Surgical Removal of Pulmonary Arteriovenous Malformations Subsequent to Total Cavopulmonary Connection Conversion Long After a Bj?rk Procedure

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• 作者：Fumihiro Ogawa (1)
  Kagami Miyaji (2)
  Masahiro Ishii (3)
  Akira Iyoda (1)
  Yukitoshi Satoh (1)
  
• 关键词：Cavopulmonary shunt ; Complex congenital heart disease ; Lobectomy ; Pulmonary arteriovenous malformations ; Total cavopulmonary connection
• 刊名：Pediatric Cardiology
• 出版年：2013
• 出版时间：March 2013
• 年：2013
• 卷：34
• 期：3
• 页码：739-742
• 全文大小：283KB
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• 作者单位：Fumihiro Ogawa (1)
  Kagami Miyaji (2)
  Masahiro Ishii (3)
  Akira Iyoda (1)
  Yukitoshi Satoh (1)
  
  1. Department of Thoracic Surgery, Kitasato University School of Medicine, 1-15-1, Kitasato, Minami-ku, Sagamihara, Kanagawa, 252-0374, Japan
  2. Department of Cardiovascular Surgery, Kitasato University School of Medicine, Sagamihara, Japan
  3. Department of Pediatrics, Kitasato University School of Medicine, Sagamihara, Japan
  
• ISSN：1432-1971

文摘

Because the cavopulmonary shunt procedure is widely used for palliation of complex congenital heart diseases, pulmonary arteriovenous malformations (PAVMs) are relatively well-known complications. The reported patient was a 23-year-old woman who experienced PAVMs in the right lower lobe after a classical Glenn anastomosis and Bj?rk procedure for tricuspid atresia. Her arterial oxygen saturation (SaO2) 14?years after the Bj?rk procedure was ~80?%. She then underwent a total cavopulmonary connection (TCPC) conversion to reduce her PAVMs in the right lower lobe using the “hepatic factor.-However, her situation remained unchanged, and she experienced severe systemic cyanosis (SaO2, 70?%) and dyspnea during physical exertion without hemoptysis due to increased blood flow to the PAVMs. Although interventional embolization was considered, it was impossible due to considerable dilation of the main PAVM. Thus, right lower lung lobectomy was performed. After surgery, the patient’s SaO2 increased to 90?%. To the authors-knowledge, this is the first case report of a lung resection for residual PAVMs after TCPC conversion.