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Thoracoabdominal aortic replacement in a 6-year-old boy with Loeys-Dietz syndrome

Jianfan Zhen1, Tucheng Sun2, Changjiang Yu1

  • 1Cardiac Surgery Unit, Guangdong Provincial People's Hospital, No.106, Zhongshan 2nd Road, Guangzhou, Guangdong Province, China.

Journal of Cardiothoracic Surgery
|September 17, 2024
PubMed

Insights

Loeys-Dietz syndrome (LDS) can cause aortic dissections in children, requiring early surgical intervention. This case highlights successful thoracoabdominal aortic replacement in a young LDS patient with a Stanford Type B dissection.

Area of Science:

  • Cardiovascular Surgery
  • Pediatric Cardiology
  • Genetics

Background:

  • Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder.
  • LDS significantly increases the risk of aortic aneurysms and dissections, particularly in children.
  • Early surgical intervention is often necessary to manage aortic complications in LDS patients.

Observation:

  • A pediatric patient with LDS underwent a Bentall procedure at age three for an aortic aneurysm.
  • At age six, the same patient presented with severe abdominal pain.
  • Computed tomographic angiography revealed a DeBakey Type III / Stanford Type B aortic dissection.

Findings:

  • A multidisciplinary team decided on surgical intervention for the thoracoabdominal aortic dissection.
  • The patient successfully underwent a thoracoabdominal aortic replacement.
  • This procedure aimed to manage the extensive aortic dissection in a young LDS patient.

Implications:

  • This case demonstrates the feasibility and success of complex aortic surgery in young children with LDS.
  • Aggressive surgical management can be life-extending for pediatric patients with connective tissue disorders and aortic dissections.
  • Highlights the importance of vigilant monitoring and timely intervention for aortic complications in pediatric LDS.

Keywords:
Aortic dissectionChildrenLoeys-Dietz syndromeThoracoabdominal aortic replacement

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