Search

Background: Metoprolol is commonly administered to critically ill patients; however, its effect on mortality in patients with sepsis-induced cardiomyopathy (SICM) remains uncertain. This study aimed to investigate the relationship between metoprolol use and mortality in patients with SICM. Methods: Adults with SICM were identified from the MIMIC-IV database. The exposure of interest was metoprolol treatment. The outcomes assessed were 30-day mortality, 1-year mortality, and in-hospital mortality. Kaplan–Meier survival analysis evaluated the effect of metoprolol on these outcomes. Multivariable Cox proportional hazards and logistic regression analyses were performed to determine the correlation between metoprolol treatment and mortality in patients with SICM. Results: 1163 patients with SICM were identified, with 882 receiving metoprolol treatment (MET group) and 281 not receiving metoprolol treatment (NOMET group). Overall, the 30-day, 1-year, and in-hospital mortality rates were 10.2%, 18.2%, and 8.9%, respectively. Significant differences in mortality existed between the groups. Multivariable Cox analysis revealed that patients in the NOMET group had a higher risk of 1-year mortality (adjusted hazard ratio [HR] 2.493; 95% confidence interval [CI] 1.800–3.451; P < 0.001) and 30-day mortality (adjusted HR 4.280; 95%CI 2.760–6.637; P < 0.001). Metoprolol treatment was associated with lower in-hospital mortality (odds ratio [OR] 5.076; 95% CI 2.848–9.047; P < 0.001). Subgroup analysis supported these findings. Conclusion: Metoprolol treatment is associated with reduced all-cause mortality in patients with SICM. Prospective studies are required to validate these findings. [ABSTRACT FROM AUTHOR]

Abstract Background Metoprolol is commonly administered to critically ill patients; however, its effect on mortality in patients with sepsis-induced cardiomyopathy (SICM) remains uncertain. This study aimed to investigate the relationship between metoprolol use and mortality in patients with SICM. Methods Adults with SICM were identified from the MIMIC-IV database. The exposure of interest was metoprolol treatment. The outcomes assessed were 30-day mortality, 1-year mortality, and in-hospital mortality. Kaplan–Meier survival analysis evaluated the effect of metoprolol on these outcomes. Multivariable Cox proportional hazards and logistic regression analyses were performed to determine the correlation between metoprolol treatment and mortality in patients with SICM. Results 1163 patients with SICM were identified, with 882 receiving metoprolol treatment (MET group) and 281 not receiving metoprolol treatment (NOMET group). Overall, the 30-day, 1-year, and in-hospital mortality rates were 10.2%, 18.2%, and 8.9%, respectively. Significant differences in mortality existed between the groups. Multivariable Cox analysis revealed that patients in the NOMET group had a higher risk of 1-year mortality (adjusted hazard ratio [HR] 2.493; 95% confidence interval [CI] 1.800–3.451; P

Abstract Background Zolpidem is a non-benzodiazepine hypnotic widely used to manage insomnia. Zolpidem-triggered atrial fibrillation (AF) in patients with cardiomyopathy has never been reported before. Case presentation A 40-year-old man with Duchenne muscular dystrophy-related cardiomyopathy attempted suicide and developed new-onset AF after zolpidem overdose. One year before admission, the patient visited our clinic due to chest discomfort and fatigue after daily walks for 1 month; both electrocardiography (ECG) and 24-hour Holter ECG results did not detect AF. After administration of cardiac medication (digoxin 0.125 mg/day, spironolactone 40 mg/day, furosemide 20 mg/day, bisoprolol 5 mg/day, sacubitril/valsartan 12/13 mg/day), he felt better. AF had never been observed before this admission via continuous monitoring during follow-up. Sixteen days before admission, the patient saw a sleep specialist and started zolpidem tartrate tablets (10 mg/day) due to insomnia for 6 months; ECG results revealed no significant change. The night before admission, the patient attempted suicide by overdosing on 40 mg of zolpidem after an argument, which resulted in severe lethargy. Upon admission, his ECG revealed new-onset AF, necessitating immediate cessation of zolpidem. Nine hours into admission, AF spontaneously terminated into normal sinus rhythm. Results from the ECG on the following days and the 24-hour Holter ECG at 1-month follow-up showed that AF was not detected. Conclusions This study provides valuable clinical evidence indicating that zolpidem overdose may induce AF in patients with cardiomyopathy. It serves as a critical warning for clinicians when prescribing zolpidem, particularly for patients with existing heart conditions. Further large-scale studies are needed to validate this finding and to explore the mechanisms between zolpidem and AF.

Background: Zolpidem is a non-benzodiazepine hypnotic widely used to manage insomnia. Zolpidem-triggered atrial fibrillation (AF) in patients with cardiomyopathy has never been reported before. Case presentation: A 40-year-old man with Duchenne muscular dystrophy-related cardiomyopathy attempted suicide and developed new-onset AF after zolpidem overdose. One year before admission, the patient visited our clinic due to chest discomfort and fatigue after daily walks for 1 month; both electrocardiography (ECG) and 24-hour Holter ECG results did not detect AF. After administration of cardiac medication (digoxin 0.125 mg/day, spironolactone 40 mg/day, furosemide 20 mg/day, bisoprolol 5 mg/day, sacubitril/valsartan 12/13 mg/day), he felt better. AF had never been observed before this admission via continuous monitoring during follow-up. Sixteen days before admission, the patient saw a sleep specialist and started zolpidem tartrate tablets (10 mg/day) due to insomnia for 6 months; ECG results revealed no significant change. The night before admission, the patient attempted suicide by overdosing on 40 mg of zolpidem after an argument, which resulted in severe lethargy. Upon admission, his ECG revealed new-onset AF, necessitating immediate cessation of zolpidem. Nine hours into admission, AF spontaneously terminated into normal sinus rhythm. Results from the ECG on the following days and the 24-hour Holter ECG at 1-month follow-up showed that AF was not detected. Conclusions: This study provides valuable clinical evidence indicating that zolpidem overdose may induce AF in patients with cardiomyopathy. It serves as a critical warning for clinicians when prescribing zolpidem, particularly for patients with existing heart conditions. Further large-scale studies are needed to validate this finding and to explore the mechanisms between zolpidem and AF. [ABSTRACT FROM AUTHOR]

Abstract Sudden cardiac death (SCD) is a major public health issue worldwide. In the young (

Abstract Background Neurofibromatosis type I (NF1) is a genetic disorder characterized by the tumor’s development in nerve tissue. Complications of NF1 can include pigmented lesions, skin neurofibromas, and heart problems such as cardiomyopathy. In this study, we performed whole-exome sequencing (WES) on an Iranian patient with NF1 to identify the genetic cause of the disease. Methods Following clinical assessment, WES was used to identify genetic variants in a family with a son suffering from NF1. No symptomatic manifestations were observed in other family members. In the studied family, in silico and segregation analysis were applied to survey candidate variants. Results Clinical manifestations were consistent with arrhythmogenic cardiomyopathy (ACM). WES detected a likely pathogenic heterozygous missense variant, c.3277G > A:p.Val1093Met, in the NF1 gene, confirmed by PCR and Sanger sequencing. The patient’s parents and brother had a normal sequence at this locus. Conclusions Although there is no cure for NF1, genetic tests, such as WES, can detect at-risk asymptomatic family members. Furthermore, cardiac evaluation could also help these patients before heart disease development.

Sudden cardiac death (SCD) is a major public health issue worldwide. In the young (< 40 years of age), genetic cardiomyopathies and viral myocarditis, sometimes in combination, are the most frequent, but underestimated, causes of SCD. Molecular autopsy is essential for prevention. Several studies have shown an association between genetic cardiomyopathies and viral myocarditis, which is probably underestimated due to insufficient post-mortem investigations. We report on four autopsy cases illustrating the pathogenesis of these combined pathologies. In two cases, a genetic hypertrophic cardiomyopathy was diagnosed in combination with Herpes Virus Type 6 (HHV6) and/or Parvovirus-B19 (PVB19) in the heart. In the third case, autopsy revealed a dilated cardiomyopathy and virological analyses revealed acute myocarditis caused by three viruses: PVB19, HHV6 and Epstein-Barr virus. Genetic analyses revealed a mutation in the gene coding for desmin. The fourth case illustrated a channelopathy and a PVB19/HHV6 coinfection. Our four cases illustrate the highly probable deleterious role of cardiotropic viruses in the occurrence of SCD in subjects with genetic cardiomyopathies. We discuss the pathogenetic link between viral myocarditis and genetic cardiomyopathy. Molecular autopsy is essential in prevention of these SCD, and a close collaboration between cardiologists, pathologists, microbiologists and geneticians is mandatory. [ABSTRACT FROM AUTHOR]

Background: Neurofibromatosis type I (NF1) is a genetic disorder characterized by the tumor's development in nerve tissue. Complications of NF1 can include pigmented lesions, skin neurofibromas, and heart problems such as cardiomyopathy. In this study, we performed whole-exome sequencing (WES) on an Iranian patient with NF1 to identify the genetic cause of the disease. Methods: Following clinical assessment, WES was used to identify genetic variants in a family with a son suffering from NF1. No symptomatic manifestations were observed in other family members. In the studied family, in silico and segregation analysis were applied to survey candidate variants. Results: Clinical manifestations were consistent with arrhythmogenic cardiomyopathy (ACM). WES detected a likely pathogenic heterozygous missense variant, c.3277G > A:p.Val1093Met, in the NF1 gene, confirmed by PCR and Sanger sequencing. The patient's parents and brother had a normal sequence at this locus. Conclusions: Although there is no cure for NF1, genetic tests, such as WES, can detect at-risk asymptomatic family members. Furthermore, cardiac evaluation could also help these patients before heart disease development. [ABSTRACT FROM AUTHOR]

Abstract Background Primary carnitine deficiency (PCD) denotes low carnitine levels with an autosomal recessive pattern of inheritance. Cardiomyopathy is the most common cardiac symptom in patients with PCD, and early diagnosis can prevent complications. Next-generation sequencing can identify genetic variants attributable to PCD efficiently. Objective We aimed to detect the genetic cause of the early manifestations of hypertrophic cardiomyopathy and metabolic abnormalities in an Iranian family. Methods We herein describe an 8-year-old boy with symptoms of weakness and lethargy diagnosed with PCD through clinical evaluations, lab tests, echocardiography, and cardiac magnetic resonance imaging. The candidate variant was confirmed through whole-exome sequencing, polymerase chain reaction, and direct Sanger sequencing. The binding efficacy of normal and mutant protein-ligand complexes were evaluated via structural modeling and docking studies. Results Clinical evaluations, echocardiography, and cardiac magnetic resonance imaging findings revealed hypertrophic cardiomyopathy as a clinical presentation of PCD. Whole-exome sequencing identified a new homozygous variant, SLC22A5 (NM_003060.4), c.821G > A: p.Trp274Ter, associated with carnitine transport. Docking analysis highlighted the impact of the variant on carnitine transport, further indicating its potential role in PCD development. Conclusions The c.821G > A: p.Trp274Ter variant in SLC22A5 potentially acted as a pathogenic factor by reducing the binding affinity of organic carnitine transporter type 2 proteins for carnitine. So, the c.821G > A variant may be associated with carnitine deficiency, metabolic abnormalities, and cardiomyopathic characteristics.

Background: Primary carnitine deficiency (PCD) denotes low carnitine levels with an autosomal recessive pattern of inheritance. Cardiomyopathy is the most common cardiac symptom in patients with PCD, and early diagnosis can prevent complications. Next-generation sequencing can identify genetic variants attributable to PCD efficiently. Objective: We aimed to detect the genetic cause of the early manifestations of hypertrophic cardiomyopathy and metabolic abnormalities in an Iranian family. Methods: We herein describe an 8-year-old boy with symptoms of weakness and lethargy diagnosed with PCD through clinical evaluations, lab tests, echocardiography, and cardiac magnetic resonance imaging. The candidate variant was confirmed through whole-exome sequencing, polymerase chain reaction, and direct Sanger sequencing. The binding efficacy of normal and mutant protein-ligand complexes were evaluated via structural modeling and docking studies. Results: Clinical evaluations, echocardiography, and cardiac magnetic resonance imaging findings revealed hypertrophic cardiomyopathy as a clinical presentation of PCD. Whole-exome sequencing identified a new homozygous variant, SLC22A5 (NM_003060.4), c.821G > A: p.Trp274Ter, associated with carnitine transport. Docking analysis highlighted the impact of the variant on carnitine transport, further indicating its potential role in PCD development. Conclusions: The c.821G > A: p.Trp274Ter variant in SLC22A5 potentially acted as a pathogenic factor by reducing the binding affinity of organic carnitine transporter type 2 proteins for carnitine. So, the c.821G > A variant may be associated with carnitine deficiency, metabolic abnormalities, and cardiomyopathic characteristics. [ABSTRACT FROM AUTHOR]

Abstract Introduction Inequities in clinical care may contribute to racial disparities observed in studies of heart disease morbidity and cardiogenetic testing outcomes. There is a lack of research aimed at understanding the complexity of those inequities, but stigma likely contributes. This qualitative exploratory study helps close that gap in the literature by describing intersectional stigma manifestations perceived by the Black cardiomyopathy patient population at one academic medical center. Methods Qualitative interviews were conducted with 14 Black cardiomyopathy patients. Interviews aimed to elicit patients’ experiences with discrimination related to diagnosis, symptoms, genetic testing, knowledge of genetic results, genetic counseling, providers’ actions, and providers’ communication. The interview guide was informed by The Health Stigma and Discrimination Framework. Data were also collected about participant demographics, type of cardiomyopathy, age of diagnosis, documentation of relevant family history, and completion of genetic counseling and/or genetic testing. Results More than half of participants reported intersectional stigma manifestations related to their race, age, and/or weight while receiving care from cardiologists, nurse practitioners, genetic counselors, or clinical support staff. Stigma manifestations included physical roughness during patient care, withholding diagnostically-relevant information from the patient, impersonal care, coercion, and use of offensive stereotyped language by providers. These stigma manifestations impacted access to care, uptake of genetic testing, timeline to diagnosis, patient emotion, patient-provider trust, and adherence to medical recommendations. Conclusions This study provides nuanced qualitative descriptions of stigma manifestations that affect patient and clinical outcomes in cardiology care and genetic services in one medical center in the Southeastern United States. The results of this study suggest that provider bias and stigma manifestations have an adverse effect on cardiogenetic and clinical outcomes among Black cardiomyopathy patients. Clinical interventions are suggested to assist health professionals in providing culturally-competent and respectful care. These results help inform patient-provider communication, clinical policies, and evidence-based practice in cardiology care and genetics. Continued study of this topic across more institutions and with a larger sample size is needed to confirm the generalizability of the conclusions.

Abstract Background Monkeypox is a zoonotic viral infection first reported in May 2022. Monkeypox cases present with prodromal symptoms, rash, and/or systemic complications. This study systematically reviews the monkeypox cases presented with any cardiac complications. Methods A systematic literature search was done to locate papers that discuss any cardiac complications associated with monkeypox; then, data were analyzed qualitatively. Results Nine articles, including the 13 cases that reported cardiac complications of the disease, were included in the review. Five cases previously had sex with men, and two cases had unprotected intercourse, which reveals the importance of the sexual route in disease transmission. All cases have a wide spectrum of cardiac complications, such as acute myocarditis, pericarditis, pericardial effusion, and myopericarditis. Conclusion This study clarifies the potential for cardiac complications in monkeypox cases and provides avenues for future research to determine the underlying mechanism. Also, we found that the cases with pericarditis were treated with colchicine, and those with myocarditis were treated with supportive care or cardioprotective treatment (Bisoprolol and Ramipril). Furthermore, Tecovirimat is used as an antiviral drug for 14 days.

Introduction: Inequities in clinical care may contribute to racial disparities observed in studies of heart disease morbidity and cardiogenetic testing outcomes. There is a lack of research aimed at understanding the complexity of those inequities, but stigma likely contributes. This qualitative exploratory study helps close that gap in the literature by describing intersectional stigma manifestations perceived by the Black cardiomyopathy patient population at one academic medical center. Methods: Qualitative interviews were conducted with 14 Black cardiomyopathy patients. Interviews aimed to elicit patients' experiences with discrimination related to diagnosis, symptoms, genetic testing, knowledge of genetic results, genetic counseling, providers' actions, and providers' communication. The interview guide was informed by The Health Stigma and Discrimination Framework. Data were also collected about participant demographics, type of cardiomyopathy, age of diagnosis, documentation of relevant family history, and completion of genetic counseling and/or genetic testing. Results: More than half of participants reported intersectional stigma manifestations related to their race, age, and/or weight while receiving care from cardiologists, nurse practitioners, genetic counselors, or clinical support staff. Stigma manifestations included physical roughness during patient care, withholding diagnostically-relevant information from the patient, impersonal care, coercion, and use of offensive stereotyped language by providers. These stigma manifestations impacted access to care, uptake of genetic testing, timeline to diagnosis, patient emotion, patient-provider trust, and adherence to medical recommendations. Conclusions: This study provides nuanced qualitative descriptions of stigma manifestations that affect patient and clinical outcomes in cardiology care and genetic services in one medical center in the Southeastern United States. The results of this study suggest that provider bias and stigma manifestations have an adverse effect on cardiogenetic and clinical outcomes among Black cardiomyopathy patients. Clinical interventions are suggested to assist health professionals in providing culturally-competent and respectful care. These results help inform patient-provider communication, clinical policies, and evidence-based practice in cardiology care and genetics. Continued study of this topic across more institutions and with a larger sample size is needed to confirm the generalizability of the conclusions. [ABSTRACT FROM AUTHOR]

Background: Monkeypox is a zoonotic viral infection first reported in May 2022. Monkeypox cases present with prodromal symptoms, rash, and/or systemic complications. This study systematically reviews the monkeypox cases presented with any cardiac complications. Methods: A systematic literature search was done to locate papers that discuss any cardiac complications associated with monkeypox; then, data were analyzed qualitatively. Results: Nine articles, including the 13 cases that reported cardiac complications of the disease, were included in the review. Five cases previously had sex with men, and two cases had unprotected intercourse, which reveals the importance of the sexual route in disease transmission. All cases have a wide spectrum of cardiac complications, such as acute myocarditis, pericarditis, pericardial effusion, and myopericarditis. Conclusion: This study clarifies the potential for cardiac complications in monkeypox cases and provides avenues for future research to determine the underlying mechanism. Also, we found that the cases with pericarditis were treated with colchicine, and those with myocarditis were treated with supportive care or cardioprotective treatment (Bisoprolol and Ramipril). Furthermore, Tecovirimat is used as an antiviral drug for 14 days. [ABSTRACT FROM AUTHOR]

Abstract Background Right ventricular (RV) dysfunction in patients with non-ischemic dilated cardiomyopathy (NICM) is associated with cardiovascular events. To analyze the feasibility of assessing RV myocardial deformation by feature tracking (FT)-cardiac magnetic resonance (CMR), and its usefulness as a prognostic marker. Methods Retrospective study of NICM patients undergoing CMR. Longitudinal FT-RV free wall (LFT-RVFW) and fractional area change (FAC) were obtained. Correlation with standard RV parameters was studied. An association with combined event (heart failure (HF), ICD implantation or cardiovascular death) was assessed using a logistic regression model. Results 98 patients (64 ± 13 years) were included. Left ventricular (LV) systolic function (LVEF 29.5 ± 9.6%, 47% with LVEF ≥ 30%) and RV (RVEF 52.2 ± 14.6%, 72% with RVEF ≥ 45%). Follow-up of 38 ± 17 months, 26.5% presented at least one admission for HF. An excellent correlation of LFT-RVFW (r = 0.82) and FAC (r = 0.83) with RVEF was evident. No association of RV-FT parameters with prognosis entire study population was found. However, in patients with LVEF ≥ 30%, admissions for HF were associated with lower LFT-RVFW (−21.6 ± 6.6% vs −31.3 ± 10%; p = 0.006) and FAC (36.6 ± 9.6% vs 50.5 ± 13.4%; p < 0.001) values. Similar differences were observed when only patients with RVEF ≥ 45% were considered. An LFT-RVFW cut-off point of -19.5% and FAC of 36.5% showed good prognostic performance. Decreased LFT-RVFW or FAC represented an independent predictor of combined event in patients with LVEF ≥ 30%. Conclusions In NICM patients without severe LV dysfunction, decreased values of LFT-RVFW and/or FAC were associated with HF admissions, independently of RVEF.

Abstract Purpose We aimed to analyze the risk of cardiomyopathies (CMPs) among first-generation and second-generation immigrants. Methods All individuals aged 18 years of age and older, n = 6,123,661 in the first-generation study, and n = 4,587,764 in the second-generation study were analyzed. CMP was defined as at least one registered diagnosis in the National Patient Register between January 1, 1998 and December 31, 2018. Cox regression analysis was used to estimate the relative risk (hazard ratios (HR) with 99% confidence intervals (CI)) of incident CMP with adjustments made for age, cancer, other comorbidities, and sociodemographic factors. Results In the first-generation study, a total of 33,321 CMP cases were registered, 20,780 men and 12,541 women, where the fully adjusted models showed HRs (99% CI) for all foreign-born men of 0.92 (0.86–0.98) and for women of 0.90 (0.83–0.98). For dilated CMP, the risk was higher for men from Nordic countries, more specifically men from Finland, and lower for men and women from Asia. For hypertrophic CMP, the risk was higher for men from Africa and Asia. For other types of CMPs, the risk was lower in men and women from Asia. In the second-generation study, a total of 26,559 cases were registered (17,620 men and 8939 women), with no significant differences overall or among specific groups, when Swedish-born with foreign-born parents were compared to Swedish-born with Swedish-born parents. Conclusions We observed a generally lower risk of CMPs among foreign-born individuals, but with a higher risk especially for hypertrophic CMPs for men from Africa and Asia, and a higher risk of dilated CMP for men from Nordic countries.

Background: Right ventricular (RV) dysfunction in patients with non-ischemic dilated cardiomyopathy (NICM) is associated with cardiovascular events. To analyze the feasibility of assessing RV myocardial deformation by feature tracking (FT)-cardiac magnetic resonance (CMR), and its usefulness as a prognostic marker. Methods: Retrospective study of NICM patients undergoing CMR. Longitudinal FT-RV free wall (LFT-RVFW) and fractional area change (FAC) were obtained. Correlation with standard RV parameters was studied. An association with combined event (heart failure (HF), ICD implantation or cardiovascular death) was assessed using a logistic regression model. Results: 98 patients (64 ± 13 years) were included. Left ventricular (LV) systolic function (LVEF 29.5 ± 9.6%, 47% with LVEF ≥ 30%) and RV (RVEF 52.2 ± 14.6%, 72% with RVEF ≥ 45%). Follow-up of 38 ± 17 months, 26.5% presented at least one admission for HF. An excellent correlation of LFT-RVFW (r = 0.82) and FAC (r = 0.83) with RVEF was evident. No association of RV-FT parameters with prognosis entire study population was found. However, in patients with LVEF ≥ 30%, admissions for HF were associated with lower LFT-RVFW (−21.6 ± 6.6% vs −31.3 ± 10%; p = 0.006) and FAC (36.6 ± 9.6% vs 50.5 ± 13.4%; p < 0.001) values. Similar differences were observed when only patients with RVEF ≥ 45% were considered. An LFT-RVFW cut-off point of -19.5% and FAC of 36.5% showed good prognostic performance. Decreased LFT-RVFW or FAC represented an independent predictor of combined event in patients with LVEF ≥ 30%. Conclusions: In NICM patients without severe LV dysfunction, decreased values of LFT-RVFW and/or FAC were associated with HF admissions, independently of RVEF. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to analyze the risk of cardiomyopathies (CMPs) among first-generation and second-generation immigrants. Methods: All individuals aged 18 years of age and older, n = 6,123,661 in the first-generation study, and n = 4,587,764 in the second-generation study were analyzed. CMP was defined as at least one registered diagnosis in the National Patient Register between January 1, 1998 and December 31, 2018. Cox regression analysis was used to estimate the relative risk (hazard ratios (HR) with 99% confidence intervals (CI)) of incident CMP with adjustments made for age, cancer, other comorbidities, and sociodemographic factors. Results: In the first-generation study, a total of 33,321 CMP cases were registered, 20,780 men and 12,541 women, where the fully adjusted models showed HRs (99% CI) for all foreign-born men of 0.92 (0.86–0.98) and for women of 0.90 (0.83–0.98). For dilated CMP, the risk was higher for men from Nordic countries, more specifically men from Finland, and lower for men and women from Asia. For hypertrophic CMP, the risk was higher for men from Africa and Asia. For other types of CMPs, the risk was lower in men and women from Asia. In the second-generation study, a total of 26,559 cases were registered (17,620 men and 8939 women), with no significant differences overall or among specific groups, when Swedish-born with foreign-born parents were compared to Swedish-born with Swedish-born parents. Conclusions: We observed a generally lower risk of CMPs among foreign-born individuals, but with a higher risk especially for hypertrophic CMPs for men from Africa and Asia, and a higher risk of dilated CMP for men from Nordic countries. [ABSTRACT FROM AUTHOR]

Abstract Background The role of the dysfunction of left atrium in the occurrence and development of cardiovascular disease has been gradually recognized. We aim to compare the impact on left atrial (LA) function between patients with hypertrophic cardiomyopathy (HCM) and hypertension (HTN) without LA enlargement using cardiovascular magnetic resonance feature tracking (CMR-FT), and if possible, explore the capability of LA function for providing clinical implication and predicting clinical adverse events in the early stage of cardiovascular disease. Methods Consecutive 60 HCM patients and 60 HTN patients with normal LA size among 1413 patients who underwent CMR were retrospectively analyzed as well as 60 controls. Left atrial and ventricular functions were quantified by volumetric and CMR-FT derived strain analysis from long and short left ventricular view cines. The primary endpoint was a composite of all-cause death, stroke, new-onset or worsening heart failure to hospitalization, and paroxysmal or persistent atrial fibrillation. Results Compared to the controls, both HTN and HCM participants had impaired LA reservoir function (εs) and conduit function (εe) with the different stage of LA booster pump dysfunction (εa). LA strain was more sensitive than LV longitudinal strain (GLS) for evaluate primary endpoint (εs: 33.9% ± 7.5 vs. 41.2% ± 14.3, p = 0.02; εe: 13.6% ± 6.2 vs. 17.4% ± 10.4, p = 0.03; εa: 20.2% ± 6.0 vs. 23.7% ± 8.8, p = 0.07; GLS: -19.4% ± 6.4 vs. -20.0% ± 6.8, p = 0.70, respectively). After a mean follow-up of 6.8 years, 23 patients reached primary endpoint. Cox regression analyses indicated impaired LA reservoir and booster pump strain were associated with clinical outcomes in patients at the early stage of HTN and HCM (p

Abstract Background Self-reported health status as measured by the Kansas City Cardiomyopathy Questionnaire (KCCQ) in patients with primary prevention implantable cardioverter defibrillators (ICDs) has mainly been reported from randomized trials. However, these studies are often limited to short follow-up and are subject to selection bias. The aim of this study was to assess KCCQ-12 in patients with primary prevention ICD due to either ischemic or nonischemic heart failure. Methods This cross-sectional observational study included all patients in Region Gävleborg, Sweden, who because of primary prevention due to heart failure, had an ICD or underwent device replacement between 2007 and 2017. After validation using medical records patients were sent and returned the KCCQ-12 by regular mail. Results A total of 118 questionnaires were analyzed (response rate 71.1%). The mean age was 70.9 ± 9.8 years, and a minority was female (n = 20, 16.9%). The mean overall summary score was 71.5 ± 22.4, there was no significant difference between ischemic and nonischemic heart failure (69.5 ± 23.1 vs. 74.4 ± 21.3; p = 0.195). Atrial fibrillation at baseline was associated with lower score for the domains Symptom frequency (70.2 ± 23.2 vs. 82.2 ± 19.2; p = 0.006) and Social limitation (62.1 ± 26.0 vs. 75.6 ± 26.6; p = 0.006) as well as the overall summary score (63.9 ± 21.3 vs. 74.8 ± 22.2; p = 0.004). Conclusion In a real-world setting, primary prevention ICD patients with heart failure report an acceptable disease-specific health status at long-term follow-up. Ischemic and nonischemic etiology showed similar health status whereas atrial fibrillation was associated with worse outcome.

Abstract Background This study aimed to determine native T1 and extracellular volume fraction (ECV) in distinct types of myocardial disease, including amyloidosis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), myocarditis and coronary artery disease (CAD), compared to controls. Methods We retrospectively enrolled patients with distinct types of myocardial disease, CAD patients, and control group (no known heart disease and negative CMR study) who underwent 3.0 Tesla CMR with routine T1 mapping. The region of interest (ROI) was drawn in the myocardium of the mid left ventricular (LV) short axis slice and at the interventricular septum of mid LV slice. ECV was calculated by actual hematocrit (Hct) and synthetic Hct. T1 mapping and ECV was compared between myocardial disease and controls, and between CAD and controls. Diagnostic yield and cut-off values were assessed. Results A total of 1188 patients were enrolled. The average T1 values in the control group were 1304 ± 42 ms at septum, and 1294 ± 37 ms at mid LV slice. The average T1 values in patients with myocardial disease and CAD were significantly higher than in controls (1441 ± 72, 1349 ± 59, 1345 ± 59, 1355 ± 56, and 1328 ± 54 ms for septum of amyloidosis, DCM, HCM, myocarditis, and CAD). Native T1 of the mid LV level and ECV at septum and mid LV with actual and synthetic Hct of patients with myocardial disease or CAD were significantly higher than in controls. Conclusions Although native T1 and ECV of patients with cardiomyopathy and CAD were significantly higher than controls, the values overlapped. The greatest clinical utilization was found for the amyloidosis group.

Abstract Background Duchenne muscular dystrophy (DMD) associated cardiomyopathy is a major cause of morbidity and mortality. In an in vitro DMD cardiomyocyte model, nicorandil reversed stress-induced cell injury through multiple pathways implicated in DMD. We aimed to test the efficacy of nicorandil on the progression of cardiomyopathy in mdx mice following a 10-day treatment protocol. Methods A subset of mdx mice was subjected to low-dose isoproterenol injections over 5 days to induce a cardiac phenotype and treated with vehicle or nicorandil for 10 days. Baseline and day 10 echocardiograms were obtained to assess cardiac function. At 10 days, cardiac tissue was harvested for further analysis, which included histologic analysis and assessment of oxidative stress. Paired student’s t test was used for in group comparison, and ANOVA was used for multiple group comparisons. Results Compared to vehicle treated mice, isoproterenol decreased ejection fraction and fractional shortening on echocardiogram. Nicorandil prevented isoproterenol induced cardiac dysfunction. Isoproterenol increased cardiac fibrosis, which nicorandil prevented. Isoproterenol increased gene expression of NADPH oxidase, which decreased to baseline with nicorandil treatment. Superoxide dismutase 2 protein expression increased in those treated with nicorandil, and xanthine oxidase activity decreased in mice treated with nicorandil during isoproterenol stress compared to all other groups. Conclusions In conclusion, nicorandil is cardioprotective in mdx mice and warrants continued investigation as a therapy for DMD associated cardiomyopathy.

Abstract Background Chagas disease is a pathogenic parasitic infection with approximately 8 million cases worldwide and greater than 300,000 cases in the United States (U.S.). Chagas disease can lead to chronic cardiomyopathy and cardiac complications, with variable cardiac presentations in pediatrics making it difficult to recognize. The purpose of our study is to better understand current knowledge and experience with Chagas related heart disease among pediatric cardiologists in the U.S. Methods We prospectively disseminated a 19-question survey to pediatric cardiologists via 3 pediatric cardiology listservs. The survey included questions about demographics, Chagas disease presentation and experience. Results Of 139 responses, 119 cardiologists treat pediatric patients in the U.S. and were included. Most providers (87%) had not seen a case of Chagas disease in their practice; however, 72% also had never tested for it. The majority of knowledge-based questions about Chagas disease cardiac presentations were answered incorrectly, and 85% of providers expressed discomfort with recognizing cardiac presentations in children. Most respondents selected that they would not include Chagas disease on their differential diagnosis for presentations such as conduction anomalies, myocarditis and/or apical aneurysms, but would be more likely to include it if found in a Latin American immigrant. Of respondents, 87% agreed that they would be likely to attend a Chagas disease-related lecture. Conclusions Pediatric cardiologists in the U.S. have seen very few cases of Chagas disease, albeit most have not sent testing or included it in their differential diagnosis. Most individuals agreed that education on Chagas disease would be worth-while.

Background: The role of the dysfunction of left atrium in the occurrence and development of cardiovascular disease has been gradually recognized. We aim to compare the impact on left atrial (LA) function between patients with hypertrophic cardiomyopathy (HCM) and hypertension (HTN) without LA enlargement using cardiovascular magnetic resonance feature tracking (CMR-FT), and if possible, explore the capability of LA function for providing clinical implication and predicting clinical adverse events in the early stage of cardiovascular disease.Methods: Consecutive 60 HCM patients and 60 HTN patients with normal LA size among 1413 patients who underwent CMR were retrospectively analyzed as well as 60 controls. Left atrial and ventricular functions were quantified by volumetric and CMR-FT derived strain analysis from long and short left ventricular view cines. The primary endpoint was a composite of all-cause death, stroke, new-onset or worsening heart failure to hospitalization, and paroxysmal or persistent atrial fibrillation.Results: Compared to the controls, both HTN and HCM participants had impaired LA reservoir function (εs) and conduit function (εe) with the different stage of LA booster pump dysfunction (εa). LA strain was more sensitive than LV longitudinal strain (GLS) for evaluate primary endpoint (εs: 33.9% ± 7.5 vs. 41.2% ± 14.3, p = 0.02; εe: 13.6% ± 6.2 vs. 17.4% ± 10.4, p = 0.03; εa: 20.2% ± 6.0 vs. 23.7% ± 8.8, p = 0.07; GLS: -19.4% ± 6.4 vs. -20.0% ± 6.8, p = 0.70, respectively). After a mean follow-up of 6.8 years, 23 patients reached primary endpoint. Cox regression analyses indicated impaired LA reservoir and booster pump strain were associated with clinical outcomes in patients at the early stage of HTN and HCM (p < 0.05).Conclusions: CMR-FT-derived strain is a potential and robust tool in demonstrating impaired LA mechanics, quantifying LA dynamics and underlining the impacts on LA-LV coupling in patients with HTN and HCM without LA enlargement. The corresponding LA dysfunction is a promising metric to assess clinical implication and predict prognosis at the early stage, superior to GLS. [ABSTRACT FROM AUTHOR]

Abstract Background Necrotizing autoimmune myopathy (NAM) is pathologically characterized by myofiber necrosis and regeneration with paucity or absence of inflammatory cells in muscle biopsy. Two autoantibodies, namely anti-signal recognition particle (SRP)-antibodies and anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR)-antibodies, are typically specific with NAM. Anti-SRP-positive NAM can be associated with cardiomyopathy which responds well to immunotherapy. Here we reported an anti-SRP-antibody and anti-MDA5-antibody NAM patient who developed severe cardiomyopathy after gaining significant improvement of myopathy and subsequently accepted heart transplantation. Case presentation A NAM case with both positive anti-SRP and MDA-5 antibodies who gained significant improvement of the skeletal muscle weakness with immunotherapy, but 3 years later he developed severe dilated cardiomyopathy and at last received heart transplantation. Myocardial biopsy showed disarranged and atrophic myofibers, remarkable interstitial fibrosis without inflammatory infiltrates. Immunohistochemistry analysis revealed increased polyubiquitin-binding protein p62/SQSTM1 protein expression and the positive staining of cleaved-caspase 3 in a few cardiomyocytes. After the transplantation, the patient was symptom-free on oral prednisone (10 mg/day) and tacrolimus (2 mg/day). Conclusions We described the first case of anti-SRP and anti-MAD5 positive NAM who had received heart transplantation because of cardiopathy. Though the myopathy had been clinically improved after immunotherapy, the cardiomyopathy remained progressive and lethal. The processes of dysfunctional autophagy and augmented apoptosis were putatively pathophysiological mechanisms underlying cardiac damage in anti-SRP and anti-MAD5 positive NAM.

Background: Self-reported health status as measured by the Kansas City Cardiomyopathy Questionnaire (KCCQ) in patients with primary prevention implantable cardioverter defibrillators (ICDs) has mainly been reported from randomized trials. However, these studies are often limited to short follow-up and are subject to selection bias. The aim of this study was to assess KCCQ-12 in patients with primary prevention ICD due to either ischemic or nonischemic heart failure.Methods: This cross-sectional observational study included all patients in Region Gävleborg, Sweden, who because of primary prevention due to heart failure, had an ICD or underwent device replacement between 2007 and 2017. After validation using medical records patients were sent and returned the KCCQ-12 by regular mail.Results: A total of 118 questionnaires were analyzed (response rate 71.1%). The mean age was 70.9 ± 9.8 years, and a minority was female (n = 20, 16.9%). The mean overall summary score was 71.5 ± 22.4, there was no significant difference between ischemic and nonischemic heart failure (69.5 ± 23.1 vs. 74.4 ± 21.3; p = 0.195). Atrial fibrillation at baseline was associated with lower score for the domains Symptom frequency (70.2 ± 23.2 vs. 82.2 ± 19.2; p = 0.006) and Social limitation (62.1 ± 26.0 vs. 75.6 ± 26.6; p = 0.006) as well as the overall summary score (63.9 ± 21.3 vs. 74.8 ± 22.2; p = 0.004).Conclusion: In a real-world setting, primary prevention ICD patients with heart failure report an acceptable disease-specific health status at long-term follow-up. Ischemic and nonischemic etiology showed similar health status whereas atrial fibrillation was associated with worse outcome. [ABSTRACT FROM AUTHOR]

Background: Duchenne muscular dystrophy (DMD) associated cardiomyopathy is a major cause of morbidity and mortality. In an in vitro DMD cardiomyocyte model, nicorandil reversed stress-induced cell injury through multiple pathways implicated in DMD. We aimed to test the efficacy of nicorandil on the progression of cardiomyopathy in mdx mice following a 10-day treatment protocol.Methods: A subset of mdx mice was subjected to low-dose isoproterenol injections over 5 days to induce a cardiac phenotype and treated with vehicle or nicorandil for 10 days. Baseline and day 10 echocardiograms were obtained to assess cardiac function. At 10 days, cardiac tissue was harvested for further analysis, which included histologic analysis and assessment of oxidative stress. Paired student's t test was used for in group comparison, and ANOVA was used for multiple group comparisons.Results: Compared to vehicle treated mice, isoproterenol decreased ejection fraction and fractional shortening on echocardiogram. Nicorandil prevented isoproterenol induced cardiac dysfunction. Isoproterenol increased cardiac fibrosis, which nicorandil prevented. Isoproterenol increased gene expression of NADPH oxidase, which decreased to baseline with nicorandil treatment. Superoxide dismutase 2 protein expression increased in those treated with nicorandil, and xanthine oxidase activity decreased in mice treated with nicorandil during isoproterenol stress compared to all other groups.Conclusions: In conclusion, nicorandil is cardioprotective in mdx mice and warrants continued investigation as a therapy for DMD associated cardiomyopathy. [ABSTRACT FROM AUTHOR]

Background: This study aimed to determine native T1 and extracellular volume fraction (ECV) in distinct types of myocardial disease, including amyloidosis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), myocarditis and coronary artery disease (CAD), compared to controls.Methods: We retrospectively enrolled patients with distinct types of myocardial disease, CAD patients, and control group (no known heart disease and negative CMR study) who underwent 3.0 Tesla CMR with routine T1 mapping. The region of interest (ROI) was drawn in the myocardium of the mid left ventricular (LV) short axis slice and at the interventricular septum of mid LV slice. ECV was calculated by actual hematocrit (Hct) and synthetic Hct. T1 mapping and ECV was compared between myocardial disease and controls, and between CAD and controls. Diagnostic yield and cut-off values were assessed.Results: A total of 1188 patients were enrolled. The average T1 values in the control group were 1304 ± 42 ms at septum, and 1294 ± 37 ms at mid LV slice. The average T1 values in patients with myocardial disease and CAD were significantly higher than in controls (1441 ± 72, 1349 ± 59, 1345 ± 59, 1355 ± 56, and 1328 ± 54 ms for septum of amyloidosis, DCM, HCM, myocarditis, and CAD). Native T1 of the mid LV level and ECV at septum and mid LV with actual and synthetic Hct of patients with myocardial disease or CAD were significantly higher than in controls.Conclusions: Although native T1 and ECV of patients with cardiomyopathy and CAD were significantly higher than controls, the values overlapped. The greatest clinical utilization was found for the amyloidosis group. [ABSTRACT FROM AUTHOR]

Background: Chagas disease is a pathogenic parasitic infection with approximately 8 million cases worldwide and greater than 300,000 cases in the United States (U.S.). Chagas disease can lead to chronic cardiomyopathy and cardiac complications, with variable cardiac presentations in pediatrics making it difficult to recognize. The purpose of our study is to better understand current knowledge and experience with Chagas related heart disease among pediatric cardiologists in the U.S.Methods: We prospectively disseminated a 19-question survey to pediatric cardiologists via 3 pediatric cardiology listservs. The survey included questions about demographics, Chagas disease presentation and experience.Results: Of 139 responses, 119 cardiologists treat pediatric patients in the U.S. and were included. Most providers (87%) had not seen a case of Chagas disease in their practice; however, 72% also had never tested for it. The majority of knowledge-based questions about Chagas disease cardiac presentations were answered incorrectly, and 85% of providers expressed discomfort with recognizing cardiac presentations in children. Most respondents selected that they would not include Chagas disease on their differential diagnosis for presentations such as conduction anomalies, myocarditis and/or apical aneurysms, but would be more likely to include it if found in a Latin American immigrant. Of respondents, 87% agreed that they would be likely to attend a Chagas disease-related lecture.Conclusions: Pediatric cardiologists in the U.S. have seen very few cases of Chagas disease, albeit most have not sent testing or included it in their differential diagnosis. Most individuals agreed that education on Chagas disease would be worth-while. [ABSTRACT FROM AUTHOR]

Background: Necrotizing autoimmune myopathy (NAM) is pathologically characterized by myofiber necrosis and regeneration with paucity or absence of inflammatory cells in muscle biopsy. Two autoantibodies, namely anti-signal recognition particle (SRP)-antibodies and anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR)-antibodies, are typically specific with NAM. Anti-SRP-positive NAM can be associated with cardiomyopathy which responds well to immunotherapy. Here we reported an anti-SRP-antibody and anti-MDA5-antibody NAM patient who developed severe cardiomyopathy after gaining significant improvement of myopathy and subsequently accepted heart transplantation.Case Presentation: A NAM case with both positive anti-SRP and MDA-5 antibodies who gained significant improvement of the skeletal muscle weakness with immunotherapy, but 3 years later he developed severe dilated cardiomyopathy and at last received heart transplantation. Myocardial biopsy showed disarranged and atrophic myofibers, remarkable interstitial fibrosis without inflammatory infiltrates. Immunohistochemistry analysis revealed increased polyubiquitin-binding protein p62/SQSTM1 protein expression and the positive staining of cleaved-caspase 3 in a few cardiomyocytes. After the transplantation, the patient was symptom-free on oral prednisone (10 mg/day) and tacrolimus (2 mg/day).Conclusions: We described the first case of anti-SRP and anti-MAD5 positive NAM who had received heart transplantation because of cardiopathy. Though the myopathy had been clinically improved after immunotherapy, the cardiomyopathy remained progressive and lethal. The processes of dysfunctional autophagy and augmented apoptosis were putatively pathophysiological mechanisms underlying cardiac damage in anti-SRP and anti-MAD5 positive NAM. [ABSTRACT FROM AUTHOR]

Abstract Background In 1951 Churg and Strauss first described the clinical condition now known as eosinophilic granulomatosis with polyangiitis (EGPA), characterized by asthma, nasal polyposis, rhinosinusitis, hypereosinophilia with organ infiltration, and necrotizing vasculitis. It is classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, but ANCA negativity is common and more frequently encountered in EGPA with myocardial involvement. Long-term survival has substantially improved with corticosteroid treatment but myocardial involvement is still the leading cause of death in EGPA. Case presentation A 53-year old man with a history of asthma and nasal polyposis presented with acute chest pain and elevated troponin; a percutaneous coronary intervention was performed. The left ventricle was described as hypertrophic. After 20 days the myocardium had markedly increased in thickness of both the right and left ventricle. Evaluation revealed hypereosinophilia in the blood and nasal mucosal tissue, which confirmed the diagnosis of EGPA. He presented with signs of active vasculitis including weight loss, tiredness, intracerebral hemorrhage, and increasing serum creatinine. After 6 days of corticosteroid treatment, the myocardium returned to its initial thickness. Conclusion Rapid and marked thickening of the myocardium is not frequently reported but may occur in EGPA. Myocardial thickening in EGPA can be quickly reversed by corticosteroids, and is most likely caused by edema.

Abstract Background Non-compaction cardiomyopathy (NCCM) is characterized by prominent trabeculations, deep intertrabecular recesses, and a thick non-compacted endocardial myocardium. Prevalence in the general population remains unclear, but echocardiography series report 0.05%. During fetal development muscle fibers and trabeculae should compact into a solid myocardium and when this fails, NCCM occurs. The condition is genetic, even though acquired forms have been described. Worsening myocardial dysfunction may lead to heart failure and/or arrhythmias. Case presentation A 52-year-old man presented with heart failure. The diagnosis of NCCM was confirmed after echocardiography and cardiac magnetic resonance tomography. Interestingly, the angiogram revealed a coronary anomaly, in which the circumflex artery rose aberrantly from the right coronary artery. Due to left ventricular ejection fraction being less than 35% despite optimal pharmacological therapy, an implantable cardioverter defibrillator (ICD) was implanted and four years later a ventricular tachycardia was terminated by antitachycardia pacing. Conclusion We describe a case of NCCM with a concomitant coronary anomaly, in which systolic myocardial dysfunction developed. The ICD subsequently terminated a life-threatening ventricular arrhythmia, which supports risk stratification based on low ejection fraction and possibly coronary anomaly.

Background: In 1951 Churg and Strauss first described the clinical condition now known as eosinophilic granulomatosis with polyangiitis (EGPA), characterized by asthma, nasal polyposis, rhinosinusitis, hypereosinophilia with organ infiltration, and necrotizing vasculitis. It is classified as an antineutrophil cytoplasmic antibody (ANCA) associated vasculitis, but ANCA negativity is common and more frequently encountered in EGPA with myocardial involvement. Long-term survival has substantially improved with corticosteroid treatment but myocardial involvement is still the leading cause of death in EGPA.Case Presentation: A 53-year old man with a history of asthma and nasal polyposis presented with acute chest pain and elevated troponin; a percutaneous coronary intervention was performed. The left ventricle was described as hypertrophic. After 20 days the myocardium had markedly increased in thickness of both the right and left ventricle. Evaluation revealed hypereosinophilia in the blood and nasal mucosal tissue, which confirmed the diagnosis of EGPA. He presented with signs of active vasculitis including weight loss, tiredness, intracerebral hemorrhage, and increasing serum creatinine. After 6 days of corticosteroid treatment, the myocardium returned to its initial thickness.Conclusion: Rapid and marked thickening of the myocardium is not frequently reported but may occur in EGPA. Myocardial thickening in EGPA can be quickly reversed by corticosteroids, and is most likely caused by edema. [ABSTRACT FROM AUTHOR]

Background: Non-compaction cardiomyopathy (NCCM) is characterized by prominent trabeculations, deep intertrabecular recesses, and a thick non-compacted endocardial myocardium. Prevalence in the general population remains unclear, but echocardiography series report 0.05%. During fetal development muscle fibers and trabeculae should compact into a solid myocardium and when this fails, NCCM occurs. The condition is genetic, even though acquired forms have been described. Worsening myocardial dysfunction may lead to heart failure and/or arrhythmias.Case Presentation: A 52-year-old man presented with heart failure. The diagnosis of NCCM was confirmed after echocardiography and cardiac magnetic resonance tomography. Interestingly, the angiogram revealed a coronary anomaly, in which the circumflex artery rose aberrantly from the right coronary artery. Due to left ventricular ejection fraction being less than 35% despite optimal pharmacological therapy, an implantable cardioverter defibrillator (ICD) was implanted and four years later a ventricular tachycardia was terminated by antitachycardia pacing.Conclusion: We describe a case of NCCM with a concomitant coronary anomaly, in which systolic myocardial dysfunction developed. The ICD subsequently terminated a life-threatening ventricular arrhythmia, which supports risk stratification based on low ejection fraction and possibly coronary anomaly. [ABSTRACT FROM AUTHOR]

Background: Identifying which patients with diastolic dysfunction will progress to heart failure with preserved ejection fraction (HFpEF) remains challenging. The goal of this study is to determine whether increased vascular stiffness as identified on 2D transthoracic echocardiography (TTE) serves as a biomarker for the development of HFpEF in patients with diastolic dysfunction.Methods: The study design is a matched retrospective case-control study. Subjects with diastolic dysfunction were divided into two groups based on whether they had a clinical diagnosis of HFpEF. The two groups were matched based on age, gender, race and body surface area, resulting in 77 matched pairs (n = 154). Data from the first TTE that documented diastolic dysfunction prior to the development of HFpEF was extracted along with baseline demographic and clinical data. Indices of vascular stiffness were measured and compared. A sub-group analysis was performed to compare diabetic subjects in Group 1 (n = 43) to those in Group 2 (n = 21).Results: Group 1 had significantly decreased aortic distensibility as measured on the initial TTE when compared to Group 2 (1.9 ± 1.0 vs. 2.8 ± 1.8 cm2dyne-110-3, p = 0.01). In the diabetic subset, Group 1 had significantly less aortic strain (6.9 ± 3.3 vs. 9.7 ± 5.6%, p = 0.02) and aortic distensibility (1.8 ± 1.0 vs. 3.5 ± 2.6 cm2dyne-110-3, p = 0.02) compared to Group 2. Other indices of vascular stiffness did not differ significantly between groups.Conclusions: This study demonstrates that increased proximal aortic stiffness is associated with the development of HFpEF in patients with asymptomatic diastolic dysfunction. Larger prospective studies are needed to further investigate this relationship. [ABSTRACT FROM AUTHOR]

Background: Anthracyclines are widely used in the treatment of childhood cancer. One of the well-recognized side-effects of anthracycline therapy is dose-dependent cardiomyopathy that may progress to heart failure (HF) years after completion of cancer-directed therapy. This study will evaluate the efficacy of low-dose beta-blocker (carvedilol) for HF risk reduction in childhood cancer survivors at highest risk for HF. The proposed intervention has the potential to significantly reduce chronic cardiac injury via interruption of neurohormonal systems responsible for left ventricular (LV) remodeling, resulting in improved cardiac function and decreased risk of HF. The intervention is informed by previous studies demonstrating efficacy in pediatric and adult non-oncology populations, yet remains unstudied in the pediatric oncology population. Methods/Design: The primary objective of the trial is to determine impact of the intervention on echocardiographic markers of cardiac remodeling and HF risk, including: LV wall thickness/ dimension ratio (LVWT/D; primary endpoint), as well as LV ejection fraction, volume, and blood biomarkers (natriuretic peptides, galectin-3) associated with HF risk. Secondary objectives are to establish safety and tolerability of the 2-year course of carvedilol using: 1) objective measures: hepatic and cardiovascular toxicity, treatment adherence, and 2) subjective measures: participant self-reported outcomes. Two hundred and fifty survivors of childhood cancer (diagnosed <21 years of age), and previously treated with high-dose (≥300 mg/m2) anthracyclines will be enrolled in a randomized, doubleblind, placebo controlled trial. After baseline assessments, participants will be randomized in a 1:1 ratio to low-dose carvedilol (maximum dose: 12.5 mg/day) or placebo. Carvedilol or placebo is up-titrated (starting dose: 3. 125 mg/day) according to tolerability. Discussion: When completed, this study will provide much-needed information regarding a physiologically plausible pharmacological risk-reduction strategy for childhood cancer survivors at high risk for developing anthracycline-related HF. [ABSTRACT FROM AUTHOR]

Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is considered a progressive cardiomyopathy. However, data on the clinical features of disease progression are limited. The aim of this study was to assess 12-lead surface electrocardiographic (ECG) changes during long-term follow-up, and to compare these findings with echocardiographic data in our large cohort of patients with ARVC/D. Methods: Baseline and follow-up ECGs of 111 patients from three tertiary care centers in Switzerland were systematically analyzed with digital calipers by two blinded observers, and correlated with findings from transthoracic echocardiography. Results: The median follow-up was 4 years (IQR 1.9-9.2 years). ECG progression was significant for epsilon waves (baseline 14% vs. follow-up 31%, p = 0.01) and QRS duration (111 ms vs. 114 ms, p = 0.04). Six patients with repolarization abnormalities according to the 2010 Task Force Criteria at baseline did not display these criteria at follow-up, whereas in all patients with epsilon waves at baseline these depolarization abnormalities also remained at follow-up. T wave inversions in inferior leads were common (36% of patients at baseline), and were significantly associated with major repolarization abnormalities (p = 0.02), extensive echocardiographic right ventricular involvement (p = 0.04), T wave inversions in lateral precordial leads (p = 0.05), and definite ARVC/D (p = 0.05). Conclusions: Our data supports the concept that ARVC/D is generally progressive, which can be detected by 12-lead surface ECG. Repolarization abnormalities may disappear during the course of the disease. Furthermore, the presence of T wave inversions in inferior leads is common in ARVC/D. [ABSTRACT FROM AUTHOR]

Background: Noncompaction cardiomyopathy (NCC) is a rare genetic cardiomyopathy characterized by a thin, compacted epicardial layer and an extensive noncompacted endocardial layer. The clinical manifestations of this disease include ventricular arrhythmia, heart failure, and systemic thromboembolism. Case presentation: A 43-year-old male was anticoagulated by pulmonary thromboembolism for 1 year when he developed progressive dyspnea. Cardiovascular magnetic resonance imaging showed severe biventricular trabeculation with an ejection fraction of 15%, ratio of maximum noncompacted/compacted diastolic myocardial thickness of 3.2 and the presence of exuberant biventricular apical thrombus. Conclusion: Still under discussion is the issue of which patients and when they should be anticoagulated. It is generally recommended to those presenting ventricular systolic dysfunction, antecedent of systemic embolism, presence of cardiac thrombus and atrial fibrillation. In clinical practice the patients with NCC and ventricular dysfunction have been given oral anticoagulation, although there are no clinical trials showing the real safety and benefit of this treatment. [ABSTRACT FROM AUTHOR]

Background: Noncompaction cardiomyopathy (NCC) is a rare genetic cardiomyopathy characterized by a thin, compacted epicardial layer and an extensive noncompacted endocardial layer. The clinical manifestations of this disease include ventricular arrhythmia, heart failure, and systemic thromboembolism. Case presentation: A 43-year-old male was anticoagulated by pulmonary thromboembolism for 1 year when he developed progressive dyspnea. Cardiovascular magnetic resonance imaging showed severe biventricular trabeculation with an ejection fraction of 15%, ratio of maximum noncompacted/compacted diastolic myocardial thickness of 3.2 and the presence of exuberant biventricular apical thrombus. Conclusion: Still under discussion is the issue of which patients and when they should be anticoagulated. It is generally recommended to those presenting ventricular systolic dysfunction, antecedent of systemic embolism, presence of cardiac thrombus and atrial fibrillation. In clinical practice the patients with NCC and ventricular dysfunction have been given oral anticoagulation, although there are no clinical trials showing the real safety and benefit of this treatment. [ABSTRACT FROM AUTHOR]