Dilated Phase of Hypertrophic Cardiomyopathy Caused by Two Different Sarcomere Mutations, Treated with Surgical Left Ventricular Reconstruction and Cardiac Resynchronization Therapy with a Defibrillator

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Author(s)

    • Sato Akihiko
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Suzuki Hitoshi
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Saitoh Shu-ichi
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Arimura Takuro
    • Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
    • Kimura Akinori
    • Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
    • Sakamoto Nobuo
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Ando Katsuya
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Kaneshiro Takashi
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Uekita Hironori
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Sugimoto Koichi
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Yamaki Takayoshi
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Kunii Hiroyuki
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan
    • Nakazato Kazuhiko
    • Department of Cardiology and Hematology, Fukushima Medical University, Japan

Abstract

We herein report the case of a 61-year-old woman with dilated phase of hypertrophic cardiomyopathy (D-HCM) who had been diagnosed with HCM 17 years previously. On admission, her left ventricle (LV) had marked dilation, dyssynchrony with diffuse severe hypokinesis, and ventricular tachycardia. She had two mutations in the cardiac myosin binding protein-C gene, which were suspected to be the causes of the D-HCM. We performed LV reconstruction surgery and cardiac resynchronization therapy with a defibrillator for her drug-resistant severe heart failure. After surgery, her New York Heart Association class dramatically improved, and she has not been re-hospitalized since these treatments.<br>

Journal

  • Internal Medicine

    Internal Medicine 51(18), 2559-2564, 2012

    The Japanese Society of Internal Medicine

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