Severe Heart Failure Symptoms Research Articles

Eplontersen (Wainua)

Canada’s Drug Agency (CDA-AMC) recommends that Wainua be reimbursed by public drug plans for the treatment of polyneuropathy (PN) associated with stage I or stage II hereditary transthyretin amyloidosis (hATTR) in adults, if certain conditions are met. Wainua should only be covered to treat adults with stage I or stage II genetically confirmed hATTR with PN (hATTR-PN) who are symptomatic with early-stage neuropathy, do not have severe heart failure symptoms, and have not had a liver transplant. A patient’s response to treatment with Wainua should be assessed at least every 6 months to determine whether they would benefit from continued treatment. Treatment with Wainua should not be continued in patients who are permanently bedridden and dependent on assistance for basic activities of daily living or who are receiving end-of-life care. Wainua should only be reimbursed if the patient is under the care of a specialist with experience in the diagnosis and management of hATTR-PN, and should not be reimbursed if it is used in combination with interfering ribonucleic acid (RNA) drugs or transthyretin stabilizers. The cost of Wainua should be reduced so that it does not cost more than other drugs for hATTR.

Перипартальна кардіоміопатія: сучасні можливості ведення пацієнток із важкою серцевою недостатністю

Peripartum cardiomyopathy (PCMP) is a cardiomyopathy with impaired LV systolic function that develops in pregnant women or in the first months after childbirth. Clinical manifestations can vary from mild or moderate to severe symptoms of heart failure with significant functional limitation and high mortality. The mechanisms of PCMP development are unknown, and there is no specific treatment. In the article, we wanted to demonstrate our own experience of managing a patient with severe PCMP, the course of which was complicated by heart rhythm disturbances, the development of cardiogenic shock and pulmonary edema. This clinical example will highlight basic information about the etiology, pathogenesis, prevention and treatment of PCMP at the current stage.

Accelerometer-measured physical activity pattern in older patients with frailty and heart failure

Abstract Background Physical activity (PA) assessed by accelerometers represents a novel method to understand the PA profile of older patients with heart failure (HF). We hypothesized that PA level and pattern vary in HF patients across the spectrum of frailty status. Aim To report the duration, intensity and volume of PA in older patients with HF according to frailty status. Methods We recruited 150 HF patients≥65 years from a hospital-based HF clinic between March and October 2023. Frailty was assessed using the Short Physical Performance Battery (SPPB); patients with SPPB 10-12, 7-9, 4-6 and ≤3 was classified as robust, mildly, moderately and severely frail respectively. To evaluate PA profile, all patients wore a wrist accelerometer on their non-dominant hand for 7 days (24 hours/ day). PA parameters including average acceleration over 24 hours (proxy of volume of activity); time spent in inactive, light, moderate and vigorous intensity activities; intensity gradient (more negative gradient equates to poorer intensity profile) and time of most active continuous bouts of PA, were compared amongst frailty groups. Results 146 participants with good quality accelerometer data were included in the analysis; median age 80 (range 65-94 years), 65% male. Thirty-eight percent had heart failure with reduced ejection fraction (HFrEF); 21% had NYHA class III/IV symptoms; median NT-proBNP was 2040 ng/L (IQR = 1110 - 4930). Frail patients were more likely to be older with more comorbidities; they also had more severe HF symptoms and higher NT-proBNP. Frail patients had lower volume of PA compared to robust patients, evidenced by lower average acceleration (milligravity-mg) over 24 hours [20.4 (robust); 16.0 (mildly frail); 13.5 (moderately frail); 13.2 (severely frail), p<0.001]. PA intensity profile was poorer in frail patients compared to robust patients [median intensity gradient: -2.72 (robust); -2.92 (mildly frail); -3.00 (moderately frail); -3.07 (severely frail), p<0.001]. (Table 1) Frail patients spent more time inactive compared to robust patients [median time spent inactive (minutes): 654 (robust); 689 (mildly frail); 780 (moderately frail); 720 (severely frail), p<0.001]. The median time (minutes) spent in light [225 (robust); 190 (mildly frail); 164 (moderately frail); 159 (severely frail)] and moderate to vigorous activity [62 (robust), 23 (mildly frail), 15 (moderately frail) and 13 (severely frail), p<0.001] were significantly shorter in frail compared to robust patients. (Figure 1) The most active continuous bout of PA of patients across the frailty spectrum appeared to between 10 AM to 2 PM. Conclusion The duration, volume and intensity of PA was generally low in older patients with HF. Worse frailty status was associated with longer duration of physical inactivity, shorter duration of light, moderate or vigorous activity, poorer PA intensity profile and lower volume of PA.

Sex differences in paediatric onset hypertrophic cardiomyopathy

Abstract Background Hypertrophic Cardiomyopathy (HCM) is a heterogeneous disease characterised by age related incomplete penetrance and variable long term outcomes. Sex differences have been described in both the clinical phenotype and natural history of adults with the disease, which have been attributed to both biological (eg sex hormone effect) and non-biological (eg societal and cultural effects) factors. It is unknown if similar sex differences exist in childhood-onset HCM. Purpose This study aimed to investigate the influence of biological sex on the clinical characteristics and outcomes of children with HCM. Methods Retrospective cohort study from an international registry of patients diagnosed under the age of 18 years with HCM. Patients with syndromic disease (RASopathy syndromes, inborn errors of metabolism, neuromuscular disease) were excluded. Sex differences in baseline characteristics and clinical outcomes were described. Primary outcomes were all-cause mortality or cardiac transplantation, Major Arrhythmic Cardiac Event (MACE) and heart failure event (defined as heart failure death or cardiac transplantation) Results Of 1262 patients, 851 (67.4%) were male (median age 11 yrs (IQR 7.4, 14.4) vs 11.1 (IQR 6.9, 14.0) in females, p=0.0312). Female patients were more likely to have a sarcomeric variant identified (n=208, 85.6% vs 388 (79.4%), p=0.041) despite equivalent genetic testing utilisation between sexes. Female patients were more likely to have severe heart failure symptoms (NYHA 3 or 4) (n=19, 6.1% vs n=20, 2.5% p value 0.030) but there was no difference in the proportion on medical therapy (male n=328, 39.7%, female n=167, 41.8, p value 0.476). The clinical phenotype in terms of degree of hypertrophy, left atrial size and left ventricular outflow tract gradient did not differ by sex. Over a median follow up of 5.2 years (IQR 2.4, 9.9) all-cause mortality or cardiac transplantation did not vary by sex but females were more likely to experience a heart failure event (females 0.6/100,000 pt years (95% CI 0.39-0.92) vs males 0.32/100,000 pt years (95% CI 0.21 – 0.50), hazard ratio 1.89, p value 0.0467). The incidence of MACE did not differ by sex but there was a trend towards higher implantable cardioverter defibrillator implantation in females (n=123, 30.7% vs n=210, 25.4%, p value 0.051). Conclusions For the first time, this study has shown sex differences in paediatric-onset hypertrophic cardiomyopathy. Despite no difference in baseline clinical phenotype, young female patients are more likely to experience heart failure symptoms and heart failure events over follow up. Further studies are required to explore the underlying mechanisms for these observed differences.Table summarising outcomes by sexSurvival free from heart failure

Eight year Follow-Up Data on a Leadless Pacing System Real life experience in comparison to data of the prospective trial

Abstract Introduction The Micra leadless intracardiac pacing system has been introduced and implemented into clinical routine in 2015, with its next generation MICRA AV in 2020. Feasibility, implantation safety, and acute success have been proven in the setting of controlled studies. Even apart from controlled studies, this new technology was beneficial for the individual patient. We aimed to report our single center follow-up (FU) data with a follow-up of up to six years. Methods In 292 patients (146 men; age: 81±5,7 y), Micra implantation was performed. Pacemaker interrogation was performed one to seven days after implantation and during FU (1; 3; 6, then every 6 month) up to 84 months. Data were assessed in a real-life setting and compared with existing data of a controlled prospective trial. Results The implantation was successful in all 292 attempts with only one pericardial effusion as major complication. During follow up there were two patients developing severe heart failure symptoms resulting in an implantation of a CRT-device and switching of the leadless pacemaker system. The average acute thresholds, sensing and impedance after system release were: 0.57±0.34V@0.24ms; 10,02±3,96mV and 752±1201Ohm. During follow up of up to 8 years neither, pacemaker failure, nor infections were reported. Measurements were reevaluated for long-term thresholds, sensing and impedance: 0.75 ± 0.38V @ 0.24 ms; 18,38 ±2,75 mV and 525 ± 102 Ohm. During eight years, no significant changes from acute to long-term measurements were detectable. In comparison to the controlled trial, our measurements of this real-life cohort were very similar. Conclusion In a real-life setting, the implantation of the leadless Micra system demonstrates a high rate of implantation success without major complications. We were also able to show stable long-term system parameters in the clinical setting of up to seven years of follow up with a very good battery longevity of about 12,3 years.

Transcatheter aortic valve replacement failure: surgical valve explantation after more than a decade.

Transcatheter aortic valve replacement (TAVR) has become an established alternative to surgical aortic valve replacement for severe aortic stenosis. However, the long-term outcomes and need for surgical reintervention following TAVR remain uncertain. This case report describes a 76-year-old woman who underwent surgical explantation of a SAPIEN-XT valve more than a decade after initial TAVR implantation due to late valve failure. The patient presented with severe aortic insufficiency and heart failure symptoms. Surgical intervention involved concomitant ascending aortic replacement, tricuspid annuloplasty, and coronary artery bypass grafting. The TAVR valve was successfully explanted using careful blunt dissection to avoid annulus damage. Postoperative recovery was uneventful, with the patient discharged after 4 weeks. This case highlights the potential need for long-term surgical management of patients after TAVR, and emphasizes the importance of surgical preparedness as TAVR indications expand. It also provides valuable insights for surgeons encountering similar cases of late TAVR failure requiring explantation.

Sexual dysfunction and its associated factors among patients with heart failure in public hospitals in Gondar town, North and West Gondar, 2024

IntroductionSexual dysfunction is characterized by difficulties that interfere with achieving satisfying sexual activity, affecting desire, arousal, orgasm, and overall satisfaction. A recent study in Ethiopia identified heart failure as one of the most common cardiovascular diseases in the country. Sexual dysfunction significantly affects the quality of life in individuals with heart failure, yet it often goes underdiagnosed and underreported. Understanding the prevalence of sexual dysfunction and the factors influencing sexuality in these patients is essential. Therefore, the primary objective of this study was to determine the prevalence of sexual dysfunction among patients with heart failure in Gondar City and the northern and western zones of Gondar public hospitals.MethodThis study included 423 participants diagnosed with heart failure, selected through simple random sampling from January 3 to February 30, 2024. Data were collected using an interviewer-administered questionnaire covering socio-demographic, behavioral, and clinical information. Sexual dysfunction in males was assessed using the International Index of Erectile Function-5, while the Female Sexual Function Index-6 was used for females. Data were entered into Epidata version 3.6 and later exported to SPSS version 25 for analysis. Binary logistic regression was employed to identify factors associated with sexual dysfunction, with a significance level set at p ≤ 0.05.ResultA total of 423 heart failure patients participated in the study, achieving a 100% response rate. The results indicated a significant prevalence of sexual dysfunction, affecting 57.92% of participants (95% CI 54.76%–63.76%). Female participants reported a higher prevalence of sexual dysfunction, with 63% of the 138 female heart failure patients affected. Among the 285 male participants, 55.4% (158 patients) were identified as having erectile dysfunction. Multivariable logistic regression analysis revealed that factors such as body mass index, age, insufficient physical activity, and smoking were significantly associated with sexual dysfunction in females. In males, erectile dysfunction was significantly linked to diabetes mellitus, heart failure classification, age, and insufficient physical activity.Conclusion and recommendationThis study identified a high prevalence of sexual dysfunction, with females being more affected than males. Additionally, the research identified several factors influencing sexual dysfunction among patients with heart failure, including BMI, age, cigarette smoking, diabetes mellitus, and the classification of heart failure. The study recommends that healthcare providers and other stakeholders take proactive measures to alleviate the burden of sexual dysfunction in patients with heart failure. Strategies should focus on controlling the severity of heart failure symptoms, effectively managing comorbidities, and addressing factors such as body weight, psychological well-being, and behavioral patterns. By targeting these areas, healthcare providers can work toward minimizing the risk of sexual dysfunction and improving the overall quality of life for patients with heart failure.

Impact of Pulmonary Hypertension on Outcomes After Transcatheter Tricuspid Valve Edge-to-Edge Repair

BackgroundData regarding the association of pulmonary hypertension (PH) and outcomes in patients undergoing transcatheter tricuspid valve edge-to-edge repair (T-TEER) are scarce. ObjectivesThe aims of this study were 1) to investigate the impact of PH on outcomes after T-TEER; and 2) to shed further light on the role of pre- and postcapillary PH in patients undergoing T-TEER for relevant tricuspid regurgitation (TR). MethodsThe study included patients from the EuroTR (European Registry of Transcatheter Repair for Tricuspid Regurgitation) registry (NCT06307262) who underwent T-TEER for relevant TR from 2016 until 2023 with available invasive evaluation of systolic pulmonary artery pressure (sPAP) using right heart catheterization. Study endpoints were procedural TR reduction, improvement in NYHA function class, and a combined endpoint of death or heart failure hospitalization (HFH) at 2 years. ResultsAmong a total of 1,230 patients (mean age 78.6 ± 7.0 years, 51.4% women), increasing sPAP was independently associated with increasing rates of 2-year death or HFH (HR: 1.027; 95% CI: 1.003-1.052; P = 0.030; median survival follow-up 343 days [Q1-Q3: 114-645 days]). No significant survival differences were observed for patients with pre- vs postcapillary PH. Sensitivity analysis revealed an sPAP value of 46 mm Hg as the optimized threshold for the prediction of death or HFH. Being observed in 526 patients (42.8%), elevated sPAP (>46 mm Hg) was associated with more severe heart failure symptoms at baseline and follow-up. Importantly, NYHA functional class significantly improved and TR severity was significantly reduced irrespective of PH. ConclusionsPH is an important outcome predictor in patients undergoing T-TEER for relevant TR. In contrast to previous studies, no significant differences were observed for patients with pre- and postcapillary PH in terms of survival free from HFH.

Quality of Life is an Independent Predictor of Mortality in Patients with Heart Failure: A Prospective Cohort Study from the Colombian Heart Failure Registry (RECOLFACA).

Patients with Heart Failure (HF) commonly have poor quality of life (QoL), secondary to the persistence and severity of HF symptoms. We aimed to evaluate the prognostic value of QoL measures on all-cause mortality in patients with HF from the Colombian registry of heart failure (RECOLFACA). We analyzed data from patients registered in RECOLFACA during 2017-2019. QoL was measured using the EuroQol-5D questionnaire (EQ-5D). From the questionnaire, two independent predictors of mortality were obtained, the visual analogue scale (VAS) and the utility score (US). Primary outcome was all-cause mortality, and secondary variables evaluated were demographic factors, comorbidities, NYHA classification, medications used and laboratory test results. To analyze survival among patients, the Kaplan-Meier method and the hierarchical Cox proportional-hazards regression model were used. This study included 2514 patients from RECOLFACA. Most patients were male (57.6%), and mean age was 67.8 years. Mean value and standard deviation (SD) of the VAS score was 78.8 ± 20.1 points, while the mean and SD of the US score was 0.81 ± 0.20. As the Kaplan-Meier curve illustrated, patients in the lower quartiles of both VAS and US scores had a significantly higher probability of mortality (log-rank test: p<0.001 for both scores). QoL, as calculated by the EQ-5D questionnaire, served as an independent predictor of mortality in patients from RECOLFACA. Further studies may be needed to evaluate whether provision of optimizing therapies and follow-up care based on patients' perceived QoL reduces short- and long-term mortality rates in this population.

Outcomes of Cardiac Resynchronization Therapy by New York Heart Association Class: A Patient-Level Meta-Analysis.

Data on the benefits of cardiac resynchronization therapy (CRT) in patients with severe heart failure symptoms are limited. We investigated the relative effects of CRT in patients with ambulatory New York Heart Association (NYHA) IV versus III functional class at the time of device implantation. In this meta-analysis, we pooled patient-level data from the MIRACLE (Multicenter InSync Randomized Clinical Evaluation), MIRACLE-ICD (Multicenter InSync Implantable Cardioversion Defibrillation Randomized Clinical Evaluation), and COMPANION (Comparison of Medical Therapy, Pacing, and Defibrillation in Heart Failure) trials. Outcomes evaluated were time to the composite end point of the first heart failure hospitalization or all-cause mortality, and time to all-cause mortality alone. The association between CRT and outcomes was evaluated using a Bayesian hierarchical Weibull survival regression model. We assessed if this association differed between NYHA III and IV groups by adding an interaction term between CRT and NYHA class as a random effect. A sensitivity analysis was performed by including data from RAFT (Resynchronization-Defibrillation for Ambulatory Heart Failure). Our pooled analysis included 2309 patients. Overall, CRT was associated with a longer time to heart failure hospitalization or all-cause mortality (adjusted hazard ratio [aHR], 0.79 [95% credible interval [CI], 0.64-0.99]; posterior probability or P=0.044), with a similar association with time to all-cause mortality (aHR, 0.78 [95% CI, 0.59-1.03]; P=0.083). Associations of CRT with outcomes were not significantly different for those in NYHA III and IV classes (ratio of aHR, 0.72 [95% CI, 0.30-1.27]; P=0.23 for heart failure hospitalization/mortality; ratio of aHR, 0.70 [95% CI, 0.35-1.34]; P=0.27 for all-cause mortality alone). The sensitivity analysis, including RAFT data, did not show a significant relative CRT benefit between NYHA III and IV classes. Overall, there was no significant difference in the association of CRT with either outcome for patients in NYHA functional class III compared with functional class IV.

Lymphatic morphology and function in chronic right heart failure due to secondary tricuspid valve regurgitation

BackgroundIn heart failure, the capacity of the lymphatic system dictates symptoms of circulatory congestion. This study aimed at describing structural and functional changes of the lymphatic system in patients with chronic right-sided heart failure. MethodsIndividuals with long-standing severe tricuspid valve regurgitation and symptoms of heart failure were compared with age- gender- and weight-matched controls. Lymphatic structure and function were examined using non-contrast MR lymphangiography and near-infrared fluorescence imaging. Microvascular fluid dynamics and distribution were evaluated using strain gauge plethysmography and bio-impedance. ResultsIn total nine patients and nine controls were included. Lymphatic morphology was unchanged in cases compared to controls with similar thoracic duct diameters 3.1(2.1–3.5) mm vs. 2.0(1.8–2.4) mm (p-value = 0.11), similar lymphatic classifications (p-value 0.34), and an identical number of lymphatic vessels in the legs 6 ± 1 vs. 6 ± 3 vessels/field (p-value = 0.72). Lymphatic function was comparable with contraction frequencies of 0.5 ± 0.2 and 0.5 ± 0.3 /min (p-value = 0.52) and a maximal lymphatic pumping pressure of 60 ± 13 and 57 ± 12 mmHg (p-value = 0.59) for cases and controls respectively. Finally, microvascular capillary filtration, isovolumetric threshold, and fluid distribution were similar between groups (p-value≥0.16 for all comparisons). ConclusionIn this small exploratory study, individuals with severe secondary tricuspid valve regurgitation and right-sided heart failure displayed a largely similar lymphatic anatomy and function. Thoracic duct diameter displayed a trend towards increased size in the patient group. We speculate that cases were indeed stable and optimally treated at the time of examination, and with a lymphatic system largely unaffected by any of the current or prior hemodynamic changes.

Expansion of the National Amyloidosis Centre staging system to detect early mortality in transthyretin cardiac amyloidosis.

Transthyretin cardiac amyloidosis (ATTR-CA) is stratified into prognostic categories using the National Amyloidosis Centre (NAC) staging system. The aims of this study were to further expand the existing NAC staging system to incorporate an additional disease stage that would identify patients at high risk of early mortality. The traditional NAC staging system (stage 1: N-terminal pro-B-type natriuretic peptide [NT-proBNP] ≤3000 ng/L and estimated glomerular filtration rate [eGFR] ≥45 ml/min; stage 3: NT-proBNP >3000 ng/L and eGFR <45 ml/min; stage 2: remainder) was expanded by the introduction of a new stage 4 (defined as NT-proBNP ≥10 000 ng/L irrespective of eGFR) and studied in 2042 patients. The optimal NT-proBNP cut-point was established using time-dependent receiver operating characteristic curves in the subgroup of patients with NAC stage 3 disease. Mortality at 1 year according to NAC stage was 2.3% (n = 20/886) for stage 1, 8.8% (n = 62/706) for stage 2, 10.4% (n = 28/270) for stage 3, and 30.6% (n = 55/180) for stage 4 (log-rank p < 0.001). After adjustment for age, mortality hazard for stage 4 was >15-fold higher than that of stage 1 (hazard ratio [HR] 15.5; 95% confidence interval [CI] 9.3-26.1) and >3-fold higher than that of stage 3 (HR 3.4; 95% CI 2.2-5.4). The increased risk of early mortality was consistent across the different genotypes and subclasses of patients based on the severity of heart failure symptoms and echocardiographic parameters. The proposed modification of the NAC staging system identifies patients with ATTR-CA at a high risk of early mortality, who may benefit from a more intensive treatment strategy, and who are most likely to experience an event early in the course of a clinical trial.

Psychosocial predictors of health behavior adherence in heart-failure patients with comorbid depression: a secondary analysis of the Hopeful Heart trial

BackgroundDepression affects 20–30% of individuals with heart failure (HF), and it is associated with worse health outcomes independent of disease severity. One potential explanation is the adverse impact of depression on HF patients’ adherence to the health behaviors needed to self-manage their condition. The aim of this study is to identify characteristics associated with lower adherence in this population, which could help to recognize individuals at higher risk and eventually tailor health behavior interventions to their needs.MethodsUsing data from a randomized, controlled, collaborative care treatment trial in 629 patients with HF and comorbid depression, we performed mixed effects logistic regression analyses to examine the cross-sectional and prospective relationships between medical and psychosocial variables and health behavior adherence, including adherence to medications, a low-sodium diet, and physician appointments.ResultsIn cross-sectional analyses, married marital status and higher physical health-related quality of life (HRQoL) were associated with greater overall adherence (compared to married, single Odds Ratio [OR] = 0.46, 95% Confidence Interval [CI] = 0.26–0.80; other OR = 0.60, CI = 0.38–0.94; p = .012. Physical HRQoL OR = 1.02, CI = 1.00-1.04, p = .047). Prospectively, greater levels of social support were associated with improved overall adherence one year later (OR = 1.04, 95% CI = 1.00-1.08, p = .037). Social support, HF symptom severity, race and ethnicity, and age were predictors of specific types of adherence. Neither depression nor optimism was significantly associated with adherence outcomes.ConclusionsThese results provide important preliminary information about risk factors for poor adherence in patients with both HF and depression, which could, in turn, contribute to the development of interventions to promote adherence in this high-risk population.Trial registrationhttps://clinicaltrials.gov/ct2/show/NCT02044211; registered 1/21/2014.

The Tricuspid Valve: A Review of Pathology, Imaging, and Current Treatment Options: A Scientific Statement From the American Heart Association.

Tricuspid valve disease is an often underrecognized clinical problem that is associated with significant morbidity and mortality. Unfortunately, patients will often present late in their disease course with severe right-sided heart failure, pulmonary hypertension, and life-limiting symptoms that have few durable treatment options. Traditionally, the only treatment for tricuspid valve disease has been medical therapy or surgery; however, there have been increasing interest and success with the use of transcatheter tricuspid valve therapies over the past several years to treat patients with previously limited therapeutic options. The tricuspid valve is complex anatomically, lying adjacent to important anatomic structures such as the right coronary artery and the atrioventricular node, and is the passageway for permanent pacemaker leads into the right ventricle. In addition, the mechanism of tricuspid pathology varies widely between patients, which can be due to primary, secondary, or a combination of causes, meaning that it is not possible for 1 type of device to be suitable for treatment of all cases of tricuspid valve disease. To best visualize the pathology, several modalities of advanced cardiac imaging are often required, including transthoracic echocardiography, transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, to best visualize the pathology. This detailed imaging provides important information for choosing the ideal transcatheter treatment options for patients with tricuspid valve disease, taking into account the need for the lifetime management of the patient. This review highlights the important background, anatomic considerations, therapeutic options, and future directions with regard to treatment of tricuspid valve disease.

Resection of hypertrophic papillary muscles and mitral valve replacement in a patient with midventricular hypertrophic obstructive cardiomyopathy – a new approach

Midventricular hypertrophic obstructive cardiomyopathy (HOCM) is characterized by hypertrophy of the interventricular septum (IVS) and - in rare cases - of the papillary muscles (PM), which subsequently can cause dynamic left ventricular outflow tract obstruction (LVOTO) and severe heart failure symptoms. We report on a rare case of a 44-year-old patient suffering from midventricular HOCM with hypertrophic anterolateral PM and an additional chorda between the PM and the IVS.We describe a new surgical approach via right anterolateral thoracotomy in 3-dimensional (3D) video-assisted minimal-invasive technique with resection of hypertrophic PMs as well as the entire mitral valve-apparatus and valve replacement avoiding septal myectomy and potentially associated complications. After an uneventful procedure clinical symptoms improved from NYHA III-IV at baseline to NYHA 0-I postoperatively and remained stable over a follow-up period of 24 months. Therefore, the presented technique may be considered as a new and alternative approach in patients with hypertrophic PMs and hypertrophic IVS as subtype of midventricular HOCM.

Vutrisiran (Amvuttra)

&#x0D; CADTH recommends that Amvuttra be reimbursed by public drug plans for the treatment of stage 1 or stage 2 polyneuropathy in adult patients with hereditary transthyretin-mediated (hATTR) amyloidosis if certain conditions are met.&#x0D; Amvuttra should only be covered to treat adults with stage 1 or stage 2 genetically confirmed hATTR amyloidosis with polyneuropathy (hATTR-PN) who are symptomatic with early-stage neuropathy, do not have severe heart failure symptoms, and have not had a liver transplant. A patient’s response to treatment with Amvuttra should be assessed at least every 6 months to determine whether they would benefit from continued treatment. Treatment with Amvuttra should not be continued in patients who are permanently bedridden and dependent on assistance for basic activities of daily living or who are receiving end-of-life care.&#x0D; Amvuttra should only be reimbursed if the patient is under the care of a specialist with experience in the diagnosis and management of hATTR-PN and should not be reimbursed if it is used in combination with interfering ribonucleic acid drugs or transthyretin stabilizers. The cost of Amvuttra should be reduced so that it does not cost more than other drugs for hATTR amyloidosis.&#x0D;

An Insidious Comorbidity: Depression and Chronic Heart Failure

Depression and heart failure (HF) are in a close bilateral relationship: the appearance of one is potentiated by the other entity and the effects on the outcome are amplified within this ”dangerous liaison”. Depression is present in about one in five HF patients and is severe in almost half of them. The risk factors for depression in HF are female gender, elderly, the severity of HF symptoms, a history of isolated systolic hypertension or coronary heart disease (CHD), previous hospitalization and previous depressive episodes. Depression in HF patients increases medication non-adherence, is associated with poor outcomes after heart transplantation and is predictive of events and mortality in cardiac resynchronization therapy (CRT). The current assumptions related to the physiopathological mechanisms include a common genetic polymorphisms, inflammation, generalized immune system disturbance, disturbances of platelet function, increased catecholamine levels, deamination of monoamines linked with monoamine oxidase activity, a dysregulation of the autonomic nervous system, increased serotonin levels, ischemia, life-style and medication non-compliance, a poor familial support and social isolation. Assessment of cognitive functioning should be part of routine clinical examination in HF, the diagnosis of depression is based on questionnaires (e.g. Patient Health Questionnaire-2 - PHQ-2) and on clinical interview. There is still no consensus on the best therapy for HF patients with depression (non-pharmacological and pharmacological therapies) but, for sure, depression in HF is harder to treat.

Human Genetics of Ebstein Anomaly.

Ebstein anomaly (EA) is a rare, congenital cardiac defect of the tricuspid valve with a birth prevalence between 0.5 and 1 in 20,000 [1]. It is characterized by displacement of the tricuspid valve toward the apex of the right ventricle (RV) and "atrialization" of the RV (Fig. 57.1) [2]. EA accounts for about 0.5% of all congenital heart diseases (CHD) [2]. Depending on severity of the defect and due to heterogeneity of the disease, patient's presentation varies from severe heart failure symptoms and arrhythmia in neonatal life to asymptomatic adults.

Depression and heart failure - the incidence of depressive symptoms assessed by the PHQ9 and their association with HF outcomes

Abstract Background Depression is a prevalent, debilitating, and potentially modifiable comorbidity that affects heart failure (HF) patients and is associated with increased mortality and morbidity. Current HF guidelines recommend addressing depression using questionnaires, including the Patient Health Questionnaire-9 (PHQ-9), which is a 9-item questionnaire that can be self-administered in less than 3 minutes. Objectives To evaluate the performance of PHQ-9 in a Portuguese cohort of HF patients and present a practical approach for the screening and management of depression in patients with HF. Methods We conducted a prospective study enrolling consecutive patients observed in the HF clinic from June 2022 to August 2022. Patients answered the PHQ-9 before the beginning of the appointment, during which the physician evaluated the results and managed them according to the suggested protocol. – figure 1. We then evaluated the severity of depression by PHQ-9 at baseline and its association with the levels of serum NT-proBNP, the severity of HF symptoms, and the incidence of a composite of all-cause mortality and HF exacerbation at 90-days follow-up. Results A total of 124 patients were observed in the HF consultation and answered PHQ-9: mean age of 74±12,1years, 68% (n=84) male, 82% (n=102) with NYHA I-II, 48% (n=60) with preserved ejection fraction, 23% (n=29) with a previous diagnosis of depression and on antidepressant therapy. After the evaluation of PHQ-9, 52% (n=65), 27% (n=33), 10% (n=12), 8% (n=10) and 3% (n=4) had minimal, mild, moderate, moderately severe, and severe depression symptoms, respectively. In the no previous depression diagnosis group, 49% (n=46) had at least mild depressive symptoms. The presence of depressive symptoms was associated with higher levels of serum NT-proBNP (r=0.31; p&amp;lt;0.001) and worse HF symptoms assessed by the NYHA classification (p&amp;lt;0,001). Patients who met the composite outcomes (death or HF exacerbation) during follow-up had a significantly higher PHQ-9 score (10±7 vs. 5±5; p&amp;lt;0,001). Conclusions This study confirmed the high prevalence of depressive symptoms in this real-world cohort of HF patients and demonstrated that screening for depressive symptoms can be efficiently integrated in the HF consultation. A higher burden of depressive symptoms was associated with higher serum NT-proBNP, more severe symptoms and higher incidence of death or HF admissions.

Choice of cardiac implantable electronic device and age-stratified clinical outcome in patients with known heart failure prior to implant

Abstract Background Patients with heart failure (HF) and bradycardia may be eligible for different types of cardiac implantable devices, depending on presence of AV conduction disease, left ventricular ejection fraction, severity of heart failure symptoms, other comorbidities, and age. Once a pacing device is in place, there is a risk that ensuing heart failure progression or worsening of symptoms do not always prompt a device upgrade to resynchronization or conduction system pacing therapy, even if this should be warranted. We therefore aimed to compare prognosis in a national cohort of patients with prior HF diagnosis, who received a pacemaker or defibrillator with or without resynchronization therapy. Methods All patients with preexisting HF diagnosis who received a pacemaker with a right ventricular lead during the period 2005-2018 in Sweden were identified via the Pacemaker Registry. Data was crossmatched with the population registry and national disease registries. Primary outcome was 5-year mortality, and secondary was mode of death and hospitalization for heart failure. Cox regression and Kaplan Meier curves with log rank test were used for time dependent outcome analyses. Demography variables were compared with Chi2 test or Mann Whitney U test, as appropriate. Adjustment was done for history of atrial fibrillation, diabetes, hypertension, malignancy, age, gender, ischemic heart disease, renal disease, lung disease and cerebrovascular disease. Separate analyses were made for patients with complete AV block, based on the assumption of 100% ventricular pacing in this group, regardless of choice of implanted device. Results 37745 patients were included in the study (see figure 1). Demographic data was similar for the different device groups, except that patients with single chamber pacemaker were older, and all those with single chamber devices (pacemaker, ICD) were more likely to have a history of atrial fibrillation. All listed comorbidities, male sex and higher age were associated with lower 5-year survival in multivariable Cox regression analyses. Unadjusted 5-year mortality was 40% (highest for single chamber pacemakers (61%), and lowest for dual chamber ICDs (17%), p=0.001). When stratified by age group, and adjusted for relevant covariables, patients with single-chamber pacemakers still had higher mortality in all age decile groups (ranging from &amp;lt;60 to &amp;gt;90 years old, all p&amp;lt;0.001). Proportion of device upgrades to CRT was very low; 2.9% for dual- and 0.8% for single-chamber pacemakers. 17% of patients with traditional pacemakers died from HF, and 31% were hospitalized for HF. Conclusions Survival for patients with prior HF diagnosis who receive traditional pacemakers is poor, risk of HF hospitalization is high, whereas upgrades to CRT are scarce. This highlights the need for choosing correct device type at the primary implant, if ventricular pacing is anticipated.Flowchart of included patientsAge stratified survival per device group