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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.

Citation

Tetsuro Yokokawa, Kazuhiko Nakazato, Yuki Kanno, Hiroyuki Mizukami, Atsushi Kobayashi, Akiomi Yoshihisa, Hiroshi Takahashi, Akiko Shichishima-Nakamura, Hiroshi Ohkawara, Hideyoshi Noji, Hitoshi Suzuki, Shu-ichi Saitoh, Kazuei Ogawa, Shinichi Hisa, Yasuchika Takeishi. Pulmonary hypertension and refractory heart failure in a patient with Crow-Fukase (POEMS) syndrome. Internal medicine (Tokyo, Japan). 2013;52(10):1061-5


PMID: 23676591

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