Valvular Damage Research Articles

Prevalence of extravalvular mitral damage in patients with rheumatic etiology mitral stenosis

Abstract Background Rheumatic fever is the leading cause of mitral stenosis (MS) worldwide. Clinically significant stenosis if accompanied by symptoms, atrial fibrillation, or pulmonary hypertension merits therapeutic interventions. Extra valvular damage has been shown to be of prognostic value in patients with mitral regurgitation and aortic stenosis. The prevalence and significance of extra valvular damage has not been studied in patients with rheumatic mitral stenosis. Objective To describe the prevalence of extra valvular damage in patients with rheumatic mitral stenosis and its relationship with major adverse cardiovascular events. Methods We prospectively enrolled patients 18 years and older with rheumatic mitral stenosis and MVA measured by planimetry less than 2 cm². Patients with more than mild mitral regurgitation and aortic stenosis or regurgitation were excluded. Patients were divided in four groups depending on the degree of extra valvular damage. Group 1: mitral stenosis without extra valvular damage, group 2: mitral stenosis with left atrial dilation (left atrial volume >34 ml/m2BSA) or atrial fibrillation, group 3: mitral stenosis with pulmonary vascular involvement (tricuspid regurgitation velocity >2.8 m/s), group 4: mitral stenosis with right ventricular systolic dysfunction (TAPSE <17). Major adverse cardiovascular events included cardiovascular death, all-cause mortality, de novo atrial fibrillation, acute myocardial infarction, stroke, as well as hospitalizations for heart failure. Results We included 92 patients age 62 ± 11, 78 female (84%). A high burden of cardiovascular risk factors was found, obesity 60 (66%), atrial fibrillation 68 (63%), hypertension 33 (30%) and type 2 diabetes 24 (26%) with no significant difference between groups (Table 1). Main echocardiographic parameters were as follows: left ventricular fraction of 56% (25-73), left atrial volume 91 ml (32-364), TAPSE 18 mm ± 4.6, systolic pulmonary artery pressure 42 mmHg ± 18, mitral valve area 0.97cm (0.3-1.7). Importantly none of the patients was classified in group 1 (without extra valvular damage). Mitral valve area was lower in patients of group 4 compared to patients with group 2 and 3. Although not statistically significant (p=0.37) a higher numerical number of events occurred in patients with higher burden of extra valvular damage: 18 (50%) in group 4, 16 (47%) and, in group 3, 7 (31%) (Table 2). Conclusions Extra valvular damage in patients with rheumatic MS is highly prevalent. None of the patients was classified in group 1, highlighting the high burden of extra valvular damage in patients with mitral stenosis. As the severity of mitral stenosis progress extra valvular damage increases. A higher number of events occurs in patients with increasing extra valvular damage. The study was limited by a small number of patients. Larger studies are needed to establish the prognostic value of extra valvular damage in rheumatic mitral stenosis. Table 1. Table 2.

Inhibition of Endothelial-Mesenchymal Transition Mediated by Activin Receptor Type IIA Attenuates Valvular Injury Induced by Group A Streptococcus in Lewis Rats.

Rheumatic heart disease (RHD), which is caused mainly by Group A Streptococcus, leads to fibrotic damage to heart valves. Recently, endothelial‒mesenchymal transition (EndMT), in which activin plays an important role, has been shown to be an important factor in RHD valvular injury. However, the mechanism of activin activity and EndMT in RHD valvular injury is not clear. Our study was divided into two parts: in vivo and in vitro. We constructed a small interfering RNA (ACVR2A-siRNA) by silencing activin receptor type IIA (ACVR2A) and an adeno-associated virus (AAV-ACVR2A) containing a sequence that silenced ACVR2A. The EndMT cell model was established via human umbilical vein endothelial cells (HUVECs), and the RHD animal model was established via female Lewis rats. ACVR2A-siRNA and AAV-ACVR2A were used in the above experiments. EndMT occurred in the valvular tissues of RHD rats, and activin and its associated intranuclear transcription factors were also activated during this process, with inflammatory infiltration and fibrotic damage also occurring in the valvular tissues. After inhibition of ACVR2A, EndMT in valvular tissues was also inhibited, and inflammatory infiltration and fibrosis were reduced. Endothelial cell experiments suggested that mesenchymal transition could be stimulated by activin and that inhibition of ACVR2A attenuated mesenchymal transition. Activin plays an important role in signal transduction during EndMT after activation, and inhibition of ACVR2A may attenuate RHD valvular damage by mediating EndMT. Targeting ACVR2A may be a therapeutic strategy to alleviate RHD valvular injury.

Streptococcus suis infective endocarditis in patients with Streptococcus suis bacteremia: a retrospective study of prevalence and outcomes

BackgroundStreptococcus suis (S. suis) is a zoonotic disease that is transmitted to humans via contact or oral route. Although the major clinical presentation of this pathogen is known to be meningitis, S. suis infective endocarditis (IE) has recently emerged as a clinical manifestation of increasing interest. Echocardiography may be an underutilized modality for evaluating patients with S. suis bacteremia.ObjectiveThe primary objective was to estimate the prevalence of S. suis IE in patients with S. suis bacteremia. The secondary objective was to investigate the predictors, echocardiographic features, and clinical outcomes of S. suis IE in patients with S. suis bacteremia.Materials and methodsThis single-center retrospective study was conducted at Siriraj Hospital – Thailand’s largest university-based tertiary referral center. Adult patients (aged > 18 years) who were admitted to our center with confirmed diagnosis of S. suis bacteremia during January 2007 to September 2023 were included. Prevalence is reported as percentage and confidence interval. Baseline characteristics and clinical manifestation were compared between the IE and non-IE groups. Factors found to be statistically significant were further analyzed using binary logistic regression analysis to identify univariate predictors of S. suis IE.ResultA total of 71 patients with S. suis bacteremia were included in this study. The prevalence of S. suis IE was 26.8% (95% confidence interval: 17.85–38.05). Perivalvular complications and significant valvular regurgitation were found in 52.6% and 80.0% of patients, respectively. Thirteen of 19 patients (68.4%) required valvular surgery according to standard guidelines. By univariate analysis, dyspnea, new murmur, immunologic phenomenon, and heart failure were predictors of S. suis IE in patients with S. suis bacteremia.ConclusionThe results of this study revealed a sizable prevalence of S. suis IE in patients with S. suis bacteremia, and there were high rates of both valvular damage and perivalvular complications. Our results strongly suggest that echocardiography may be indicated to evaluate for S. suis IE in patients diagnosed with S. suis bacteremia. Reclassification of S. suis from an atypical organism to a typical organism should be considered.

Streptococcus equi Subspecies zooepidemicus Endocarditis and Meningitis in a 62-Year-Old Horse Rider Patient: A Case Report and Literature Review.

The present article presents a case report and literature review concerning the Streptococcus equi subspecies zooepidemicus (SEZ), a rare zoonotic pathogen in humans. The case involves a 62-year-old man with no prior heart disease, presenting with endocarditis, pneumonia, and meningitis following close contact with a horse. The patient underwent urgent aortic valve replacement due to severe valvular damage caused by the infection. Blood and cerebrospinal fluid cultures confirmed the presence of SEZ, and the patient was treated with a combination of antibiotics, followed by a successful step-down to oral therapy using linezolid. A review of 25 additional Streptococcus equi endocarditis cases highlights the rarity of the condition, its association with animal contact, and its tendency to cause multi-site infections, such as pneumonia and meningitis. Early diagnosis, appropriate antibiotic therapy, and, in severe cases, surgical intervention are critical for a favorable outcome. This report emphasizes the importance of recognizing zoonotic infections in at-risk populations and the potential need for public health surveillance in these scenarios.

ASSESSMENT OF KNOWLEDGE, ATTITUDES AND PRACTICES ON RHEUMATIC HEART DISEASE AMONG CARDIAC PATIENTS IN AMRAN CITY, YEMEN

Background: All three layers of the heart—the pericardium, myocardium, and endocardium (including the valves)—may sustain irreversible damage as a result of rheumatic heart disease (RHD). Rheumatic heart disease is the result of valvular damage caused by an abnormal immune response to group a streptococcal infection, usually during childhood. The study aims to achieve the assessment of Knowledge, Attitudes and Practices (KAP) on rheumatic heart disease among cardiac patients in Amran city, Yemen. Method: The descriptive analytical method was used. The questionnaire was used as a tool for collecting data the research sample was (121) of those accompanying rheumatic heart patients in clinics and medical centers in the city of Amran, and they were selected in a simple random method. The data was analyzed and processed statistically using the Statistical Package for the Social Sciences program (SPSS) and many statistical methods, and after conducting the data analysis process and testing the study hypotheses. Result: The study found that heart patients in Amran have knowledge about rheumatic heart disease causes and symptoms, but lack knowledge about risk factors and disease history. Most suffer from arthritis and do not practice regular exercise. There were no significant differences in opinions about rheumatic heart disease and risk factors among heart patients due to variables like gender, age, education, and living conditions. Rural residents showed statistically significant differences. Conclusions: The study reveals that patients with rheumatic heart disease have the highest knowledge about the causes and symptoms, but also the risk factors associated with the disease. The knowledge of the disease's history is the most common, followed by rheumatic fever. Arthritis is the most common risk factor. The study found no significant differences in opinions among heart patients in Amran, but significant differences were observed in rural residents' opinions due to factors like gender, age, and living conditions. Peer Review History: Received 13 May 2024; Reviewed 14 July 2024; Accepted 22 August; Available online 15 September 2024 Academic Editor: Dr. Sally A. El-Zahaby, Pharos University in Alexandria, Egypt, sally.elzahaby@yahoo.com Average Peer review marks at initial stage: 6.0/10 Average Peer review marks at publication stage: 7.0/10 Reviewers: Dina Abd Elfattah Eldakhs, Pharos university (PUA), Egypt, dina_eldakhs@yahoo.com Prof. Hassan A.H. Al-Shamahy, Sana'a University, Yemen, shmahe@yemen.net.ye

Infective Endocarditis in Patients Receiving Hemodialysis: A Current Review.

Cardiovascular and infective complications are commonly observed in patients receiving hemodialysis (HD) with cardiovascular events and infection-related complications being the first and second leading causes of death. Infective endocarditis (IE) is characterized by inflammation of the endocardium caused by infection, typically affecting the cardiac valves and can be in acute, subacute, or chronic forms. It is a serious complication within the HD population due to their predisposition for both infection and valvular damage. Considering the frailty and burden of comorbidities in those receiving HD, management of IE in the HD population is very challenging. There has been continuous discussion and debate on optimizing the diagnostic and treatment approach of IE in this patient group to improve their clinical outcomes. Currently, reported outcomes are relatively poor and there are updates from numerous guidelines relating to advances in IE management. In this review, we will evaluate the evidence in relation to the epidemiology of HD-associated IE and discuss the important risk factors of IE in patients requiring dialysis. We will also evaluate the current recommendations regarding diagnosis and treatment for suspected or confirmed IE cases amongst HD patients and present the updated data regarding clinical outcomes relating to HD-associated IE. The incidence of IE in HD patients is expected to increase going forward as HD becomes more easily accessible alongside an emerging uptake of home HD. A more thorough insight into this topic is required to improve clinical practice relating to IE prevention and management in the HD population, given relatively poor clinical outcomes.

Vicious Circle With Venous Hypertension, Irregular Flow, Pathological Venous Wall Remodeling, and Valve Destruction in Chronic Venous Disease: A Review.

Substantial advances occurred in phlebological practice in the last two decades. With the use of modern diagnostic equipment, the patients' venous hemodynamics can be examined in detail in everyday practice. Application of venous segments for arterial bypasses motivated studies on the effect of hemodynamic load on the venous wall. New animal models have been developed to study hemodynamic effects on the venous system. In vivo and in vitro studies revealed cellular phase transitions of venous endothelial, smooth muscle, and fibroblastic cells and changes in connective tissue composition, under hemodynamic load and at different locations of the chronically diseased venous system. This review is an attempt to integrate our knowledge from epidemiology, paleoanthropology and anthropology, clinical and experimental hemodynamic studies, histology, cell physiology, cell pathology, and molecular biology on the complex pathomechanism of this frequent disease. Our conclusion is that the disease is initiated by limited genetic adaptation of mankind not to bipedalism but to bipedalism in the unmoving standing or sitting position. In the course of the disease several pathologic vicious circles emerge, sustained venous hypertension inducing cellular phase transitions, chronic wall inflammation, apoptosis of cells, pathologic dilation, and valvular damage which, in turn, further aggravate the venous hypertension.

SEVERE MITRAL INSUFFICIENCY FROM RHEUMATIC DISEASE: A CASE REPORT

Abstract Acute rheumatic fever continues to be a major health problem in many parts of thw world. It’s more common in low–income or developing parts of the world. Antibiotics for bacterial infections may not be available in these areas. In developed countries, cardiac involvement is quite rare. Although traditionally considered to be a disease associated with poverty, rheumatic fever continues to persist even among the middle–class population in developed countries. Children and teenagers with intreated strep infections are the most likely to get rheumatic fever. Signs of heart damage can develop years after the infection. Rheumatic heart disease is the result of valvular damage caused by an abnormal immune response to Streptococcus pyogenes infection. After multiple episodes of rheumatic fever, progressive fibrosis of the heart valves can occur: mitral regurgitation is the most common valvular lesion. We report the case of a 13–year–old, Italian, middle–class girl with rheumatic mitral valve involvement. A 13–years–old comes to the clinic for onset of worsening dyspnea ad easy fatigability. History turns out to be negative for cardiovascular disease and history of recent infections. Echocardiographic examination showed a dilated left ventricle (VTD 148ml, 98 m/mq) with preserved chinese. Severe dilatation of the left atrium with atrio–aortic ratio 2.3 and atrial volume 91 ml/mq. The mitral valve presented a myxomatous, thickened leaflets, predominantly the posterior which appeared retracted and hypomobile with thickening of the subvalvular apparatus with coaptation deficit along the entire coaptation rhyme realizing severe degree insufficiency (CV 7mm, ERO 0.8 cm2, CO 39 l/min). A small cleft at the level of A1, main lesion jet (A1–P3–P2) was also visible. The Echocardiographic appearance suggested outcomes from rheumatic disease so antistreptolysin titer and anti–DNase B antibodies were assayed with high levels found. Penicillin prophylaxis was therefore initiated. For the continuation of the therapeutic course, the case was discussed collegially and a valve replacement rather tan repair was advocated because the valvular and subvalvular apparatus were far too damaged.

Mitral Regurgitation in Unpublished Mitral Lesions in Cutis Laxa: Case Report and Review of the Literature

Congenital mitral regurgitation (BMI), a rare disease, is defined as any left ventriculo auricular regurgitation due to dysplasia of the congenital mitral valve. It is accompanied by some degree of relative stenosis, and frequently associated with other malformations. Curtis Laxa is a skin disease, either acquired or hereditary, with autosomal and sexual transmission. Redundant skin is often most visible on the neck, hands and groin, but can also be seen on the face, creating an appearance of premature aging. It is associated with severe systemic involvement in some forms. We report a case of severe mitral regurgitation associated with valvular damage in a patient being treated for hereditary Cutis Laxa with other associated systemic damage.

Application of Transthoracic Echocardiography for Cardiac Safety Evaluation in the Clinical Development Process of Vaccines Against Streptococcus pyogenes.

Superficial infections with Streptococcus pyogenes (Strep A), pharyngitis and impetigo can induce acute rheumatic fever, an autoimmune sequela manifesting mostly with arthritis and rheumatic carditis. Valvular heart damage can persist or advance following repeated episodes of acute rheumatic fever, causing rheumatic heart disease. Acute rheumatic fever and rheumatic heart disease disproportionately affect children and young adults in developing countries and disadvantaged communities in developed countries. People living with rheumatic heart disease are at risk of experiencing potentially fatal complications such as heart failure, bacterial endocarditis or stroke. Transthoracic echocardiography plays a central role in diagnosing both rheumatic carditis and rheumatic heart disease. Despite the obvious medical need, no licensed Strep A vaccines are currently available, as their clinical development process faces several challenges, including concerns for cardiac safety. However, the development of Strep A vaccines has been recently relaunched by many vaccine developers. In this context, a reliable and consistent safety evaluation of Strep A vaccine candidates, including the use of transthoracic echocardiography for detecting cardiac adverse events, could greatly contribute to developing a safe and efficacious product in the near future. Here, we propose a framework for the consistent use of transthoracic echocardiography to proactively detect cardiac safety events in clinical trials of Strep A vaccine candidates.

Risk Factors for Acute Rheumatic Disease: Exploring Factors at Individual and Collective Levels.

Acute rheumatic fever (ARF) is a complex disease with several clinical manifestations. Its most significant long-term complication is valvular heart damage, commonly referred to as chronic rheumatic heart disease. The risk of ARF varies globally, with over 80% of cases occurring in low- and middle-income countries, highlighting the role of socioeconomic factors. A comprehensive understanding of the risk factors associated with ARF and its clinical, genetic, and sociodemographic mediators can help clinicians identify high-risk individuals, develop effective management strategies, enhance target screening and active case-finding initiatives, and ultimately improve patient outcomes. This review aimed to provide an overview of ARF and its global burden, focusing on the established and potential risk factors associated with its development.

Penetrating chest injury.

A 33-year-old man presented to the emergency department with a 24-hour history of chest pain and dyspnea after an accidental penetrating chest trauma while working in construction. He lived in a rural area. He had no known medical history. His heart rate was 113 beats per minute, blood pressure 106/64 mmHg, respiratory rate 24 breaths per minute, and oxygen saturation was 89%. On physical examination, he appeared well, had a left parasternal wound without active bleeding, and a decreased breath sound in the left lung field, without jugular ingurgitation. A contrasted computed tomography (CT) was requested (Figures 1 and 2). Traumatic intraventricular foreign body. CT revealed a linear structure with metallic density across the fourth costal arc and his left ventricle, associated with pericardial effusion and left hemothorax. An emergency sternotomy was performed, and findings confirmed an injury of 1 cm diameter by a screw on the left ventricle, without coronary injuries; additionally, he required left thoracostomy, and pericardial and mediastinal drainage. Echocardiography 12 hours later informed a normal ventricular function without a segmentary defect, pericardial effusion, or valvular damage. Clinicians should have a high index of suspicion of a life-threatening condition, even if the injury is due to a low-energy trauma. At 4 days follow-up hospitalization without complications, the patient was discharged in good condition. Penetrating chest trauma is a surgical emergency, with a high risk of complications and death.1 Low energy injuries occurring due to non-explosive objects are a rare event.2 Multidetector CT may be essential to confirm the diagnosis and specify the location and additional findings that allow defining the most appropriate treatment.3 The authors received no financial support for the research, authorship, or publication of this article.

Abstract ED13-1: Beyond anthracyclines and trastuzumab: Cardiotoxicity of novel agents

Abstract Breast cancer treatment can have a diverse array of delayed and long-term effects, including cardiovascular events such as heart failure, valvular damage, coronary artery disease, arrythmias, and cardiac dysfunction. The adverse cardiovascular effects of anthracyclines and trastuzumab in the treatment of early and advanced breast cancer have been well elucidated withing the literature; however, there is less understanding of the potential cardiovascular impact of more recent novel breast cancer therapies. Dual HER2 targeted therapy (pertuzumab/trastuzumab) is now standard of care for neoadjuvant and adjuvant treatment, particularly in the setting of lymph node involvement, but is there a higher risk of cardiotoxicity? There is a limited understanding of the potential short- and long-term cardiovascular toxicity associated with antibody-drug conjugates, such as T-DM1 and fam-trastuzumab deruxtecan, and well as oral anti-HER2 tyrosine kinase inhibitors such as lapatinib, neratinib, and more recently tucatinib. CDK4/6 inhibitors (eg ribociclib) combined with endocrine therapy have significantly improved clinical outcomes for individuals with hormone receptor positive, HER-2 advanced breast cancer but are associated with an increased risk of QTc prolongation, which can be compounded by electrolyte abnormalities and drug-drug interactions. Immune checkpoint inhibitors (pembrolizumab) are approved in the neoadjuvant (high risk) and advanced setting (PDL-1 positive) for triple negative breast cancer but are associated with several toxicities including endocrinopathies and rarely fatal myocarditis. The goal of this session is to improve the awareness and understanding of the potential cardiovascular toxicities associated with modern anti-cancer therapies used in the treatment of breast cancer. Citation Format: Susan Dent. Beyond anthracyclines and trastuzumab: Cardiotoxicity of novel agents [abstract]. In: Proceedings of the 2022 San Antonio Breast Cancer Symposium; 2022 Dec 6-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2023;83(5 Suppl):Abstract nr ED13-1.

Neutrophilic Leukocytes and Neutrophil Extracellular Traps in Native Aortic Valve Endocarditis

Abstract Infective endocarditis is a disease that affects the endocardium and often alters heart valves, notably the aortic valve. Bacteraemia and valvular endothelial damage play an essential role in the pathogenesis of infective endocarditis. The pertinent literature suggests that neutrophil extracellular traps are important contributors to the development of the disease. However, features of the valvular damage and contribution of neutrophils to the alteration of cardiac tissue are not explored sufficiently. The purpose of this study was to investigate the occurrence and distribution of neutrophilic leukocytes and neutrophil extracellular traps in native aortic valves affected by infective endocarditis, using histopathology and immunohistochemistry assays. In addition, the presence of vegetations on the heart valve was determined. Infiltration of neutrophils into the valvular leaflet was significantly more severe at the free margin (mean 5.89 ± 3.00, p < 0.001) and the middle portion (mean 4.58 ± 3.64, p = 0.032) when compared to the base portion (2.05 ± 1.90). No significant differences in neutrophilic leukocyte infiltrating inflammatory lesions were found between cusp layers. The presence of myeloperoxidase and citrullinated histone expression characteristic of neutrophil extracellular traps was demonstrated by the use of immunohistochemistry in IE-affected valvular leaflets and vegetations. Collectively, the study results suggest that the free cusp margin and its middle portion of the aortic valve are exposed to enforced blood flow; endothelial damage and vegetation formation are likely to occur along with the presence of infective endocarditis-related bacteraemia.

Plausible Role of NLRP3 Inflammasome and Associated Cytokines in Pathogenesis of Rheumatic Heart Disease.

Rheumatic heart disease (RHD) is a post-streptococcal sequela caused by Streptococcus pyogenes. The global burden of disease is high among people with low socio-economic status, with significant cases emerging every year despite global eradication efforts. The current treatment includes antibiotic therapies to target strep throat and rheumatic fever and valve replacement strategies as a corrective measure for chronic RHD patients. Valvular damage and valve calcification are considered to be the end-stage processes of the disease resulting from impairment of the endothelial arrangement due to immune infiltration. This immune infiltration is mediated by a cascade of events involving NLRP3 inflammasome activation. NLRP3 inflammasome is activated by wide range of stimuli including bacterial cell wall components like M proteins and leukocidal toxins like nicotinamide dehydrogenase (NADase) and streptolysin O (SLO) and these play a major role in sustaining the virulence of Streptococcus pyogenes and progression of RHD. In this review, we are discussing NLRP3 inflammasome and its plausible role in the pathogenesis of RHD by exploiting the host-pathogen interaction mainly focusing on the NLRP3 inflammasome-mediated cytokines IL-1β and IL-18. Different therapeutic approaches involving NLRP3 inflammasome inactivation, caspase-1 inhibition, and blockade of IL-1β and IL-18 are discussed in this review and may be promising for treating RHD patients.

352: RARE PRESENTATION OF CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME IN A TWENTY-SEVEN-YEAR-OLD MAN

Introduction: Antiphospholipid syndrome (APS) is an autoimmune condition manifested by arterial and venous thrombosis, fetal loss, cardiac valvular damage, neurological white matter changes and renal dysfunction. APS is uncommon, with an incidence of 1-2/100,000; close to 80% are in women. We present a rare case of newly diagnosed APS in a 27 year old male. Description: A 27-year-old Hispanic male with no past medical history presented with a chief complaint of difficulty walking. Five weeks prior to admission he began to experience dry eyes and blurred vision, subjective fevers, night sweats, unintentional 20lbs weight loss, and progressive joint pain with inability to ambulate. Vital signs in the emergency room pertinent for temperature 38.5 ℃, blood pressure 126/81 mmHg, tachycardia 108 BPM, SpO2 100% on room air. Physical exam demonstrated tenderness to palpation of lower extremities. Laboratory analysis showed 2.2 x 103/uL white blood cells, hemoglobin 11.4 G/DL, and thrombocytopenia 144 x 103 /uL. Liver enzymes were elevated: ALT 58 U/L, AST 59 U/L. Creatinine was normal at 0.81 mg/dL, however the patient was noted to have an elevated erythrocyte sedimentation rate, 95 MM/HR, and C-reactive protein 47.7. Duplex ultrasound of the lower extremities bilaterally depicted diffuse deep venous thrombosis of right deep veins including the proximal femoral, popliteal, mid and distal femoral vein, and left distal femoral and popliteal vein, with mild residual flow. Given the concern for catastrophic antiphospholipid syndrome, the patient was started on full dose anticoagulation with intravenous heparin, high dose intravenous methylprednisolone, and plasmapheresis. Antinuclear antibody and rheumatoid factor were positive, and the anti-DNA DS antibody grossly elevated at 108 IU/mL. Discussion: Catastrophic Antiphospholipid Syndrome (CAPS) is a rare presentation of APS with mortality as high as 37%, requiring critical care monitoring given the high risk of decompensation. It is characterized by multiorgan dysfunction and thrombosis. A high index of clinical suspicion is essential to initiate prompt treatment. Our patient recovered well after plasmapheresis and was discharged home on apixaban.

An observational analysis of trends in rheumatic heart disease incidence and mortality in EU15+ countries over 29 years

Abstract Background Rheumatic heart disease (RHD) is a debilitating sequela of acute rheumatic fever (ARF), caused by Group A streptococcus (GAS) infection. Repeated episodes of ARF results in valvular damage over time. As a preventable disorder which was once common worldwide, RHD has largely been eradicated in affluent nations due to widespread availability of penicillin, improvement in socioeconomic standards and advancements in health and social infrastructures. However, it has been speculated that the global refugee crisis, especially in Europe, might contribute to a resurgence of RHD cases in these regions. Purpose This observational study aimed to analyse trends in RHD incidence and mortality rates in European Union 15+ (EU15+) over a 29-year period. Methods Data was obtained from the Global Burden of Disease database. Age-standardised mortality and incidence rates for RHD were extracted for the EU15+ countries for the years 1990–2019. Trends were subsequently analysed using Joinpoint regression analysis. Results Over the 29-year period, an overall declining trend in RHD incidence and mortality across EU 15+ nations was observed. The United Kingdom demonstrated the largest decrease in RHD incidence amongst females (−54.9%) and Finland amongst males (−55.3%). Both RHD incidence and mortality were higher among females compared to males across EU15+ countries over the observed period. The most recent incidence trends, starting predominantly after 2014, demonstrated a rise in RHD incidence in most countries. For both sexes, increases were seen in Australia, Belgium, Ireland, Italy, Netherlands, Norway, Sweden and USA. For males specifically, increase in RHD incidence was seen in Spain, and Finland, and for females only in Canada and Ireland. The recent increasing RHD incidence rates ranged from +0.4% to + 24.7% for males and +0.6% to +11.4% for females. Conclusion Whilst overall there are decreases in incidence and mortality from RHD, we observe more than half of EU15+ countries have increasing incidence trends in RHD in recent years. This increasing trend primarily started after 2014, overlapping with the start of the European migration crisis. Although speculative, disparities in access to healthcare for migrants, amongst other socioeconomic factors, may be potential causes; subsequently, further efforts by governments and public health officials are required to recognise and control the resurgence of RHD in high income nations. Funding Acknowledgement Type of funding sources: None.

Relationship between left ventriculoarterial coupling and damage stage in severe aortic stenosis

Abstract Background In patients with severe aortic stenosis (AS), an anatomic and functional staging classification has been proposed to assess extent of cardiac chambers damage to improve the decision of aortic valve replacement based only in symptoms or ejection fraction (EF) <50%. This staging relies on the concept that left ventricular (LV) afterload secondary to AS induce LV dysfunction with progression to LV and right ventricular damage. However, afterload has valvular and vascular components, and the latter is not evaluated by the classification of stages. Characteristics of arterial vasculature can impact on LV function through the alteration in LV-VA coupling which is the relationship between effective arterial elastance (Ea) and LV end-systolic elastance (Emax). Objective To evaluate the relationship between LV-VA coupling and the different stages of cardiac damage in severe AS. Methods We studied 205 patients, age average 70±11 years, 117 men, with severe AS (AVA <1 cm2) with Doppler echocardiography. Measurements of cardiac chambers were made according to ASE/EACVI Guidelines. Ea was calculated as end-systolic pressure divided by stroke volume. End-systolic pressure was obtained from tonometry of carotid pulse. Emax was estimated by the method of Senzaki (1) as an index of LV contractility. Ea / Emax ratio was used to assess LV-VA coupling. Patients were divided in five groups according to the classification of staging cardiac damage proposed by Tasted (2): stage 0, no valvular cardiac damage; stage 1, LV damage (EF <60%); stage 2, left atrial or mitral valve damage; stage 3, pulmonary vasculature or tricuspid valve damage and stage 4, right ventricular damage, or subclinical heart failure (LV stroke volume index <30 ml/m2). Results Mean ± standard deviation (see Table 1). No patient was in stage 0. Symptoms was present in 40% of stage 1, 85% of stage 2 and 100% of stage 3 and 4. Ea increases from stage 2 to stage 4 while Emax did not change in those groups but decreased from stage 1 to 2. Consequently, LV-VA coupling progressively decreased from stage 1 to 4. Conclusions Worsening of LV-VA coupling increase with staging cardiac damage. The alteration of LV-VA coupling from stage 2 to 4 is due to an increase in Ea without significant changes in level of contractility (Emax). The progression of cardiac damage appears to be related not only to valve disease but also to the characteristics of arterial vasculature. Funding Acknowledgement Type of funding sources: None.

Characteristics, management, and outcomes of patients with left-sided infective endocarditis complicated by heart failure: a substudy of the ESC-EORP EURO-ENDO (European infective endocarditis) regist

Abstract Background/Introduction Congestive heart failure (CHF) is a strong prognostic factor in infective endocarditis (IE), but data are lacking regarding its current management and outcome in Europe. Purpose To evaluate the current management and survival of patients with left-sided IE complicated by CHF. Methods We used data of the ESC-EORP EURO-ENDO registry, which is a prospective multicentre observational study conducted between January 1, 2016 and March 31, 2018 in 156 centres in 40 countries. The primary endpoints were 30-day and one-year mortality. Results Among the 3116 patients enrolled in this prospective registry, 2449 (mean age: 60 years, 69% male) with left-sided (native or prosthetic) IE were included in this study. Patients with CHF (n=698, 28.5%) were older, with more comorbidity and more severe valvular damage (mitro-aortic involvement, vegetations >10mm and severe regurgitation/new prosthesis dehiscence) than those without CHF (all p≤0.019). Patients with CHF experienced higher 30-day and one-year mortality than those without (20.5% vs. 9.0% and 36.1% vs. 19.3%, respectively) and CHF remained strongly associated with 30-day (OR [95% CI]: 2.37 [1.73–3.24]; p<0.001) and one-year mortality (HR [95% CI]: 1.69 [1.40–2.05]; p<0.001) after adjustment for established outcome predictors, including early surgery, or after propensity matching for age, sex, and comorbidity (n=618 [88.5%] for each group, both p<0.001). Early surgery, performed on 49% of these patients with IE complicated by CHF, remained associated with a substantial reduction in 30-day mortality following multivariable analysis, after adjustment for age, sex, Charlson index, cerebrovascular accident, staphylococcus aureus IE, Streptococcal IE, uncontrolled infection, vegetation size >10mm, severe valvular regurgitation and/or new prosthetic dehiscence, perivalvular complication, and prosthetic IE (OR [95% CI]: 0.22 [0.12–0.38]; p<0.001) and in one-year mortality (HR [95% CI]: 0.29 [0.20–0.41]; p<0.001). Conclusion CHF is common in left-sided IE and is associated with older age, greater comorbidity, more advanced lesions, and markedly higher 30-day and one-year mortality. Early surgery is strongly associated with lower mortality but is performed on only approximately half of patients with CHF, mainly because of a surgical risk considered prohibitive. Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): Abbott Vascular Int. (2011–2014) Amgen Cardiovascular (2009–2018),

Evaluation of HIV-Related Cardiomyopathy in HIV-Positive Patients in Bushehr, Iran.

ObjectivesIn 2020, according to the UNAIDS (Joint United Nations Programme on HIV/AIDS), more than 37 million people lived with human immunodeficiency virus (HIV) infection worldwide. The disease is known to affect several organs, and one of the most affected organs is the heart. Cardiac diseases are highly prevalent among HIV-infected individuals, and recent findings suggest that this could be due to the damage caused by the virus. HIV patients are subject to advanced immunosuppression, which may lead to cardiac muscle damage and, in turn, cardiomyopathy. We aimed to study the incidence of HIV-related cardiomyopathy.MethodsA pilot cross-sectional study was conducted to assess cardiomyopathy among 200 HIV patients who presented to the Heart Center, Bushehr, Iran. Patients’ files were used to determine the demographic data including age, gender, education, marital status, history of illicit drug use, unsafe/unprotected sexual contact, and whether the patient was a prisoner. Several laboratory data were also collected from these files. Physical examination of the cardiovascular system and echocardiography were also included as part of the evaluation.ResultsAlthough at least four out of five patients presented with some kind of cardiac damage, including valvular damage and pericardial effusion, none was diagnosed with cardiomyopathy. Valvular dysfunction was detected in 88.5% of the patients. Diastolic dysfunction was found in 7.7% of them. The mean ejection fraction was found to be 58%. In addition to cardiomyopathy, none of the patients developed systolic dysfunction, wall motion abnormality, intra-cardiac mass, or vegetation.ConclusionsCardiovascular complications are common among HIV-infected patients. Cardiomyopathy was not detected in our patients. In addition, the most common manifestations that were detected among our patients were valvular heart diseases and pericardial effusion.