Yokokawa Tetsuro, Nakazato Kazuhiko, Kanno Yuki, Mizukami Hiroyuki, Kobayashi Atsushi, Yoshihisa Akiomi, Takahashi Hiroshi, Shichishima-Nakamura Akiko, Ohkawara Hiroshi, Noji Hideyoshi, Suzuki Hitoshi, Saitoh Shu-ichi, Ogawa Kazuei, Hisa Shinichi, Takeishi Yasuchika
Department of Cardiology and Hematology, Fukushima Medical University, Japan.
Intern Med. 2013;52(10):1061-5. doi: 10.2169/internalmedicine.52.9256. Epub 2013 May 15.
We experienced the case of a 67-year-old man with refractory heart failure. He presented with dyspnea and progressive pitting edema of the lower limbs. Diuretics were insufficient to improve his symptoms. Cardiac catheterization demonstrated pulmonary hypertension. Additional examinations confirmed polyneuropathy, organomegaly, endocrinopathy and monoclonal gammopathy. The plasma vascular endothelial growth factor (VEGF) level was 1,340 pg/mL. The patient was diagnosed with Crow-Fukase (POEMS) syndrome. Echocardiography detected left ventricular hypertrophy and diastolic dysfunction. Polysomnography demonstrated severe sleep-disordered breathing. We herein describe a case of pulmonary hypertension with Crow-Fukase syndrome accompanied by left ventricular diastolic dysfunction and sleep-disordered breathing.
我们遇到了一例67岁的难治性心力衰竭男性患者。他表现为呼吸困难和下肢进行性凹陷性水肿。利尿剂不足以改善他的症状。心脏导管检查显示有肺动脉高压。进一步检查证实存在多神经病变、器官肿大、内分泌病和单克隆丙种球蛋白病。血浆血管内皮生长因子（VEGF）水平为1340 pg/mL。该患者被诊断为Crow-Fukase（POEMS）综合征。超声心动图检测到左心室肥厚和舒张功能障碍。多导睡眠图显示严重的睡眠呼吸紊乱。我们在此描述一例伴有左心室舒张功能障碍和睡眠呼吸紊乱的Crow-Fukase综合征合并肺动脉高压的病例。
