Noda Hikari, Nitta Manabu, Taguchi Yuka, Matsumoto Katsumi, Sugano Teruyasu, Ishigami Tomoaki, Ishikawa Toshiyuki, Tamura Koichi, Kimura Kazuo
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
J Cardiol Cases. 2020 Apr 16;21(6):205-208. doi: 10.1016/j.jccase.2020.01.003. eCollection 2020 Jun.
A 76-year-old male was admitted to our hospital for progressive bilateral pleural effusion. Because of typical echocardiographic findings such as left ventricular (LV) hypertrophy, thickness of the mitral valve, and a granular sparkling appearance of the LV wall, amyloid cardiomyopathy was suspected. Regardless of up-titration of several diuretic agents, the bilateral pleural effusion did not improve. Because the histological findings of the right ventricular septum (direct-fast-scarlet staining) obtained by biopsy that demonstrated amyloid deposits in perivascular and pericellular lesions, amyloid cardiomyopathy was diagnosed. However, cardiac catheterization revealed normal right and left atrial pressure and normal right and left ventricular end-diastolic pressure. Therefore, hemodynamic deterioration was less likely to be the cause of persistent pleural effusion. Amyloid deposits were also detected in the pleural biopsy specimen, so pleural amyloidosis was diagnosed and may have played an important role in the refractoriness of the pleural effusion. < Systolic and diastolic dysfunction of various degrees can occur in patients with amyloid cardiomyopathy, which is usually progressive and induces heart failure. In these patients, diuretics are key drugs for resolving fluid retention issues such as pleural effusion. In cases of refractory pleural effusion associated with amyloid cardiomyopathy despite aggressive diuretic therapy, these may be induced by pleural amyloidosis.>.
一名76岁男性因进行性双侧胸腔积液入住我院。由于典型的超声心动图表现,如左心室肥厚、二尖瓣增厚以及左心室壁颗粒状闪烁外观,怀疑为淀粉样变心肌病。尽管几种利尿剂的剂量不断增加,但双侧胸腔积液并未改善。通过活检获得的右心室间隔组织学检查结果(直接快速猩红染色)显示血管周围和细胞周围病变中有淀粉样沉积物,因此诊断为淀粉样变心肌病。然而,心导管检查显示右心房和左心房压力正常,右心室和左心室舒张末期压力正常。因此,血流动力学恶化不太可能是持续性胸腔积液的原因。在胸膜活检标本中也检测到淀粉样沉积物,因此诊断为胸膜淀粉样变,这可能在胸腔积液的难治性中起重要作用。<淀粉样变心肌病患者可出现不同程度的收缩和舒张功能障碍,通常呈进行性发展并导致心力衰竭。在这些患者中,利尿剂是解决胸腔积液等液体潴留问题的关键药物。在尽管积极进行利尿治疗但仍与淀粉样变心肌病相关的难治性胸腔积液病例中,可能是由胸膜淀粉样变引起的。>