Pulmonary and systemic hypertension in the setting of Moya-Moya disease due to disseminated fibromuscular dysplasia

  • Takeshi Nakano, Department of Pediatric Cardiology, Ehime central prefectural Hospital, Ehime, Japan, Japan
  • Shin Ono, Department of Pediatric Cardiology, Ehime central prefectural Hospital, Ehime, Japan, Japan
  • Toshiyuki Chisaka, Department of Pediatric Cardiology, Ehime central prefectural Hospital, Ehime, Japan, Japan
  • Takashi Higaki, Department of Pediatrics, Ehime University Graduate School of Medicine, Japan
  • Eiichi Yamamoto, Department of Pediatrics, Ehime University Graduate School of Medicine, Japan
  • Dr Masaaki Ohta, Department of Pediatrics, Ehime University Graduate School of Medicine, Japan
  • Dr Kikuko Murao, Department of Pediatrics, Ehime University Graduate School of Medicine, Japan
  • Dr Eiichi Ishii, Department of Pediatrics, Ehime University Graduate School of Medicine, Japan

    • A case report
    The patient, a girl aged 13 years, had been diagnosed acquired cytomegalovirus encephalitis at the age of a month and followed up as epilepsy since then. She was diagnosed Moya-moya disease due to cataplexy of lower extremity at the age of 10 years and underwent surgical treatment, but she had felt general fatigue and shortness of breath on exertion since these time.
    At the age of 12 years, she fell unconscious and was diagnosed plumonary hypertension. ECG pattern was marked right ventricle hypertrophy. Pulmonary perfusion scintigraphy showed multifocal perfusion defects.
    Cardiac catheterization revealed systolic pressure of aorta/pulmonary artery of 160/100 mmHg and pulmonary vascular resistance was 17Um2. Selective pulmonary angiography demonstrated multiple stenosis of peripheral pulmonary artery, and intimal thickening was observed by the intravascular ultrasound.
    Segmental stenosis at the proximal potion of the celiac trunk and narrowing of the coronary artery, supramesenteriac artery and abdominal aorta extending to the femoral arteries were noted. No findings of segmental stenosis of the renal arteries and serum renin level was not elevated. Systemic blood pressure at rest is fluctuating at the range of 120 to 160mmHg because the elasticity of the systemic arteries is decreased.
    She is currently receiving warfarin, beraprost, bosentan and O2 inhalation, her symptoms almost disappear. Brain natriuretic peptide level and six-minutes walk test are improved.
    We estimate the stenotic lesions are progressive, therefore we are considering to perform the balloon angioplasty for pulmonary stenosis if the symptoms worsen in future.