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

Akihiko Sato; Nobuo Sakamoto; Katsuya Ando; Takashi Kaneshiro; Hironori Uekita; Koichi Sugimoto; Takayoshi Yamaki; Hiroyuki Kunii; Kazuhiko Nakazato; Hitoshi Suzuki; Shu-ichi Saitoh; Masatomo Sato; Kazuaki Tamagawa; Takuro Arimura; Akinori Kimura; Yasuchika Takeishi

doi:10.2169/internalmedicine.51.7684

CASE REPORTS

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

Akihiko Sato, Nobuo Sakamoto, Katsuya Ando, Takashi Kaneshiro, Hironori Uekita, Koichi Sugimoto, Takayoshi Yamaki, Hiroyuki Kunii, Kazuhiko Nakazato, Hitoshi Suzuki, Shu-ichi Saitoh, Masatomo Sato, Kazuaki Tamagawa, Takuro Arimura, Akinori Kimura, Yasuchika Takeishi

Author information

Akihiko Sato
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Nobuo Sakamoto
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Katsuya Ando
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Takashi Kaneshiro
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Hironori Uekita
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Koichi Sugimoto
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Takayoshi Yamaki
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Hiroyuki Kunii
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Kazuhiko Nakazato
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Hitoshi Suzuki
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Shu-ichi Saitoh
Department of Cardiology and Hematology, Fukushima Medical University, Japan
Masatomo Sato
Medical Corporation Shoseikai Sato Clinic, Japan
Kazuaki Tamagawa
Fukushima Prefectural Aizu General Hospital, Japan
Takuro Arimura
Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
Akinori Kimura
Department of Molecular Pathogenesis, Medical Research Institute, Tokyo Medical and Dental University, Japan
Yasuchika Takeishi
Department of Cardiology and Hematology, Fukushima Medical University, Japan

Keywords: dilated phase of hypertrophic cardiomyopathy (D-HCM), severe heart failure, sarcomere gene mutation, surgical ventricular reconstruction (SVR), cardiac resynchronization therapy with a defibrillator (CRT-D)

JOURNAL OPEN ACCESS

2012 Volume 51 Issue 18 Pages 2559-2564

DOI https://doi.org/10.2169/internalmedicine.51.7684

Details

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.

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