Pulmonary Hypertension and Refractory Heart Failure in a Patient with Crow-Fukase (POEMS) Syndrome

作者：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

来源：Internal Medicine, 2013, 52(10): 1061-1065.

DOI：10.2169/internalmedicine.52.9256

• pulmonary hypertension
• Crow-Fukase (POEMS) syndrome
• heart failure
• vascular endothelial growth factor (VEGF)
• diastolic dysfunction
• sleep-disordered breathing

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.

• 出版日期2013