Valvuler heart disease

Right bundle branch block (RBBB) and resulting RV electromechanical discoordination are thought to play a role in the disease process of subpulmonary right ventricular (RV) dysfunction that frequently occur post-repair tetralogy of Fallot. We sought to describe this disease entity, the role of pulmonary re-valvulation and the potential added value of RV cardiac resynchronization therapy (RV-CRT). Two patients with repaired tetralogy of Fallot, complete right bundle branch block, pulmonary regurgitation and significantly decreased RV function underwent echocardiography, cardiac magnetic resonance, and an invasive study to evaluate the potential for RV-CRT as part of the management strategy...

OBJECTIVE: The purpose of this study is to describe the long-term results of the « réparation à l'étage ventriculaire » (REV) technique for double outlet right ventricle (DORV) and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect (VSD), and PS (n = 116, 73...

Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support...

UNLABELLED: Adult T-cell leukemia/lymphoma (ATLL) is a mature peripheral T-cell neoplasm caused by human T-cell leukemia virus type I (HTLV-1) infection. Besides the oncogenic property, HTLV-1 causes HTLV-1-associated myelopathy/tropical spastic paraparesis and certain inflammatory diseases via a complex host immune response to latent virus infection. Cardiac involvement of ATLL is rare, with the majority of cases being disclosed in postmortem autopsy in patients with advanced subtypes...

We have developed a candidate vaccine to protect against multiple strains of Streptococcus pyogenes infections. The candidate vaccine contains two synthetic peptides derived from S. pyogenes proteins: the M-protein epitope, p*17 and the IL-8 degrading S. pyogenes Cell-Envelope Proteinase (SpyCEP) epitope, K4S2. In this study we utilise a rat autoimmune valvulitis model that displays both the cardiac and neurobehavioural pathology associated with post-streptococcal sequelae, to assess if the vaccine candidate antigens induce autoimmune complications and inflammatory pathology...

Background: Rheumatic heart disease (RHD) is endemic in sub-Saharan Africa where it is the leading cause of cardiovascular mortality in the young. Rheumatic heart disease results from recurrent episodes of acute rheumatic fever (ARF), which are often difficult to diagnose clinically. Acute rheumatic fever may be diagnosed based on the revised Jones Criteria 2015 for the diagnosis of ARF. Histologically, acute rheumatic valvulitis manifests with active inflammation characterized by lymphocytic infiltration, Aschoff bodies, and Anitschkow cells...

Severe sepsis is a dreaded disease with high mortality, especially in the case of delayed detection. Early diagnosis and treatment initiation is critical for patient survival. However, the septic conditions might be masked by other clinical conditions such as stroke, which may result in a serious delay in diagnosis and treatment. We report a case of iliopsoas abscess that initially presented with cerebellar infarction and subarachnoid hemorrhage. Although severe neurological symptoms were prominent, some signs indicating systemic infection, such as "psoas position", prompted us to investigate the existence of systemic infection...

Acute rheumatic fever (ARF) is considered as a disorder of children, and attacks in adults are usually a recurrence of disease acquired in the child's life. Although the incidence of ARF in children has a decreasing trend in developed countries, resurgent and sporadic epidemics still occur in adults. The first attacks of ARF in adult patients without a childhood history can lead to a diagnostic dilemma. A medical record review in adults at least 18 years of age with an arthralgia complaint fulfilling 2015 revised Jones criteria was performed from January 1, 2000 to December 31, 2019...

BACKGROUND: Acute rheumatic fever (ARF) and its post-inflammatory sequel chronic rheumatic heart disease (RHD) are endemic in the Indian setting. Despite the updated Jones criteria, many cases of ARF remain undiagnosed or are missed. AIMS: This study aims to analyze pathological profiles of such cases and their importance in context of clinical presentation and Jones criteria. MATERIALS AND METHODS: A 22-year retrospective observational study of ARF was conducted in the Department of Pathology in a tertiary care institute...

Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve...

Rheumatoid Arthritis associated valvular heart disease (RA-VHD) may occur in patients in varying degrees of severity. Aortic valve involvement leading to severe symptomatic aortic insufficiency is a rare complication of rheumatoid arthritis. This entity has not been well characterized and its clinical predictors are undefined. The pathology of RA-VHD can extend from benign nodular development to acute valvulitis with late-stage leaflet fibrosis and severe valvular regurgitation. In this report, we describe a rare case of acute heart failure (AHF) resulting from severe aortic valve destruction and insufficiency due to persistent chronic inflammation in a patient with long-standing RA...

The pathogenesis of Acute Rheumatic Fever/Rheumatic Heart Disease (ARF/RHD) and associated neurobehavioral complications including Sydenham's chorea (SC) is complex. Disease complications triggered by Group A streptococcal (GAS) infection are confined to human and determining the early events leading to pathology requires a robust animal model that reflects the hallmark features of the disease. However, modeling these conditions in a laboratory animal, of a uniquely human disease is challenging. Animal models including cattle, sheep, pig, dog, cat, guinea pigs rats and mice have been used extensively to dissect molecular mechanisms of the autoimmune inflammatory responses in ARF/RHD...

BACKGROUND: "Sarcoidosis-like" paradoxical reactions to Antitumor necrosis factor α (anti-TNFα) treatment have been reported. The clinical presentations are varied, most of the time, with a relatively typical picture of mediastinopulmonary involvement. More rarely, isolated granulomatous locations from various organs are described, leading to difficulties in diagnosis. CASE PRESENTATION: We report a granulomatous cardiac valve location complicating etanercept treatment in a 26-years-old caucasian male with rheumatoid arthritis...

Rheumatic fever (RF) is a complex syndrome in which the human body develops antibodies against β-haemolytic streptococcus, and triggers inflammation in various organs. RF valvulitis mostly affects the mitral valve (MV) and, to a lesser extent, the aortic valve (AV). Isolated rheumatic tricuspid valve (TV) disease and pericarditis is extremely rare and is not well described in the RF guidelines. The current case demonstrates the echocardiographic findings of TV valvulitis of RF and describes the presentation and electrocardiogram (ECG) changes in pericarditis...

BACKGROUND: Extra-articular manifestations (EAMs) are common in patients with rheumatoid arthritis (RA). Cardiac EAMs are rare but may cause complete heart block and damage to the heart valves. CASE SUMMARY: We present the case of a middle-aged woman with long-standing RA and EAMs as the most prominent symptoms. The patient experienced complete atrioventricular heart block and developed nodular vegetations affecting the mitral valve, ultimately leading to severe mitral regurgitation and valve replacement...

Background During the SARS-CoV2 pandemic, there has been increase in hyperinflammatory presentation in previously healthy children with a variety of cardiac manifestations. Our objective is to describe the cardiac manifestations found in an international cohort of 55 pediatric cases with multi-system inflammatory syndrome (MIS-C) during the SARS-CoV2 pandemic. Methods and Results We reviewed data on previously healthy pediatric patients (≤18 years) with structurally normal hearts who presented at hospitals in the United States, United Kingdom, Spain and Pakistan with MIS-C and had consultation with a pediatric cardiologist...

Cardiac involvement as a complication of severe acute respiratory syndrome coronavirus 2 infection in children is a relatively new entity. We present our initial experience managing children with coronavirus disease 2019-related acute myocardial injury. The 3 patients presented here represent a spectrum of the cardiac involvement noted in children with coronavirus disease 2019-related multisystem inflammatory syndrome, including myocarditis presenting as cardiogenic shock or heart failure with biventricular dysfunction, valvulitis, coronary artery changes, and pericardial effusion...

Herein, we report a case of 61-year-old male with congestive heart failure (HF) due to severe aortic regurgitation (AR) caused by ruptured aortic valve aneurysm (VA). Aortic valve replacement (AVR) was performed after improvement of HF symptoms. Pathological examination of resected aortic VA showed neutrophil infiltration. Although he did not have typical clinical features associated with infective endocarditis (IE) such as fever, leukocytosis, or positive blood culture, we misdiagnosed this case as "concealed IE"...

The role of group A streptococcal and Streptococcus dysgalactiae subspecies equisimilis M-protein specific Abs and T-cells in endothelial cell activation was investigated using cultured rat aortic endothelial cells, and in a rat model of autoimmune valvulitis. Heat inactivated serum and mononuclear cells from streptococcal M-protein immunized rats independently induced upregulation of the endothelial cell adhesion molecules, vascular cell adhesion molecule (VCAM)-1 and intercellular adhesion molecule (ICAM)-1 in cultured cells...

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune mediated diseases triggered by group A streptococcal (GAS) infections. Molecular mimicry between GAS M-proteins and host tissue proteins has been proposed as the mechanism that initiates autoreactive immune responses in ARF/RHD. However, the individual role of antibodies and T-cells specific for GAS M-proteins in the pathogenesis of autoimmune carditis remains under-explored. The current study investigated the role of antibodies and T-cells in the development of carditis in the Lewis rat autoimmune valvultis (RAV) model by transferring serum and/or splenic T-cells from rats previously injected with GAS recombinant M5 protein...