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Cardiomyopathies - The special entity of myocarditis and inflammatory cardiomyopathy

Published on: 1st July, 2019

OCLC Number/Unique Identifier: 8192807959

Cardiomyopathy is a heart muscle disease with structural and functional myocardial abnormalities in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease. However, it has become clear that diverse etiologies and clinical manifestations (e.g. arrhythmogenic right-ventricular cardiomyopathy/dysplasia (ARVC/D), ARVD/C, left-ventricular non-compaction cardiomyopathy (LVNC)) are responsible for the clinical picture of dilated cardiomyopathy (DCM). The American Heart Association (AHA) classification grouped cardiomyopathies into genetic, mixed and acquired forms, while the European Society of Cardiology (ESC) classification proposed the subgrouping of each major type of cardiomyopathy into familial or genetic, and nonfamilial or nongenetic, forms [1-4]. Cardiomyopathies are clinically heterogeneous diseases, and there are differences in sex, age of onset, rate of progression, risk of development of overt heart failure and likelihood of sudden death within each cardiomyopathy subtype [5]. Because of the complex etiology and clinical presentation, the diagnostic spectrum in cardiomyopathies spans the entire range of non-invasive and invasive cardiological examination techniques including genetic analysis. The exact verification of certain cardiomyopathies necessitates additional investigations. So, histological, immunohistological and molecular biological/virological investigations of endomyocardial biopsies are the gold standard to confirm the diagnosis of an inflammatory cardiomyopathy (DCMi) [6-10]. This review focuses on myocarditis and inflammatory cardiomyopathies underlying an immune-mediated process or persistent viral infection.
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Single Trans apical access for double aortic and mitral valves-in-valves procedures with high risk of thrombus embolism

Published on: 16th July, 2019

OCLC Number/Unique Identifier: 8192819595

Background: Persistent left atrial thrombus remains a contra indication to transeptal valves in valves procedure. We aimed to perform a double valves in valves replacement through transapical access with cerebral angiography control during the procedure just after implantation. Our case shows the feasibility of this strategy and the management of right ventricle laceration successfully treated after extra corporeal membrane oxygenation implantation and local hemostasis. We reported a feasibility case report of successful double valves in valves implantation through transapical access with 6 months of clinical and computed tomography follow up.
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Assessment of lipid and hematological profile among blood donors in European Gaza Hospital, Palestine

Published on: 16th July, 2019

OCLC Number/Unique Identifier: 8192805778

Background: There is an important shortage of blood in the greatest blood banks worldwide to meet up with requirements for numerous medical interventions. Limited studies have associated regular blood donation to the lowering of lipid function parameters. Assessing the lipid function is a classical method of evaluating an individual’s risk for coronary heart disease. Objective: The general goal of the study is to determine lipid and hematological profile among blood donors in European Gaza Hospital, Palestine. Materials and Methods: This study was a case-control study that involved 120 male, 40 of whom were regular blood donors (study group), 40 first time donors and 40 non- donors (control group) aged between 18-60 years. A volume of 5ml venous blood was drawn from each fasting participant into a dry biochemistry screw-capped tube. This was allowed to clot and the serum was used to determine total cholesterol (TC), triglycerides, High-density lipoprotein cholesterol (HDL-C), Low-density lipoprotein cholesterol (HDL-C), while HDL-C/LDL-C and TC/LDL ratio were calculated by using the following formula. Anthropometric parameters (weight, height) of donors were measured using standard protocol. The height (in meter), weight (in kilogram) were used to calculate the body mass index (BMI) using the following formula. BMI= weight (kg)/ (height in meter)² and blood was collected from each participant in EDTA (for hematocrit, ESR). Three groups were matched for age and BMI. Data were analyzed using SPSS version 23. Chi-square (χ²) was used to compare the relationship between categorical variables, ANOVA was used to measure the difference between means. Data were summarized using tables, pie charts, histograms. A P-value < 0.05 was considered to be statistically significant for all tests conducted. Results: The mean total cholesterol (169±10.85 mg/dl), triglycerides (116±9.73 mg/dl), HDL (54±2.5 mg/dl ), LDL (92±11.4mg/dl), LDL/HDL ratio (1.73±0.25) and TC/HDL ratio (3.16±0.26) were lower in the regular blood donors than the first time donors(198±10.13, 179±5.82, 42.33±1.6, 120±11.2, 2.85±0.36, 4.7±0.40) and non- donors (202±10.19, 180±12.68, 41.75±1.4, 125±11.7, 2.99±0.33, 4.86±0.32) respectively and statistically significant (P < 0.05).The mean ESR (6.63±0.87mm/hr) was lower statistically significant in the regular blood donors than the first time donors (7.40±1.17) and non- donors (7.60±1.48) respectively (P < 0.05). The mean HCT (42.98±0.86%) was lower statistically significant in the regular blood donors than the first time donors (44.63±0.90) and non- donors (44.75±0.74, P < 0.05). Conclusion: Regular donors have reduced risk of developing coronary heart disease as reflected by the low total cholesterol, triglycerides, LDL-c, LDL-c/HDL-c ratio, TC/HDL-c ratio and HCT and high HDL. BMI in regular donor was less than the donor for the first time and did not donate, but did not reach the statistical significance. Also in our study regular donors have reduced risk of developing inflammation as reflected by low ESR.
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Severe Infantile Coarctation and Mid Aortic Stenosis in Williams Syndrome

Published on: 30th July, 2019

OCLC Number/Unique Identifier: 8192818729

We report a challenging patient with Williams syndrome and severe coarctation of the aorta. As in a few similar cases reported, several surgical and catheter interventions for recoarctation, intrastent intimal proliferation and stenosis of the left sub-clavian artery were required. Aortic patch angioplasty is planned for the future in a grower child.
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Preclinical stiff heart is a marker of cardiovascular morbimortality in apparently healthy population

Published on: 31st July, 2019

OCLC Number/Unique Identifier: 8192795566

Background: The prognostic significance of impaired left ventricular (LV) relaxation and increased LV stiffness as precursor of heart failure with preserved ejection fraction and death is still largely unknown in apparently healthy subjects. Methods: We constituted a cohort of 353 patients with normal ejection fraction (>45%) and no significant heart disease, based on a total of 3,575 consecutive left-sided heart catheterizations performed. We measured peak negative first derivative of LV pressure (-dP/dt) and operating chamber stiffness (Κ) using a validated equation. Patients were categorized as having: 1) normal diastolic function, 2) isolated relaxation abnormalities (-dP/dt > 1860mm Hg/sec and K <0.025mm Hg/ml), or 3) predominant stiff heart (K ≥0.025mm Hg/ml). Results: During a follow-up of at least 5 years, the incidence of the primary composite endpoint (death, major arterial event, heart failure, and arrhythmia) was 23.2% (82 patients). Compared to isolated relaxation abnormalities, predominant stiff heart showed stronger prognostic significance for all events (p=0.002), namely heart failure (HR, 2.9; p=0.0499), cardiac death (HR, 5.8; p=0.03), and heart failure and cardiac death combined (HR, 3.7; p=0.003). Conclusion: In this apparently healthy population referred to our center for cardiac catheterization, the prevalence of diastolic dysfunction was very high. Moreover, predominant stiff heart was a better predictor of cardiovascular outcomes than isolated relaxation abnormalities.
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Timing of cardiac surgery and other intervention among children with congenital heart disease: A review article

Published on: 9th August, 2019

OCLC Number/Unique Identifier: 8212836826

Background: Early diagnosis and improved facilities are necessary for determining the optimal timing of surgery and other interventions in children with congenital heart diseases in Nigeria. This is because late presentation, late diagnosis and delayed surgery can lead to mortality and affect the quality of life among these children. Objectives: This review article is aimed at enumerating the timing of cardiac surgeries and other interventions and to seek if there is any factor associated with the timing of cardiac surgery. Methods: A search on PubMed database, World Health Organization libraries, Google scholar, TRIP database, and reference lists of selected articles on timing of cardiac surgery in children was done. The Cochrane Database of Systematic Reviews was also searched. We noted few data from African setting. Key words such as timing of cardiac surgery; children, congenital heart defect were used. Conclusion: Appropriate timing for cardiac surgery in children with congenital heart disease is very important as late surgical intervention could result in several morbidities and mortality.
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Late discover of a traumatic cardiac injury: Case report

Published on: 19th August, 2019

OCLC Number/Unique Identifier: 8212828090

Blunt chest trauma leads to a wide range of lesions, relatively minor parietal injuries to potentially fatal cardiac lesions, making diagnosis and management difficult. The diagnosis is currently facilitated by imaging, however, these lesions may go unnoticed and be discovered late through complications. We report the case of a neglected heart wound revealed by a heart failure. This case is notable due to a favourable outcome despite a delay in diagnosis due to a lack of pericardial effusion and the absence of cardiac symptoms, and a long delay from injury to appropriate treatment in the presence of a penetrating cardiac wound deep enough to cause a muscular ventricular septal defect and lacerate the anterior mitral leaflet.
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Role of novel cardiac biomarkers for the diagnosis, risk stratification, and prognostication among patients with heart failure

Published on: 22nd August, 2019

OCLC Number/Unique Identifier: 8212771729

Background: Current guidelines for diagnosis and management of heart failure (HF) rely on clinical findings and natriuretic peptide values, but evidence suggests that recently identified cardiac biomarkers may aid in early detection of HF and improve risk stratification. The aim of this study was to assess the diagnostic and prognostic utility of multiple biomarkers in patients with HF and left ventricular systolic dysfunction (LVSD). Methods: High-sensitivity cardiac troponin I (cTnI), N-terminal pro b-type natriuretic peptide (NT-proBNP), interleukin-6 (IL-6), endothelin-1 (ET-1), pro-matrix metalloproteinase-9 (pMMP-9), and tumor necrosis factor-alpha (TNF-α) were measured using single-molecule counting technology in 200 patients with varying stages of HF. Plasma detection with cross-sectional associations of biomarkers across all HF stages, and advanced-therapy and transplant-free survival were assessed using multivariate analysis and Cox regression analyses, respectively. Results: NTproBNP, pMMP-9, IL-6 were elevated in early, asymptomatic stages of HF, and increased with HF severity. Higher circulating levels of combined IL-6, NTproBNP, and cTnI predicted significantly worse survival at 1500-day follow-up. Cox regression analysis adjusted for ACC/AHA HF stages demonstrated that a higher concentration of IL-6 and cTnI conferred greater risks in terms of time to death, implantation of left ventricular assist device (LVAD), or heart transplantation. Conclusion: Biomarkers of inflammation, LV remodeling, and myocardial injury were elevated in HF and increased with HF severity. Patients had a significantly higher risk of serious cardiac events if multiple biomarkers were elevated. These findings support measuring NTproBNP, cTnI and IL-6 among patients with HF and LVSD for diagnostic and prognostic purposes.
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How often is Klippel-Feil Syndrome associated with congential heart disease presentation of five cases and a review of the literature

Published on: 3rd September, 2019

OCLC Number/Unique Identifier: 8270717914

Introduction: Klippel-Feil syndrome (KFS), is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. In some individuals, KFS can be associated with a variety of additional symptoms and physical abnormalities which contribute in the deterioration and complication of the condition of the child. Aim of presentation: Here, we report five children from Kosovo with KFS associated with different heart abnormalities, clinical presentation, diagnosis, management, and outcomes of selected conditions in resources-limited settings. Methods: Retrospectively we analysed medical reports of five children, diagnosed at different age with congenital disease and clinical and lab signs of Klippel-Feil syndrome. Conclusion: Basing on our cases, all diagnosed in a small country as a Kosovo, we can conclude that KFS is not such a rare condition. In addition, such syndrome is not so rarely associated with different congenital heart disease. In four cases cardiac surgery was indicated and successfully was done abroad Kosovo in the lack of such services in Kosovo.
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A mouse model of coronary microvacsular disease using a photochemical approach

Published on: 18th September, 2019

OCLC Number/Unique Identifier: 8270725834

The development of reproducible rodent models of coronary microvascular disease (MVD) is essential for the early detection, treatment, and mechanism study of the pathophysiology. We hypothesized that endothelial dysfunction and subsequent microthrombi in the coronary arterioles, two early events in clinical coronary MVD, could be reproduced by photochemical reaction (PCR) technology in mice hearts. After rose bengal (one of photosensitizers) was administrated systemically, a green light was locally used to activate the photosensitizer, inducing over-production of oxidative stress in the heart. Following PCR, animals demonstrated reproducible endothelial injury, occlusion in arterioles, focal ischemia, and infarct-let with preserved cardiac function. Our technique has proven to be a reliable and reproducible means of creating coronary MVD in mice. We believe that this is an ideal model for developing a novel molecular tracer for earlier detection of coronary MVD, for testing new anti-fibrinolytic drugs, and for investigating the complex pathophysiology of coronary MVD. The protocol for establishing this model takes about thirty to forty minutes.
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Arrhythmia of the heart - computer analysis

Published on: 20th September, 2019

The problem of synchronization of oscillations of various physical nature is discussed. From the standpoint of the theory of synchronism, a model of the heart is considered as a system of four connected between self-oscillating links: two atria and two ventricles. The synchronous and asynchronous operating modes are considered at sinusoidal and relaxation oscillations. A computer program has been compiled that simulates the fluctuations in the heart using four differential equations. Four examples of calculation according to the program are given for asynchronous and synchronous operation modes. The possibility of evaluating the ablation procedure from the perspective of a computer model is discussed.
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Novel paclitaxel-coated balloon angioplasty via single retrograde popliteal access for challenging superficial femoral artery and iliac artery lesions?

Published on: 24th September, 2019

OCLC Number/Unique Identifier: 8261371866

Objectives: We report our results regarding the use of BioPath™ paclitaxel-coated balloon catheters for superficial or distal external iliac artery revascularization via single retrograde popliteal access. Methods: We included 105 prospective consecutive patients. Single retrograde popliteal access was achieved under ultrasound guidance with the patients laid prone. An over-the-wire atherectomy system was used if risk of distal embolization was high due to plaque intensity of the target lesion. A 4 to 7 mm-diameter BioPath™ 035 balloon catheter was used for all lesions. Follow-up at 6th month included doppler ultrasound examination for patency. Results: Seventy-two patients (68.6%) had total SFA occlusion and 41 patients (39%) had concomitant external iliac artery involvement, out of whom 31 (29.5%) had total occlusion. Procedural success 90.5% for superficial femoral artery and 85.3% for external iliac artery. One-year patency rates in SFA and EIA were 84.8% and 80.4%, respectively Conclusion: Single retrograde popliteal access and drug-coated balloon angioplasty may offer a useful alternative to known modalities in treatment of challenging superficial femoral artery and concomitant iliac artery lesions
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Plaque morphology in diabetic vs. non diabetic patients assessed by Multi-Slice Computed Tomography coronary angiography

Published on: 4th October, 2019

OCLC Number/Unique Identifier: 8333008298

Background and Objectives: Multi-slice computed tomography (MSCT) provides high accuracy for noninvasive assessment of coronary artery disease (CAD). The introduction of the latest computed tomography technology allows comprehensive evaluation of various aspects of CAD, including the coronary calcium score, coronary artery stenosis, bypass patency, and myocardial function. This study aimed to assess the effect of DM on coronary arteries evaluated by MSCT-CA Comparing Plaque Morphology in Diabetic patients with Non-Diabetic Whoever Controlled or not assessed by HbA1c. Methods: In this study we randomly assigned 150 adult patients were diagnosed with suspected coronary artery disease underwent MSCT-CA for evaluation their coronaries regarding luminal stenosis, Plaque analysis, Remodeling index, SSS, SIS and Ca score. Results: There was statistically significant difference between diabetics & non-diabetic groups in LM lesions with (P = 0.029). also, the results of multivariate logistic regression analysis after adjustment for age and sex, diabetics were shown a trend toward more mixed plaque with statistically significant {(OR): 3.422, 95% CI 1.66-7.023, P = 0.001}; whereas, after adjustment for age, sex, history of hypertension, smoking, and hypercholesterolemia, patients with diabetes also shown a trend toward more mixed plaque with statistically significant (OR: 3.456, 95% CI 1.668-7.160, P = 0.001). It means significant differences in coronary atherosclerotic plaque burden and composition between diabetic and non-diabetic patients, with a higher proportion of mixed plaques, a more vulnerable form of atherosclerotic plaque in diabetics (P < 0.001) otherwise No significant difference. Conclusion: MSCT angiography may be useful for the identification of CAD in diabetic and non-diabetic patients. There were statistically significant differences in coronary atherosclerotic plaque burden and composition, with a higher proportion of mixed plaques, between diabetic and nondiabetic patients. Furthermore, MSCT may give accurate information about plaque characteristics according to different coronary risk factors, thereby identifying high risk features warranting a more intensive anti-atherosclerotic treatment.   
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Scintigraphic non-invasive diagnosis of amyloid cardiomyopathy

Published on: 4th October, 2019

OCLC Number/Unique Identifier: 8457482817

Amyloidosis encompasses a heterogeneous group of disorders, characterized by extracellular deposition of insoluble abnormal amyloid aggregates, due to a failure in protein quality control. Cardiac amyloidosis is a disorder in which proteins misfold and deposit as amyloid fibrils that infiltrate the myocardial extracellular space [1]. Transthyretin (ATTR) and light chain (AL) are the most frequent types of cardiac amyloidosis. Transthyretin is a protein mainly synthesized by the liver, it may be hereditary or acquired from either wild-type (ATTRwt) or mutant (ATTRm) amyloid [2]. Cardiomyopathy is a common manifestation of ATTR amyloidosis with a particularly poor life expectancy of 2 to 6 years after diagnosis [3]. Although considered rare, the prevalence of this serious disease is likely underestimated because symptoms can be non-specific, and diagnosis largely relies on amyloid detection in tissue biopsies.
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The effect of anemia on serum hepcidin levels in patients with heart failure

Published on: 17th October, 2019

OCLC Number/Unique Identifier: 8303641326

Background: Anemia is an accelerating problem among patients with heart failure (HF) and its presence is associated with more symptoms. In this study, we investigated whether anemia in heart failure was related to hepcidin concentration. Methods: 50 patients with heart failure and 20 healthy subjects with no history of a chronic illness including heart failure as control group, were included in the study. Heart failure was verified by echocardiography in each subject and patients were defined as ones with reduced ejection fraction (HFrEF) if EF ≤ 40% and with preserved ejection fraction (HFpEF) if EF 40% - 50%. Blood samples were taken from all patients after 10-12 hours fasting. Anemia assessment was performed according to World Health Organization (WHO) criterias. Results: There was a positive correlation between hepcidin concentration and urea, ferritin, hemoglobin, hematocrite, C-reactive protein (p < 0,05). Hepcidin concentrations of anemic heart failure patients were significantly lower than the non-anemic heart failure patients (p < 0,05). Conclusion: We found that serum hepcidin concentration in anemic patients with heart failure was lower than in heart failure patients without anemia. We believe that iron defiency occurs as a result of inflammatory process in heart failure and therefore hepcidin concentrations decrease as a response. However, long-term follow up studies are needed.
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Impact of Pacemaker Implantation on 12-Month Resource Utilization Following TAVR Hospitalization

Published on: 21st October, 2019

OCLC Number/Unique Identifier: 8286124047

Purpose: This study reports resource utilization during a Medicare Beneficiary’s (MBs) Transcatheter Aortic Valve Replacement (TAVR) index hospitalization and all subsequent encounters for 12 months and compares data between MBs who did or did not receive a pacemaker implantation (PPM) during their index hospitalization. Method: This retrospective study examined Medicare hospital claims from January 1, 2014 through June 30, 2015. 15,533 MBs who survived for 365 days were studied. Information from all encounters during the study period was combined to compare hospital resource utilization and outcomes. Results: 14.8% of MBs had a PPM during the index hospitalization. 46.0% of MBs had at least one readmission to a hospital during the 365-day follow-up period. 54.6% of MB’s first hospital readmission occurred within 90 days of their TAVR discharge date. Average total Medicare reimbursement for all hospitalizations was $60,638 ± $28,974 associated with average total hospital length of stay of 11.2 ± 11.7 days. After adjusting for demographics and 47 comorbid conditions, MBs receiving a PPM during the index TAVR had significantly higher estimated Medicare reimbursement ($5,132) and longer total length of stay (1.8 days) for the entire study period than MBs not receiving a PPM. Conclusion: Total Medicare reimbursement and hospital LOS were significantly higher among MBs that had a PPM implantation during their index admission; however, there were no significant differences in readmission rates, readmission length of stay, or days to first readmission during the follow-up period between the two study cohorts.
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Evaluation of the predictive value of CHA2DS2-VASc Score for no-reflow phenomenon in patients with ST-segment elevation myocardial infarction who underwent Primary Percutaneous Coronary Intervention

Published on: 28th October, 2019

OCLC Number/Unique Identifier: 8303626780

Objective: The aim of this study was to estimate the predictive clinical value of CHA2DS2-VASc score for no-reflow phenomena in patients having ST-segment elevation myocardial infarction (STEMI) who applied to primary percutaneous coronary intervention (PCI). Subjects and Methods: Three-hundred STEMI patients underwent primary PCI. They were classified into: group (1) included 27 patients with no-reflow and group (2) included 273 patients without no-reflow (control). CHA2DS2-VASc risk score was computed for each patient. Results: This study found statistically significant difference (p < 0.05) in multivariate analysis of the association between CHA2DS2-VASc score and no-reflow phenomenon. The predictive power of individual components in CHA2DS2-VASc score for no-reflow was statistically significant difference (p < 0.05). So, significantly higher CHA2DS2-VASc score is connected with higher risk of no-reflow and in-hospital mortality rate. Conclusion: Significantly higher CHA2DS2-VASc score is associated with higher risk of no- reflow phenomenon and in-hospital mortality rates in patients with STEMI who underwent primary PCI.
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Do beta adrenoceptor blocking agents provide the same degree of clinically convincing morbidity and mortality benefits in patients with chronic heart failure? A literature review

Published on: 4th November, 2019

OCLC Number/Unique Identifier: 8304637476

Chronic heart failure has been extensively characterized as a disorder arising from a complex interaction between impaired ventricular performance and neurohormonal activation. Since beta adrenoceptor blocking agents are currently considered an integral component of therapy for the management of patients with severe chronic heart failure; several well designed clinical trials have been conducted to determine the morbidity and mortality benefits of these agents these studies, however did not yield the same results in terms of morbidity and mortality benefits. Currently only Bisoprolol, Carvedilol and sustained release metoprolol succinate have clinically proven and convincing morbidity and mortality benefits the current list of approved medicines of the National Health Insurance Scheme (NHIS) of the republic of Ghana does not provide coverage for these lifesaving therapeutic agents. The objective of this review was to collate the relevant scientific evidence that will convince the authorities at the National Health Insurance Authority (NHIA) of the Republic of Ghana to include at least one of the evidence based beta adrenoceptor blocking agents in the list of approved medicines. A thorough search on the internet was conducted using Google scholar to obtain only the clinically relevant studies associated with the benefits of beta adrenoceptor blocking agents in patients with chronic heart failure published in the English language. The phrases beta adrenoceptor blocking agents and chronic heart failure were used as search engines. The search engine yielded several studies that met the predefined inclusion criteria. However, only the Cardiac Insufficiency BIsoprolol Studies (CIBIS-I and CIBIS-II), Carvedilol Prospective Randomized Cumulative Survival Study (COPERNICUS) and Metoprolol CR/XL Randomized Intervention Trial (MERIF-HF) because of the clinical relevance of their findings Beta adrenoceptor blocking agents such as atenolol and propranolol have been used in the management of patients with chronic heart failure. However, their efficacy and optimal dose in reducing mortality have not been scientifically established not all beta adrenoceptor blocking agents scientifically studied provide the same degree of clinically meaningful and convincing morbidity and mortality benefits in patients with chronic heart failure.
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A Systematic review for sudden cardiac death in hypertrophic cardiomyopathy patients with Myocardial Fibrosis: A CMR LGE Study

Published on: 4th November, 2019

OCLC Number/Unique Identifier: 1185948222

Background: Hypertrophic cardiomyopathy (HCM) patients have a predisposition for malignant VT/VF and consequently, sudden cardiac death (SCD). In single center studies, late gadolinium enhancement (LGE) defined fibrosis has been linked to VT/VF. However, despite innumerable investigations, SCD has not been definitely attributable to LGE. Explanations for these are believed to be related to insufficient statistical power. Methods: We performed an electronic search of MEDLINE, PubMed: and CMR abstracts for original data published or presented between Jan 2001 to Mar 2011. Key search terms: HCM, LV fibrosis, SCD and LGE. Studies were screened for eligibility based on inclusion criteria: referral for CMR exam with LGE for HCM; and follow-up for incidence of VT/VF and SCD. Categorical variables were evaluated between patient groups via Chi-square test. Results: A total of 64 studies were initially identified. Of these, 4 (6.3%) were identified and included (n = 1063 patients). Three prospective and one retrospective study were included. LGE was detected in 59.6% of patients. As expected, the presence of myocardial fibrosis was associated with VT/VF (x2 = 6.5, p < 0.05; OR 9.0, (95% CI 1.2 to 68.7). Moreover, myocardial fibrosis strongly predicted SCD (x2 = 6.6, p < 0.05; OR 3.3 (95% CI 1.2 to 9.7). Conclusion: Despite single center CMR studies, LGE has consistently predicted VT/VF while prediction of SCD has remained paradoxically unlinked. Although the lack of studies meeting our criteria limited our ability to perform a comprehensive meta-analysis, we have been able to demonstrate for the first time that LGE-defined fibrosis is a predictor of SCD in patients with HCM0.
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Pulse Synchronized Contractions (PSCs)

Published on: 15th November, 2019

OCLC Number/Unique Identifier: 8319360286

A key platform underpinning the traditional understanding of the cardiovascular system, with respect to the behavior of large arterial vessels, is Otto Frank’s Windkessel Hypothesis [1]. This hypothesis posits simply that the smooth muscle walls of large arteries do not undergo rhythmic contractions in synchrony with the heartbeat but, rather, behave as passive elastic tubes undergoing distension from pulsatile pressure waves. The Windkessel Hypothesis is elegant, well described for over a century, ingrained in the understanding of cardiovascular medicine and physiology, and simply wrong. Several groups have now shown that the arterial smooth muscle wall undergoes rhythmic activation in synchrony with the heartbeat in a variety of tissues, including human brachial artery; canine coronary, femoral, and carotid arteries; rabbit aorta; feline pulmonary artery and rodent aorta [2-8]. The phasing of these events is such that the upstroke of the contraction slightly precedes the upstroke of the pulse wave, suggesting nomenclature for the events as pulse synchronized contractions, or PSCs [3,6-8]. PSCs have been found to be of neurogenic origin, sensitive to the neural blocker tetrodotoxin [3,8]. Although the specific neural pathways regulating PSCs have not been elucidated, the alpha-adrenergic system is at least partially involved, as evidenced by reduction or blockade of PSCs by the alpha-adrenergic blocker phentolamine [8]. Further, PSCs have not been observed following vessel excision in in vitro studies, as an intact nervous system is not present. The pacemaker for the PSC resides in the right atrium, as suggested by two lines of evidence. First, pacing of the right atrial region to faster than spontaneous frequencies leads to a one-to-one correspondence of PSC frequency with the stimulation rate [3]. Additionally, excision of the right, but not the left, atrial appendage results in elimination of PSCs [3]. As the pacemaker region for PSCs and the heartbeat both lie in the right atrium, this may potentially allow for coordination between the heartbeat and pulse wave with PSCs [3,5,8]. Extensive evaluations also have been performed showing the PSC was not an artifact produced either by cardiac contractility or from the vessel distension from the pulse wave [3,5,6].
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