Maia Cintia Prado, Gali Luís Gustavo, Schmidt André, de Almeida Filho Oswaldo César, Santos Marcel Koenigkam, Saraiva Luciano Albuquerque Lima, Rodrigues Alfredo José, Maciel Benedito Carlos, Romano Minna Moreira Dias
Internal Medical Department, Cardiology Center, Medicine School of Ribeirão Preto, São Paulo University, Ribeirão Preto, Brazil.
Internal Medicine Department, Image and Medical Physics Center, Medicine School of Ribeirão Preto, São Paulo University, Ribeirão Preto, Brazil.
Echocardiography. 2016 Jul;33(7):1080-4. doi: 10.1111/echo.13244. Epub 2016 May 4.
Endomyocardial fibrosis, which is a cause of restrictive cardiomyopathy, is characterized by the deposition of fibrous tissue in the apical region of 1 or both ventricles. The condition not only affects the diastolic dynamics of the ventricles, but also the function of the atrioventricular valves. The disease occurs predominantly in tropical regions worldwide and in sub-Saharan Africa. This condition is not well understood, with varied manifestations, from subclinical presentations to chronic and progressive edematous syndromes. Here, we present the challenging case of a patient with an indeterminate echocardiographic image, suggesting apical hypertrophy, plus severe aortic stenosis and fibrosis of the left ventricular outflow tract. An electrocardiogram revealed symmetrical T-wave inversion, which is a characteristic manifestation of apical hypertrophy. The importance of cardiac imaging examinations such as echocardiography and cardiac magnetic resonance for differentiating between endomyocardial fibrosis and apical hypertrophy is highlighted in this patient's case.
心内膜心肌纤维化是限制性心肌病的一个病因,其特征是在一个或两个心室的心尖区域有纤维组织沉积。这种病症不仅会影响心室的舒张动力学,还会影响房室瓣的功能。该疾病主要发生在全球热带地区以及撒哈拉以南非洲。这种病症尚未得到充分了解,表现形式多样,从亚临床症状到慢性进行性水肿综合征。在此,我们介绍一例具有挑战性的病例,该患者超声心动图图像不明确,提示心尖肥厚,同时伴有严重主动脉瓣狭窄和左心室流出道纤维化。心电图显示T波对称性倒置,这是心尖肥厚的特征性表现。该患者病例突出了超声心动图和心脏磁共振等心脏成像检查在鉴别心内膜心肌纤维化和心尖肥厚方面的重要性。