Chopra P, Bhatia M L
Department of Pathology, All India Institute of Medical Sciences, New Delhi.
J Heart Valve Dis. 1992 Sep;1(1):92-101.
Rheumatic heart disease contributes to significant cardiac morbidity and mortality in India. The disease predominantly affects the valvular endocardium culminating in crippling valvular deformities, preferentially involving the mitral valve which may be severely affected in children and young adults. This appears to be unique to India and has been termed juvenile mitral stenosis. It is characterized by cardiomegaly, refractory congestive heart failure, and marked by elevated pulmonary vascular pressures and a progressive, fulminant clinical course. Autopsies of patients dying of rheumatic heart disease revealed that the mitral valve was most commonly afflicted either alone or in combination with the aortic and tricuspid valves in 31.6% and 52.8%, respectively. Organic involvement of the tricuspid valve was documented in 38.4% of cases. The extent and severity of the disease process was most marked in the mitral valve, followed by the aortic and tricuspid valves. Mitral valves showed various degrees of calcification, moderate or severe calcification being observed in 36.4%. Chronic inflammatory cell infiltration was observed in both calcified and non-calcified valves. The phenotypic profile of the inflammatory cells by immunohistochemical staining revealed a significant number to be T-helper/inducer lymphocytes. Lungs from cases of mitral stenosis exhibited prominent vascular and parenchymal changes. Pulmonary vessels revealed moderate to marked medial hypertrophy of the medium sized branches of the pulmonary artery. Dilatation lesions were also seen in a few cases. The most striking parenchymal change was the prominent smooth muscle in the bronchoalveolar walls. The extent and severity of the vascular and parenchymal changes were more marked in juvenile patients. The presence of inflammatory cells in cases of chronic heart disease reflects a possible ongoing insult/injury to some persistent antigenic stimulus by beta hemolytic streptococcal antigens that have primed the various target tissues. Further study of surface characteristics of various mesenchymal cells may help in understanding the nature and pathogenesis of this serious cardiac problem.
风湿性心脏病在印度导致了严重的心脏发病率和死亡率。该疾病主要影响瓣膜性心内膜，最终导致致残性瓣膜畸形，尤其累及二尖瓣，儿童和年轻人可能会受到严重影响。这在印度似乎是独特的，被称为青少年二尖瓣狭窄。其特征为心脏肥大、难治性充血性心力衰竭，表现为肺血管压力升高以及进展迅速、病情凶险的临床过程。对死于风湿性心脏病患者的尸检显示，二尖瓣最常单独受累，或分别与主动脉瓣和三尖瓣联合受累，比例分别为31.6%和52.8%。38.4%的病例记录有三尖瓣的器质性受累。疾病过程的范围和严重程度在二尖瓣最为明显，其次是主动脉瓣和三尖瓣。二尖瓣显示出不同程度的钙化，36.4%观察到中度或重度钙化。在钙化和未钙化的瓣膜中均观察到慢性炎症细胞浸润。通过免疫组织化学染色对炎症细胞的表型分析显示，大量为辅助性T/诱导性淋巴细胞。二尖瓣狭窄病例的肺部表现出明显的血管和实质改变。肺血管显示肺动脉中、小分支有中度至明显的中膜肥厚。少数病例还可见扩张性病变。最显著的实质改变是支气管肺泡壁中突出的平滑肌。血管和实质改变的范围和严重程度在青少年患者中更为明显。慢性心脏病病例中炎症细胞的存在反映了可能存在对某些持续抗原刺激的持续损伤，这些刺激是由引发各种靶组织的β溶血性链球菌抗原引起的。进一步研究各种间充质细胞的表面特征可能有助于理解这一严重心脏问题的性质和发病机制。
