title: Pulmonary hypertension and refractory heart failure in a patient with Crow-Fukase (POEMS) syndrome.
abstract: 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.
authors.affiliation: Department of Cardiology and Hematology, Fukushima Medical University, Japan.
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Yokokawa
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Nakazato
authors.forename: Yuki
authors.initials: Y
authors.lastname: Kanno
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Mizukami
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kobayashi
authors.forename: Akiomi
authors.initials: A
authors.lastname: Yoshihisa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Akiko
authors.initials: A
authors.lastname: Shichishima-Nakamura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ohkawara
authors.forename: Hideyoshi
authors.initials: H
authors.lastname: Noji
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Suzuki
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Saitoh
authors.forename: Kazuei
authors.initials: K
authors.lastname: Ogawa
authors.forename: Shinichi
authors.initials: S
authors.lastname: Hisa
authors.forename: Yasuchika
authors.initials: Y
authors.lastname: Takeishi
mesh_terms: D000368:Aged; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D004351:Drug Resistance; D004487:Edema / Q000209:etiology; D005665:Furosemide / Q000627:therapeutic use; D006333:Heart Failure / Q000209:etiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging / Q000209:etiology; D008297:Male; D008558:Melphalan / Q000008:administration & dosage / Q000627:therapeutic use; D016878:POEMS Syndrome / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D017286:Polysomnography; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D011669:Pulmonary Wedge Pressure; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000209:etiology; D013792:Thalidomide / Q000008:administration & dosage / Q000627:therapeutic use; D014463:Ultrasonography; D042461:Vascular Endothelial Growth Factor A / Q000097:blood; D018487:Ventricular Dysfunction, Left / Q000209:etiology / Q000503:physiopathology
chemical_list: D004232:Diuretics; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D013792:Thalidomide; D005665:Furosemide; D003907:Dexamethasone; D011239:Prednisolone; D008558:Melphalan