Advanced Pulmonary Hypertension Due to Congenital Double-shunt Successfully Treated with Surgical Repair and Up-front Combination Therapy

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  • Suto Makiko
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Matsumoto Kensuke
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Kido Sachiko
    Division of Cardiology, Kobe Children's Hospital, Japan
  • Mori Shumpei
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Nakayama Kazuhiko
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Tanaka Hidekazu
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Inoue Takeshi
    Division of Cardiovascular Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Japan
  • Emoto Noriaki
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan
  • Okita Yutaka
    Division of Cardiovascular Surgery, Department of Surgery, Kobe University Graduate School of Medicine, Japan
  • Hirata Ken-ichi
    Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan

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<p>This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg. Although both shunts had bidirectional flow, based on the results of acute pulmonary vasoreactive testing, one-stage surgical closure was performed followed by up-front combination therapy for post-operative pulmonary hypertensive crisis and residual PAH. At 14 months after the surgery, her symptoms were markedly improved, and her mean PAP had dramatically decreased to 13 mmHg. </p>

収録刊行物

  • Internal Medicine

    Internal Medicine 58 (9), 1301-1305, 2019-05-01

    一般社団法人 日本内科学会

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