Valvulopathies of Rheumatic Origin: Management and Complications

Results

• Valvular Heart Disease Epidemiology Aluru JS, et al. [1] Valvular heart disease is a rapidly growing cause of global cardiovascular morbidity and mortality with diverse and evolving geographic distribution. Infective endocarditis prevalence has also grown in developed nations, likely due to population aging and the increased utilization of transcatheter valve replacement and prosthetic valves as interventions against the previously discussed valvular pathologies.
• Contemporary Diagnosis and Management of Rheumatic Heart Disease: Implications for Closing the Gap: A Scientific Statement From the American Heart Association, Kumar RK, et al. [2]. In most endemic regions, affected patients present with heart failure. This statement will seek to examine the current state-of-the- art recommendations and to identify gaps in diagnosis and treatment globally that can inform strategies for reducing disease burden. This set of works forms the basis on which a complementary document on the defense of rheumatic heart diseases was developed. Ultimately, the combination of expanded treatment options, research and advocacy based on existing knowledge and science provides the best opportunity to address the burden of rheumatic heart disease.
• Rheumatic Heart Disease current status of diagnosis and therapy Peters F, et al. [5]. Rheumatic heart disease (RHD) is the only preventable cardiovascular disease which causes significant morbidity and mortality particularly in low- and middle-income countries. As we are already more than a year from the historic 2018 World Heart Organization Resolution against Rheumatic Fever and Rheumatic Heart Disease, we advocate strongly for renewed efforts to prioritize this disease across the endemic regions of the world.
• The occult rheumatic scourge: A clinicopathological analysis of missed rheumatic heart disease DAGA P, et al. [6]. To study the clinical and pathological manifestations of missed cases of rheumatic heart disease (RHD) and postulate possible reasons behind a missed diagnosis. Our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies.
• Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease Gomes NFA, et al. [7]. The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. Despite an intense degree of fibrosis, the inflammatory process remains active in the rheumatic mitral valve, even at late disease with valve dysfunction. Calcification predominated in stenotic valves and in patients with right ventricular dysfunction.
• Rheumatic Heart Disease: An assessment of the incidence of hospitalizations and its challenges for public health Chitolina BC, et al. [8]. Hospitalization for rheumatic heart disease is a topic of significant importance in the health sector, especially in regions where socioeconomic conditions can impact access to adequate medical care. While we have seen advances in the understanding and treatment of rheumatic heart disease over the past few decades, the persistence of these hospitalizations highlights significant gaps in primary prevention, early diagnosis, and access to appropriate healthcare, especially in underserved communities.
• Rheumatic Heart Disease in the Developing World Simpson MT, et al. [9]. Despite recent public policy initiatives, rheumatic heart disease (RHD) remains a major source of morbidity worldwide. Rheumatic heart disease occurs as a sequela of Streptococcus pyogenes (group A streptococcal [GAS]) infection in patients with genetic susceptibility.

The cardiac surgical community has attempted to improve the availability of surgery in RHD-endemic areas with some success, and operative techniques and outcomes of valve repair continue to improve, potentially offering patients a safer, more durable operation. Innovation offers hope for a more scalable solution with improved biomaterials and transcatheter delivery technology; however, cost remains a barrier.

Aortic valve stenotic disease is the most commonly occurring valvular pathology in developed nations (afflicting 9 million people worldwide) and its prevalence has been increasing with population aging and the increased prevalence of atherosclerosis. Aortic regurgitation is associated with diastolic, but not systolic, hypertension and it has likewise seen a rise in the developed world. Mitral regurgitation affects 24 million people worldwide, with great variability between and among nations. Primary mitral regurgitation arises as a consequence of myxomatous degeneration and mitral valve prolapse, which is largely due to genetic predispositions, while secondary mitral regurgitation accounts for 65% of cases and arises secondary to dilation and heart failure. Tricuspid regurgitation has become more prevalent in developed nations due to the increased usage of intracardiac pacemakers [1].

Patients with isolated mitral stenosis often benefit from percutaneous mitral balloon valvuloplasty. Timely heart valve surgery can mitigate progression to heart failure, disability, and death. Valve repair is preferable to replacement for rheumatic mitral regurgitation, but is not available for the vast majority of patients in endemic regions. . Ultimately, the combination of expanded treatment options, research and advocacy based on existing knowledge and science provides the best opportunity to address the burden of rheumatic heart disease [2].

RHD typically affects left-sided valves, with greater affinity and consequence for the mitral valve. Characteristic acute mitral valvulitis shows mitral annulus dilatation, chordal elongation, and anterior leaflet prolapse, with varying degrees of MR and rarely chordal rupture. Isolated aortic disease occurs in 2% of cases. Right-sided valve disease is not infrequent, typically affects the tricuspid valve (as primary valvulitis or as the result of deleterious hemodynamic consequences of left-sidedvalve disease), and rarely affects the pulmonic valve. Acute rheumatic valvulitis manifests as valvular regurgitation, but over time, chronic inflammation leads to valve stenosis from commissural fusion with or without associated regurgitation in a subset of patients. MS from commissural fusion, with variable degrees of involvement of other parts of the mitral valve apparatus, is the hallmark lesion of the later stages of RHD. The more malignant fulminant course of RHD, linked to recurrent bouts of ARF, occurs in the most endemic regions of the world [2].

The key to the diagnosis of valvular dysfunction requires integration of typical morphological abnormality with evidence of pathological valvular regurgitation. Echocardiography can also be utilized to identify pericardial effusion as well as focal or global left ventricular dysfunction which are other supportive manifestations of carditis. Contemporary data from the Global Rheumatic Heart Disease Registry (REMEDY) revealed that the most common valve lesions encountered were mixed left sided lesions with multivalvular involvement occurred in almost two- thirds of subjects. Once a diagnosis of ARF has been made definitive therapy to eradicate Strep A in the pharynx with penicillin is required. This can be either an intramuscular dose of benzathine penicillin or oral penicillin for 10 days. In patients who present with heart failure, the use of diuretics, ACE inhibitors, digoxin and beta blockers are used to stabilize patients prior to definitive corrective surgery or as definitive therapy when surgery is not available, or patients are deemed surgically inoperable. In patients with RHVD and AF, restoration of sinus rhythm maybe beneficial for selected patients with either amiodarone, cardioversion or even catheter ablation although definitive surgical correction of the primary valve lesions often offers the greatest predictable success [5].

In a study conducted on patients with clinical and pathological manifestations of undiagnosed cases of rheumatic heart disease (RHD) and postulating possible reasons behind an undiagnosed diagnosis, it was observed that: our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies [6].

Studies indicate that the autoimmune process involved in DCR begins when reactive antibodies bind to the valve endothelium, leading to inflammation and cellular infiltration. Once activated, the valve endothelium increases the expression of adhesion molecules, which facilitates the binding and infiltration of T cells. After the initial valve insult, the process triggers a cascade that leads to the recognition of additional epitopes, leading to progressive damage of the valve. Evidence that the continuous presentation of autoantigens at the site of injury contributes to an amplification of the immune response is reinforced by the significant reduction in autoantibody levels after surgical removal of the affected leaflets [7]. Hospitalizations for rheumatic heart disease reflect not only the clinical challenges faced by patients, but also the complexities of the healthcare system in meeting the needs of these individuals [8].

Rheumatic heart disease (RHD) remains an underrecognized health issue globally, despite initiatives introduced over the last several decades that have helped decrease the global number of cases during this time. This is in part due to the highly effective public health policies put forth in industrialized nations in the last half-century to prevent, recognize, and treat group A streptococcal (GAS) infections. However, worldwide RHD cases number over 15 million and contribute to over two hundred thousand deaths per year.1 Progress has been made over the last 2 decades as several nations in the developing world with high disease prevalence have prioritized its treatment and global health organizations have pursued the elimination of RHD with renewed vigor. The effects of RHD are disproportionately felt by school-aged children and young adults in their most economically productive years. The cost of premature deaths due to RHD is estimated to be on the order of trillions of US dollars annually.6 Effects on children and adolescents include increased school absence and rates of dropout, which have a downstream impact on the ability of children to eventually support their own families independently. At the same time, young adults sick with RHD will have decreased capacity to work, impairing their ability to provide for themselves and their families. The impact of disability caused by RHD accounts for nearly 0.5% of worldwide disability adjusted life years [9].