Norwood Procedure Performed on a Patient With Trisomy 13

  • Oka Norihiko
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Inoue Takamichi
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Shibata Miyuki
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Yoshii Takeshi
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Nakamura Yuki
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Araki Haruna
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Matsunaga Yoshikiyo
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Tamura Tomoki
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Itatani Keiichi
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Horai Tetsuya
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Kitamura Tadashi
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Torii Shinzo
    Department of Cardiovascular Surgery, Kitasato University School of Medicine
  • Miyaji Kagami
    Department of Cardiovascular Surgery, Kitasato University School of Medicine

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Abstract

Trisomy 13 is associated with a variety of congenital anomalies, some of which are life-threatening and related to poor prognosis. Therefore, cardiac surgery is rarely offered to these patients, especially to those with complex cardiac anomalies. We report the case of a neonate weighing 2324 g who was born with severe congenital heart defects. Transthoracic echocardiography revealed the diagnoses of asplenia, single ventricle, aortic stenosis, coarctation of the aorta, hypoplastic aortic arch, and total anomalous pulmonary venous return. She was hemodynamically unstable. Palliative Norwood procedure with right ventricle–pulmonary artery conduit (RV–PA conduit) was performed at the age of 1 day to save her life. On postoperative day 7, chromosome analysis revealed trisomy 13. Echocardiography revealed good heart function; stable hemodynamic status was achieved with minimal amounts of inotropic agents. However, she developed anuria, which did not improve despite situational possible interventions, including peritoneal dialysis and continuous hemodiafiltration. On postoperative day 37, she succumbed to sudden cardiorespiratory failure. Nevertheless, this case indicates that a neonate with trisomy 13 can have a better chance at survival with cardiac surgery such as the Norwood procedure with an RV–PA conduit.

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