Restrictive ventricular septal defect and critical subaortic stenosis in tetralogy of Fallot

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• 作者：Gunter Kerst (1)
  Renate Kaulitz (1)
  Ludger Sieverding (1)
  Christian Apitz (1)
  Gerhard Ziemer (2)
  Michael Hofbeck (1)
  
• 刊名：Clinical Research in Cardiology
• 出版年：2010
• 出版时间：April 2010
• 年：2010
• 卷：99
• 期：4
• 页码：247-249
• 全文大小：205KB
• 参考文献：1. Flanagan MF, Foran RB, Van Praagh R, Jonas R, Sanders SP (1988) Tetralogy of Fallot with obstruction of the ventricular septal defect: spectrum of echocardiographic findings. J Am Coll Cardiol 11:386鈥?95 CrossRef
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• 作者单位：Gunter Kerst (1)
  Renate Kaulitz (1)
  Ludger Sieverding (1)
  Christian Apitz (1)
  Gerhard Ziemer (2)
  Michael Hofbeck (1)
  
  1. Department of Pediatric Cardiology, University Children鈥檚 Hospital, Hoppe-Seyler-Str. 1, 72076, T眉bingen, Germany
  2. Department of Thoracic and Cardiovascular Surgery, University Children鈥檚 Hospital, Hoppe-Seyler-Str. 1, 72076, T眉bingen, Germany
  

文摘

Introduction Tetralogy of Fallot is characterized by a non-restrictive malalignment ventricular septal defect (VSD), an overriding aorta and right ventricular outflow tract obstruction resulting from anterior deviation of the infundibular septum. Due to the large VSD, systolic pressures are equal in both ventricles. In rare cases, redundant tricuspid valve tissue may prolapse into the VSD resulting in restriction of the defect size and causing suprasystemic pressure in the right ventricle. Subaortic obstruction by prolapse of the redundant tricuspid tissue into the left ventricular outflow tract, although theoretically possible in this situation, has not been described yet in the literature. Case report We report on a newborn with tetralogy of Fallot presenting with cyanosis and severe arterial hypotension a few hours after birth. Echocardiography revealed redundant hammock-like accessory tricuspid valve tissue almost completely occluding the originally large VSD. Suprasystemic pressure in the right ventricle resulted in protrusion of tricuspid valve tissue across the VSD and thereby caused severe left ventricular outflow tract obstruction. Emergency pulmonary balloon valvuloplasty performed for decompression of the right ventricle, reduced prolapse of tricuspid tissue into the left ventricular outflow tract and resulted in significant improvement of pulmonary and systemic blood flow. Conclusion In tetralogy of Fallot, accessory tricuspid valve tissue may obstruct the VSD as well as the left ventricular outflow tract resulting in a life threatening condition in newborns shortly after birth.