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Background <p>Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. While most undergo corrective surgery in childhood, a subset of patients survives to adulthood without repair, facing risks of heart failure, malignant arrhythmias, and acute decompensation, which pose significant management challenges.</p> Case presentation <p>This case report describes a 38-year-old male with uncorrected Tetralogy of Fallot (TOF) who presented with altered mental status, severe hypoxemia, and circulatory instability. Diagnosis was confirmed by congenital heart disease-related great vessel CTA and transesophageal echocardiography. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) was initiated to correct hypoxia and provide circulatory support, resulting in marked improvement in oxygenation, hemodynamics, and recovery of consciousness. Subsequently, the patient underwent complete TOF repair with ongoing ECMO support and recovered favorably postoperatively. This article also reviews the literature regarding management strategies for adult TOF and highlights the role of ECMO in the perioperative setting.</p> Conclusions <p>This case demonstrates that early consideration of V-A ECMO, guided by multidisciplinary decision-making, can be a lifesaving intervention for stabilising adult patients with uncorrected TOF presenting in extremis, successfully bridging them to definitive surgical repair. However, further studies are needed to establish standardized criteria for ECMO use in this population. Accurate and timely diagnosis relies heavily on advanced cardiovascular imaging, and multidisciplinary collaboration is paramount for managing perioperative complications.</p>

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Venoarterial extracorporeal membrane oxygenation as a bridge to definitive repair in an adult with uncorrected Tetralogy of Fallot: a case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年5月13日

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  • Yun Li,
  • Xianjun Chen,
  • Wei Zhao,
  • Ning Zhu,
  • Yehong Li,
  • Guangsu Li,
  • Junling Tao,
  • Ouyang Rao,
  • Sha Xie,
  • Jie Hu,
  • Ying Liu

摘要

Background

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. While most undergo corrective surgery in childhood, a subset of patients survives to adulthood without repair, facing risks of heart failure, malignant arrhythmias, and acute decompensation, which pose significant management challenges.

Case presentation

This case report describes a 38-year-old male with uncorrected Tetralogy of Fallot (TOF) who presented with altered mental status, severe hypoxemia, and circulatory instability. Diagnosis was confirmed by congenital heart disease-related great vessel CTA and transesophageal echocardiography. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) was initiated to correct hypoxia and provide circulatory support, resulting in marked improvement in oxygenation, hemodynamics, and recovery of consciousness. Subsequently, the patient underwent complete TOF repair with ongoing ECMO support and recovered favorably postoperatively. This article also reviews the literature regarding management strategies for adult TOF and highlights the role of ECMO in the perioperative setting.

Conclusions

This case demonstrates that early consideration of V-A ECMO, guided by multidisciplinary decision-making, can be a lifesaving intervention for stabilising adult patients with uncorrected TOF presenting in extremis, successfully bridging them to definitive surgical repair. However, further studies are needed to establish standardized criteria for ECMO use in this population. Accurate and timely diagnosis relies heavily on advanced cardiovascular imaging, and multidisciplinary collaboration is paramount for managing perioperative complications.