Abstract
Aims
Familial ST-segment Depression Syndrome (Fam-STD) is a novel inherited cardiac disease associated with arrhythmias and sudden cardiac death. This study aimed at investigating the ...cardiac activation pathway in patients with Fam-STD, modelling the electrocardiogram (ECG) phenotype, and performing in-depth ST-segment analyses.
Methods and results
CineECG analysis of patients with Fam-STD and age- and sex-matched controls. The groups were compared using the CineECG software which included the trans-cardiac ratio and the electrical activation pathway. We simulated the Fam-STD ECG phenotype by adjusting action potential duration (APD) and action potential amplitude (APA) in specific cardiac regions. High-resolution ST-segment analyses were performed per lead by dividing the ST-segment into nine 10 ms subintervals. Twenty-seven Fam-STD patients (74% females, mean age 51.6 ± 6.2 years) and 83 matched controls were included. Among Fam-STD patients, electrical activation pathway analysis in the anterior-basal orientation showed significantly abnormal direction toward the basal areas of the heart starting from QRS 60–89 ms until Tpeak-Tend (all P < 0.001). Simulations with shortened APD and reduced APA in the left ventricle basal regions recapitulated the Fam-STD ECG phenotype. Detailed ST-segment analyses showed significant differences in all nine 10 ms subintervals (all P < 0.01), with the most prominent findings during the 70–79/80–89 ms intervals.
Conclusion
CineECG analyses indicated abnormal repolarization with basal directions, and the Fam-STD ECG phenotype was simulated by reducing APD and APA in the left ventricle basal regions. Detailed ST-analysis showed amplitudes consistent with the proposed diagnostic criteria for Fam-STD patients. Our findings provide new insight into the electrophysiological abnormalities of Fam-STD.
Graphical Abstract
Graphical Abstract
Abstract
Aims
Atrioventricular block (AVB) of unknown aetiology is rare in the young, and outcome in these patients is unknown. We aimed to assess long-term morbidity and mortality in young patients ...with AVB of unknown aetiology.
Methods and results
We identified all Danish patients younger than 50 years receiving a first pacemaker due to AVB between January 1996 and December 2015. By reviewing medical records, we included patients with AVB of unknown aetiology. A matched control cohort was established. Follow-up was performed using national registries. The primary outcome was a composite endpoint consisting of death, heart failure hospitalization, ventricular tachyarrhythmia, and cardiac arrest with successful resuscitation. We included 517 patients, and 5170 controls. Median age at first pacemaker implantation was 41.3 years interquartile range (IQR) 32.7–46.2 years. After a median follow-up of 9.8 years (IQR 5.7–14.5 years), the primary endpoint had occurred in 14.9% of patients and 3.2% of controls hazard ratio (HR) 3.8; 95% confidence interval (CI) 2.9–5.1; P < 0.001. Patients with persistent AVB at time of diagnosis had a higher risk of the primary endpoint (HR 10.6; 95% CI 5.7–20.0; P < 0.001), and risk was highest early in the follow-up period (HR 6.8; 95% CI 4.6–10.0; P < 0.001, during 0–5 years of follow-up).
Conclusion
Atrioventricular block of unknown aetiology presenting before the age of 50 years and treated with pacemaker implantation was associated with a three- to four-fold higher rate of the composite endpoint of death or hospitalization for heart failure, ventricular tachyarrhythmia, or cardiac arrest with successful resuscitation. Patients with persistent AVB were at higher risk. These findings warrant improved follow-up strategies for young patients with AVB of unknown aetiology.
Graphical Abstract
Atrioventricular block of unknown aetiology presenting before the age of 50 years and treated with pacemaker implantation was associated with a three- to four-fold higher rate of the composite endpoint of death or hospitalization for heart failure, ventricular tachyarrhythmia, or cardiac arrest with successful resuscitation. CI, confidence interval.
The penetrance of hypertrophic cardiomyopathy (HCM) during childhood and adolescence has been only sparsely described. We studied the penetrance of HCM and the short- and long-term outcomes of ...clinical screening and predictive genetic testing of child relatives of patients with HCM.
Ninety probands and 361 relatives were included in a family screening program for HCM (1994-2001). Eleven sarcomere genes, CRYAB, α-GAL, and titin were screened. Sixty-six relatives and 4 probands were <18 years of age at inclusion. Twelve child relatives were mutation carriers (age, 12 ± 5 years), and 26 had unknown genetic status, ie, relatives from families without identified mutations (n = 21) or not tested (n = 5) (age, 11 ± 5 years). Twenty-eight noncarriers (42%; age, 10 ± 4 years) served as control subjects. Two of 38 child relatives (5%) at risk of developing HCM fulfilled diagnostic criteria for HCM at inclusion. After 12 ± 1 years of follow-up, 2 of the 36 (6%; 95% confidence interval, 2-18) at-risk child relatives who were phenotype negative at inclusion had developed the HCM phenotype at 26 and 28 years of age. During follow-up, none of the child relatives experienced serious cardiac events. Participation in the screening program had no long-term negative psychological impact.
The penetrance of HCM in phenotype-negative child relatives at risk of developing HCM was 6% after 12 years of follow-up. The finding of phenotype conversion in the mid-20s warrants continued screening into adulthood. Forty-two percent of the child relatives were noncarriers, and repeat clinical follow-up could be safely limited to the remaining children.
Abstract Objectives The aim of this study was to describe the safety and outcomes of alcohol septal ablation (ASA) in younger patients with obstructive hypertrophic cardiomyopathy. Background The ...American College of Cardiology Foundation/American Heart Association guidelines reserve ASA for older patients and patients with serious comorbidities. Data on long-term age-specific outcomes after ASA are scarce. Methods A total of 1,197 patients (mean age 58 ± 14 years) underwent ASA for obstructive hypertrophic cardiomyopathy. Patients were divided into young (≤50 years), middle-age (51 to 64 years), and older (≥65 years) groups. Results Thirty-day mortality and pacemaker implantation rates were lower in young compared with older patients (0.3% vs. 2% p = 0.03 and 8% vs. 16% p < 0.001, respectively). Ninety-five percent of young patients were in New York Heart Association functional class I or II at last follow-up. During a mean follow-up period of 5.4 ± 4.2 years, 165 patients (14%) died. Annual mortality rates of young, middle-age, and older patients were 1%, 2%, and 5%, respectively (p < 0.01). Annual adverse arrhythmic event rates were similar in the 3 age groups at about 1% (p = 0.90). Independent predictors of mortality in young patients were age, female sex, and residual left ventricular outflow tract gradient. Additionally, young patients treated with ≥2.5 ml alcohol had a higher all-cause mortality rate (0.6% vs. 1.4% per year in patients treated with <2.5 ml, p = 0.03). Conclusions ASA in younger patients with obstructive hypertrophic cardiomyopathy was safe and effective for relief of symptoms at long-term follow-up. The authors propose that the indication for ASA can be broadened to younger patients.
Background COVID‐19 infection has been hypothesized to affect left ventricular function; however, the underlying mechanisms and the association to clinical outcome are not understood. The global work ...index (GWI) is a novel echocardiographic measure of systolic function that may offer insights on cardiac dysfunction in COVID‐19. We hypothesized that GWI was associated with disease severity and all‐cause death in patients with COVID‐19. Methods and Results In a multicenter study of patients admitted with COVID‐19 (n=305), 249 underwent pressure‐strain loop analyses to quantify GWI at a median time of 4 days after admission. We examined the association of GWI to cardiac biomarkers (troponin and NT‐proBNP N‐terminal pro‐B‐type natriuretic peptide), disease severity (oxygen requirement and CRP C‐reactive protein), and all‐cause death. Patients with elevated troponin (n=71) exhibited significantly reduced GWI (1508 versus 1707 mm Hg%; P =0.018). A curvilinear association to NT‐proBNP was observed, with increasing NT‐proBNP once GWI decreased below 1446 mm Hg%. Moreover, GWI was significantly associated with a higher oxygen requirement (relative increase of 6% per 100–mm Hg% decrease). No association was observed with CRP. Of the 249 patients, 37 died during follow‐up (median, 58 days). In multivariable Cox regression, GWI was associated with all‐cause death (hazard ratio, 1.08 95% CI, 1.01–1.15, per 100–mm Hg% decrease), but did not increase C‐statistics when added to clinical parameters. Conclusions In patients admitted with COVID‐19, our findings indicate that NT‐proBNP and troponin may be associated with lower GWI, whereas CRP is not. GWI was independently associated with all‐cause death, but did not provide prognostic information beyond readily available clinical parameters. Registration URL: https://www.clinicaltrials.gov ; Unique identifier: NCT04377035.
The current American College of Cardiology Foundation/American Heart Association guidelines on hypertrophic cardiomyopathy state that institutional experience is a key determinant of successful ...outcomes and lower complication rates of alcohol septal ablation (ASA). The aim of this study was to evaluate the safety and efficacy of ASA according to institutional experience with the procedure.
We retrospectively evaluated 1310 patients with symptomatic obstructive hypertrophic cardiomyopathy who underwent ASA and were divided into 2 groups. The first-50 group consisted of the first consecutive 50 patients treated at each centre, and the over-50 group consisted of patients treated thereafter (patients 51 and above).
In the 30-day follow-up, there was a significant difference in the occurrence of major cardiovascular adverse events (21% in the first-50 group vs 12% in the over-50 group; P < 0.01), which was driven by the occurrence of cardiovascular deaths (2.1% vs 0.4%; P = 0.01) and implanted pacemakers (15% vs 9%; P < 0.01). In the long-term follow-up (5.5 ± 4.1 years), the first-50 group was associated with a significantly higher occurrence of major adverse events (P < 0.01) and higher cardiovascular mortality (P < 0.01). Also, patients in the first-50 group were more likely to self-report dyspnea of New York Heart Association class III/IV (16% vs 10%), to have a left ventricular outflow gradient > 30 mm Hg (16% vs 10%) at the last clinical check-up (P < 0.01 for both), and a probability of repeated septal reduction therapy (P = 0.03).
An institutional experience of > 50 ASA procedures was associated with a lower occurrence of ASA complications, better cardiovascular survival, better hemodynamic and clinical effect, and less need for repeated septal reduction therapy.
Les lignes directrices actuelles sur la cardiomyopathie hypertrophique de l’American College of Cardiology Foundation et de l’American Heart Association indiquent que l’expérience de l’établissement est un déterminant clé du succès de l’ablation septale à l’alcool (ASA) et du faible taux de complications de cette intervention. La présente étude visait à évaluer l’innocuité et l’efficacité de l’ASA en fonction de l’expérience de l’établissement relativement à cette intervention.
Nous avons effectué une évaluation rétrospective de 1310 patients atteints de cardiomyopathie hypertrophique obstructive symptomatique ayant subi une ASA, qui ont été divisés en 2 groupes. Le groupe des 50 premiers patients était constitué des 50 premiers patients consécutifs traités dans chaque centre, tandis que le groupe des patients suivant les 50 premiers était constitué des patients traités par la suite (patient no 51 et suivants).
Durant la période de suivi de 30 jours, une différence significative a été observée sur le plan de la survenue d’événements indésirables cardiovasculaires majeurs (21 % dans le groupe des 50 premiers patients comparativement à 12 % dans le groupe des patients suivant les 50 premiers; p < 0,01) attribuables principalement aux décès d’origine cardiovasculaire (2,1 % vs 0,4 %; p = 0,01) et à l’implantation de stimulateurs cardiaques (15 % vs 9 %; p < 0,01). Au cours du suivi à long terme (5,5 ± 4,1 ans), le groupe des 50 premiers patients était associé à un taux significativement plus élevé d’événements indésirables majeurs (p < 0,01) et de décès d’origine cardiovasculaire (p < 0,01). De plus, les patients du groupe des 50 premiers patients étaient plus nombreux à signaler spontanément une dyspnée de classe III/IV selon l’échelle de la New York Heart Association (16 % vs 10 %) et à afficher un gradient de pression au niveau du ventricule gauche supérieur à 30 mm Hg (16 % vs 10 %) lors du dernier bilan clinique (p < 0,01 pour ces deux paramètres), et présentaient également une probabilité plus élevée de répétition de l’intervention de réduction septale (p = 0,03).
Une expérience au sein de l’établissement supérieure à 50 ASA a été associée à un taux plus faible de complications de cette intervention, à une meilleure survie cardiovasculaire, à des effets hémodynamiques et cliniques supérieurs et à une diminution de la nécessité de répéter l’intervention de réduction septale.
Infective endocarditis (IE) is associated with high mortality. Surgery may improve survival and reduce complications, but the balance between benefit and harm is difficult and may be closely related ...to age and type of surgical intervention. We aimed to examine how age and type of left-sided surgical intervention modified mortality in patients undergoing surgery for IE. By crosslinking nationwide Danish registries we identified patients with first-time IE undergoing surgical treatment 2000-2017. Patients were grouped by age 60 years, 60-75 years, and greater than or equai to 75 years. Multivariable adjusted Cox proportional hazard analysis was used to examine factors associated with 90-day mortality. In patients undergoing surgery for IE, mortality increased significantly with age and 1 in 5 died above age 75 years. Mitral valve surgery as well as multiple valve interventions augmented mortality further.
Aim
To explore and describe how the National Early Warning Score (NEWS) and Individual Early Warning Score (I‐EWS) are used and how they support nurses' patient risk assessment practice.
Design
A ...qualitative observational fieldwork study drawing on ethnographical principles was performed in six hospitals in two regions of Denmark in 2019.
Methods
Data were generated from participant observations and informal interviews with 32 nurses across 15 different wards in the hospitals. A total of 180 h of participant observation was performed. The observations lasted between 1.5 and 8 h and were conducted during day or evening shifts.
Results
NEWS and I‐EWS supported nurses' observations of patients, providing useful knowledge for planning patient care, and prompting critical thinking. However, the risk assessment task was sometimes delegated to less experienced staff members, such as nursing students and healthcare assistants. The Early Warning Score (EWS) systems were often adapted by nurses according to contextual aspects, such as the culture of the speciality in which the nurses worked and their levels of competency. In some situations, I‐EWS had the effect of enhancing nurse autonomy and responsibility for decision‐making in relation to patient care.
Conclusions
EWS systems support nurses' patient risk assessment practice, providing useful information. I‐EWS makes it easier to factor the heterogeneity of patients and the clinical situation into the risk assessments. The delegation of risk assessment to other, less experienced staff members pose a risk to patient safety, which needs to be addressed in the ongoing debate regarding the shortage of nurses.
Impact
The findings of this study can help ward nurses, hospital managers and policymakers to develop and improve strategies for improved person‐centred nursing care.
Objective:
To determine the frequency and extent of cardiac involvement in female carriers of pathogenic variants in
DMD
, 53 women were examined through an observational, cross-sectional study.
...Methods:
Genetically verified female carriers of pathogenic
DMD
variants were examined by cardiac magnetic resonance imaging (CMR) with late gadolinium enhancement, echocardiography, 24-h Holter monitoring, ECG, and blood concentrations of skeletal and cardiac muscle biomarkers.
Results:
Fifty-three female carriers of pathogenic
DMD
variants (mean age 49.6 years, 33 associated with DMD, and 20 with BMD) were included in the study. Sixty-two percent had cardiac dysfunction on echocardiography. On CMR, 49% had myocardial fibrosis, 35% had dilated left ventricles, and 10% had left ventricular hypertrophy. ECGs were abnormal in 72%, and abnormal Holter monitoring was found in 43%. Age did not correlate with myocardial fibrosis or cardiac dysfunction. Myocardial fibrosis was more frequent in carriers of pathogenic variants associated with DMD vs. BMD (61 vs. 28%,
p
= 0.02).
Conclusion:
This study shows that cardiac involvement, affecting both structure and function of the heart, is found in over 2/3 of women with a pathogenic
DMD
variant. The study supports early cardiac screening, including ECG, Holter, and cardiac imaging, in this group of carriers, so that symptoms related to pathogenic variants in
DMD
can be recognized, and relevant treatment can be initiated. Longitudinal studies are needed to assess morbidity and mortality related to single, pathogenic
DMD
variants in women.
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