (47%). 2 women had a history of supraventricular tachycardia. Median gestation at delivery was 39 ± 2.6 weeks. No thromboembolic events, pulmonary hypertension, or desaturation occurred during pregnancy. However maternal cardiovascular complications all affected women with RV volume overload: 2 cases (5.2%) of supraventricular tachycardia, 2 (5.2%) cases with NYHA functional class deterioration and 1 case (2.6%) of syncope were documented. Only one obstetric event; preeclampsia occured. Neonatal complications were as follows: premature delivery in 3 cases (7.9%) and recurrence of congenital heart disease (CHD) in 4 pregnancies (10.5%). After the pregnancy device or surgical closure of the ASD was indicated for 11 women (45.8%). Since then, five already underwent device closure, one had surgical repair, while five women are still waiting for intervention.

Conclusions

Our results align with the mWHO classification. Pregnancy with an unrepaired ASD is well tolerated and has a low risk of neonatal and maternal cardiovascular events. However, women with right ventricle volume overload are prone to develop arrhythmia or right heart failure during pregnancy. Therefore, cardiology follow-up during pregnancy should be decided on an individual basis. In our cohort no thromboembolic events were documented during pregnancy, however the prevalence of congenital heart disease in the foetus was higher compared to the literature.
Effect of Fontan circulation on body composition and correlation with exercise capacity
Authors: Pomiato E , Rasoul H , Duong P , Frigiola A
Published: October 2024

Background

There is growing attention to body composition in Fontan patients (FP) and preliminary results suggest that FP have an increased fat mass and a decreased muscle mass compared to the healthy population. FP also have reduced exercise capacity compared to health peers. However, data are still limited.

Purpose

The aim of our study was to compare the body composition (BC) of adult patients with Fontan circulation and healthy controls and to assess the correlation with functional capacity.

Material and Methods

Case control study on adult patients with Fontan circulation (FP) and age and gender matched healthy controls (HC). The following characteristics were recorded: age, gender, morphology of the systemic ventricle, type of Fontan circulation, level of physical activity (PAR, 0-7 points, 0 = sedentary - 7 = running > 3hours/week). S-score muscle, T-score bone, fat mass (FM%), intra-muscular adipose tissue (IMAT), abdominal adipose tissue (AAT), intracellular water (ICW), extracellular water (ECW), total body water (TBW), were measured through multi-frequency bioelectrical impedance technology in the FP and in the HC. Correlation was made for FP between body composition and oxygen consumption at peak exercise (VO2 peak), VE/VCO2 slope and NYHA class as indicator of functional capacity.

Results

Thirty Fontan patients (FP, mean age 24±6 yrs, 70% males) and 30 matched healthy controls (HC) were recruited in the study. The systemic ventricle was dominant left in 14 patients (47%), dominant right in 16 (53%). Four patients had atrio-pulmonary connection, the remaining were palliated with a lateral tunnel (n=26, 87%). The analysed parameters are summarised in Table 1. PAR was reduced in FP compared to HC (median 4.0 points in FP versus 7.0 points in HC, p 0.024). There was no difference in the BMI, fat mass, and body water between FP and HC. However, FP showed significantly lower S and T scores compared to the HC (S-score: -0,75 in FP versus 0,0 in HC, p 0.003. T-score: -0.9 in FP versus -0.25 in HC, p 0.001). In FP, NYHA Class was I in 28 patients (93%), II in 1 (3,5%) and III in 1 (3.5%) patient. The mean VO2 was 23.4±5.4mlO2/kg/min and mean VE/VCO2 slope 32±7. There was a direct correlation between VO2 peak and the PAR (rho 0.443, p 0.044). No correlation was found between the VO2 peak, the VE/VCO2 slope or the NYHA Class and the analysed parameters of body composition.

Conclusion

FP have reduced muscle mass, and bone density compared to HC. In addition to the known reduced efficiency of the cardiac pump during exercise in FP, the lower level of conditioning contributes to the reduced exercise capacity in this population. Programs aiming to encourage physical activity are key and should be part of patients’ care.