Taçoy Gülten, Tavil Yusuf, Abaci Adnan
Department of Cardiology, Medicine Faculty of Gazi University, Ankara, Turkey.
Turk Kardiyol Dern Ars. 2009 Sep;37(6):417-20.
Endomyocardial fibrosis is a cause of restrictive cardiomyopathy and it generally occurs in tropical regions more commonly affecting children and young adults. A 19-year-old male patient presented with edema in the lower extremities and fatigue. Transthoracic echocardiography showed dilated right heart chambers, restrictive physiology in the left ventricle, and increased tissue growth in the right ventricle that caused a 60-mmHg gradient and obliteration. Magnetic resonance imaging confirmed the presence of increased tissue formation in the right ventricular inflow region. Surgical resection was not considered taking into account the functional capacity of the patient (class II), disappearance of symptoms following medical treatment, and the high risk for operative mortality. The presented case may arouse interest in that increased tissue growth in the right ventricle inflow region caused a gradient in the right ventricle, leading to an incorrect diagnosis, at another center, as idiopathic pulmonary hypertension.
心内膜心肌纤维化是限制性心肌病的一个病因,通常发生在热带地区,更常见于儿童和年轻人。一名19岁男性患者出现下肢水肿和疲劳。经胸超声心动图显示右心腔扩张、左心室限制性生理改变以及右心室组织生长增加,导致60 mmHg的压力阶差和管腔闭塞。磁共振成像证实右心室流入区域存在组织形成增加。考虑到患者的功能状态(II级)、药物治疗后症状消失以及手术死亡率高,未考虑手术切除。该病例可能引起关注,因为右心室流入区域组织生长增加导致右心室压力阶差,在另一个中心被误诊为特发性肺动脉高压。