Noonan Syndrome with Double-Chambered Right Ventricle and Atrial Septal Defect -1 Case Report-

Noonan 증후군에 동반된 DCRV와 심방중격결손증 -1례 보고-

  • Park, Young-Woo (Department of thoracic and cardiovascular surgery, Soon Chun Hyang University Medical College) ;
  • Lee, Seock-Yeol (Department of thoracic and cardiovascular surgery, Soon Chun Hyang University Medical College) ;
  • Jeong, Yoon-Seop (Department of thoracic and cardiovascular surgery, Soon Chun Hyang University Medical College) ;
  • Youm, Wook (Department of thoracic and cardiovascular surgery, Soon Chun Hyang University Medical College)
  • 박영우 (순천향대학교 의과대학 흉부외과학교실) ;
  • 이석열 (순천향대학교 의과대학 흉부외과학교실) ;
  • 정윤섭 (순천향대학교 의과대학 흉부외과학교실) ;
  • 염욱 (순천향대학교 의과대학 흉부외과학교실)
  • Published : 2000.05.01

Abstract

Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

Keywords

References

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