Caracterização Histológica das Lesões da Valva Mitral de Pacientes com Cardiopatia Reumática / Histopathological Characterization of Mitral Valvular Lesions from Patients with Rheumatic Heart Disease

Resumo Fundamentos: Os mecanismos subjacentes pelos quais a doença cardíaca reumática (DCR) levam à disfunção valvar grave não são totalmente compreendidos. Objetivo: O presente estudo avaliou as alterações histopatológicas nas valvas mitrais (VM) buscando uma associação entre o padrão de disfunção valvar predominante e os achados histopatológicos. Métodos: Em 40 pacientes submetidos à troca da VM devido a DCR e em 20 controles submetidos a transplante cardíaco, foram analisados os aspectos histológicos da VM excisada. Dados clínicos e ecocardiográficos também foram coletados. As análises histológicas foram realizadas usando coloração com hematoxilina-eosina. Determinou-se inflamação, fibrose, neoangiogênese, calcificação e metaplasia adiposa. Valores de p<0,05 foram considerados estatisticamente significativos. Resultados: A idade média dos pacientes com DCR foi de 53±13 anos, sendo 36 (90%) do sexo feminino, enquanto a idade média dos controles foi de 50±12 anos, semelhante aos casos, sendo a maioria do sexo masculino (70%). O endocárdio valvar reumático apresentou espessura maior que os controles (1,3±0,5 mm versus 0,90±0,4 mm, p=0,003, respectivamente), e infiltrado inflamatório mais intenso no endocárdio (78% versus 36%; p=0,004), com predominância de células mononucleares. Ocorreu fibrose moderada a acentuada mais frequentemente em válvulas reumáticas do que em válvulas controle (100% vs. 29%; p<0,001). Ocorreu calcificação em 35% das valvas reumáticas, principalmente entre as valvas estenóticas, associada à área valvar mitral (p=0,003). Conclusões: Apesar do intenso grau de fibrose, o processo inflamatório permanece ativo na valva mitral reumática, mesmo em doença tardia com disfunção valvar. A calcificação predominou em valvas estenóticas e em pacientes com disfunção ventricular direita.

Abstract Background: The underlying mechanisms by which rheumatic heart disease (RHD) lead to severe valve dysfunction are not completely understood. Objective: The present study evaluated the histopathological changes in mitral valves (MV) seeking an association between the pattern of predominant valvular dysfunction and histopathological findings. Methods: In 40 patients who underwent MV replacement due to RHD, and in 20 controls that underwent heart transplant, histological aspects of the excised MV were analyzed. Clinical and echocardiographic data were also collected. Histological analyses were performed using hematoxylin-eosin staining. Inflammation, fibrosis, neoangiogenesis, calcification and adipose metaplasia were determined. A p value<0.05 was considered to be statistically significant. Results: The mean age of RHD patients was 53±13 years, 36 (90%) were female, whereas the mean age of controls was 50±12 years, similar to the cases, with the majority of males (70%). The rheumatic valve endocardium presented greater thickness than the controls (1.3±0.5 mm versus 0.90±0.4 mm, p=0.003, respectively), and a more intense inflammatory infiltrate in the endocardium (78% versus 36%; p=0.004), with predominance of mononuclear cells. Moderate to marked fibrosis occurred more frequently in rheumatic valves than in control valves (100% vs. 29%; p<0.001). Calcification occurred in 35% of rheumatic valves, especially among stenotic valves, which was associated with the mitral valve area (p=0.003). Conclusions: Despite 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 in the modern era: recent developments and current challenges

Abstract Rheumatic heart disease (RHD) remains a major cause of preventable death and disability in children and young adults. Despite significant advances in medical technology and increased understanding of disease mechanisms, RHD continues to be a serious public health problem throughout the world, especially in low- and middle-income countries. Echocardiographic screening has played a key role in improving the accuracy of diagnosing RHD and has highlighted the disease burden. Most affected patients present with severe valve disease and limited access to life-saving cardiac surgery or percutaneous valve intervention, contributing to increased mortality and other complications. Although understanding of disease pathogenesis has advanced in recent years, key questions remain to be addressed. Preventing or providing early treatment for streptococcal infections is the most important step in reducing the burden of this disease.

Prevalence of Rheumatic Fever and Rheumatic Heart Disease Among School Children.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are significant public health concerns in the developing countries like India. A total number of 10330 school children were examined clinically for evidence of acute rheumatic fever and rheumatic heart disease . Those suffering with the disease were subjected to electrocardiographic, rontgenographic and echocardiographic examination. Prevalence of acute rheumatic fever and rheumatic heart disease was found to be 0.87 per 1000.

Clinical and echocardiographic features of children with rheumatic carditis: correlation with high sensitivity C-reactive protein

Rheumatic fever [RF] and rheumatic heart disease [RHD] are leading causes of cardiovascular mortality and morbidity in developing countries. To describe the clinical and echocardiographic features of children with RF and RHD and compare these features with their serum C-reactive protein in 2 pediatric cardiology centers in Khartoum. It was a prospective cross sectional study. Patients were examined clinically and by echocardiography. Serum high sensitivity C-reactive protein [hsCRP] was measured from children with Acute RF as well as from healthy age and sex matched controls selected from children attending the clinics. Statistical Analysis Used: Mean and standard deviation, P value using Fisher's exact test. Sixty six patients [45% males] were enrolled. Mitral regurgitation [MR] was found in 65 patients [98%], it was severe in 42 patients [64%], combined with aortic regurgitation [AR] in 27 patients [41%] and with Mitral stenosis [MS] in 3 patients [4.5%]. For patients with carditis, hsCRP ranged between 1.10 and 15 mg/1 [mean 8.0817, SD 4.47]. In the control group it was 0.6-1.3 mg/1 [mean 0.93 SD 0.23] P<0.0001.Patients with Acute RF had hsCRP mean of 12.35 mg/1 [SD 2.11] while those with chronic RHD had hsCRP mean of 7.34 mg/1 [SD 4.16], P<0.0001. RHD is manifested in our patients with severe valve damage dominated by MR and there is evidence of an ongoing inflammation during the chronic phase. RHD is manifested in a severe form in Sudan. High sensitivity CRP is elevated in acute phase as well as in the chronic phase

Efficacy of echocardiographic criteria for diagnosis of carditis in acute rheumatic fever.

Background: There is a great need forECHOcriteria for accurate diagnosis of carditis in acute rheumatic fever. Aim: To propose and test the efficacy of ECHO criteria for accurate diagnosis of carditis. Material and Methods: The 333 cases underwent detailed clinical examination, laboratory tests and meticulous Echocardiographic study.Vijay’s ECHO criteria for the diagnosis of carditis / subclinical valvulitis was used. 220 (66.06%) cases were both Jones’ positive and ECHO positive [True +ve], 52 cases (15.61%), probably had subclinical carditis as murmur was not heard (Jones’-ve) but ECHO was positive [False - ve]. Four cases, clinically diagnosed as carditis were Jones’+ve ,but ECHO showed congenital heart disease [False +ve]. 57 cases (17.11%) were clinically , echocardiographically and Jones’ negative were taken as control (True –ve). Sensitivity is81%and specificity is 93%. Conclusions: Precise diagnosis of both carditis /subclinical valvulitis is possible with Vijay’s ECHO criteria. ECHO should be included as a major criterion in Jones’criteria.

Usefulness of echocardiography in detection of subclinical carditis in acute rheumatic polyarthritis and rheumatic chorea.

Detailed echocardiographic analysis was performed in 10 children with first episode of acute rheumatic fever who presented with acute rheumatic polyarthritis or rheumatic chorea and had no clinically detectable evidence of active carditis. Significant changes were observed in the form of mitral valve prolapse with regurgitation in 3, aortic valve prolapse with regurgitation in 1 and mitral valve billowing without regurgitation in 1 patient each. A significant (p < 0.001) anterior mitral chordal elongation was observed in both the groups--rheumatic polyarthritis and chorea when compared with age and sex matched control subjects. Mitral annular diameter was found to be increased (p < 0.001) in patients presenting with polyarthritis alone. These observations of clinically silent but echocardiographically detectable element of carditis forms the basis of how patients of acute rheumatic fever develop permanent valvular deformities in their latter lives without revealing any cardiac affection earlier.

Subclinical carditis in pure Sydenham's chorea: a color doppler study

Sydenham's chorea [SC] is a late manifestation of rheumatic fever [RF] and its presence alone warrants the diagnosis of RF. Although SC seems to be a benign condition, 27% of patients who present with "pure chorea" to develop most likely rheumatic valvular disease within thirty years of the initial attack. To study the cardiac involvement in chorea patients without clinical signs of carditis the following investigation was carried out: Seventeen patients with chorea aged 7-15 years [mean +/- SD: 11.3 +/- 1.9] and 17 neurologic patients together with 28 normal children were surveyed with color flow imaging [CFI]. All chorea patients had left sided valvular regurgitation, whereas only one in each control group had the same finding, [p< 0.001]. We conclude that many, if not all, of the Sydenham's chorea patients have subclinical carditis. Doppler study is a valuable tool for detection of cardiac involvement. This underlines the rationale for patients with SC and "normal" heart to be placed on chemoprophylaxis, despite the absence of auscultatory findings on physical examination. To further evaluate these findings a larger group of children should be studied

Multiplane transesophageal echocardiography: our initial experience.

We present our experience in the diagnostic assessment of a wide spectrum of cardiovascular disorders using multiplane transesophageal echocardiography (MP-TEE). Two hundred and seventeen patients in the age range of 11-71 years were subjected to MP-TEE from January to November 1993. The male:female ratio was 1.1:1. One hundred and ten patients had predominantly mitral valve disease of rheumatic origin, eleven had mitral valve prolapse, twenty patients had aortic valve disease and thirty seven patients had more than one valve involvement. Six patients with suspected prosthetic heart valve dysfunction and ten patients of hypertrophic cardiomyopathy were also studied. Two patients had unexplained pulmonary hypertension, three had pericardial disease and three had proximal aortic dissections. Twenty six patients with congenital heart disease were studied of which nineteen had atrial septal defects, one had corrected transposition of great vessels with pulmonic stenosis and one adult had Ebstein's anomaly of the tricuspid valve. In our experience, MP-TEE enhances the versatility of TEE by providing incremental diagnostic information and enhancing delineation of pathology. The procedure was well-tolerated and no complications occurred.

Noninvasive estimation of pulmonary artery wedge pressure by dual-M-mode-echocardiography.

Pulmonary artery wedge pressure (PAWP) measurement is invasive, associated with complications, contraindications and its high cost limits its use in clinical practice. We evaluated the use of dual-M-mode-echocardiography as a possible noninvasive alternative method in 20 patients for estimating the PAWP. The interval from the Q-wave of the electrocardiogram (ECG) to the mitral valve closure on the mitral valve M-mode-echocardiogram (Q-MVC), the interval between the aortic valve closure and mitral valve E point (AVC-E) on aortic and mitral valve M-mode-ECG respectively and the ratio of these intervals (Q-MVC/AVC-E) was correlated to the mean PAWP measured at catheterization. The mean PAWP correlated excellently with Q-MVC/AVC-E ratio [r = 0.89, p < 0.0001, y = 14.51 (Q-MVC/AVC-E) + 6.71]. The estimation of PAWP by a dual-M-mode-ECG offers a useful estimate of mean PAWP noninvasively.