Background <p>Personalized external aortic root support (PEARS) is a prophylactic surgical technique designed to stabilize the aortic root in patients with heritable thoracic aortic disease (HTAD), including Marfan and Loeys-Dietz syndromes. Although early outcomes have been encouraging, distal aortic complications after PEARS remain poorly characterized. Type B aortic dissection (TBAD) following PEARS implantation has rarely been reported.</p> Case presentation <p>We report two patients with HTAD who developed complicated TBAD several years after PEARS implantation. The first patient, a woman with Loeys-Dietz syndrome, presented with acute TBAD complicated by visceral malperfusion and underwent emergency total arch replacement using the frozen elephant trunk (FET) technique, followed by extensive abdominal surgery for bowel ischemia. The second patient, a man with Marfan syndrome, presented with TBAD and progressive descending thoracic aortic dilatation. He initially underwent open descending thoracic aortic replacement and subsequently required staged arch replacement with FET after retrograde extension of the dissection into the proximal aorta. Both patients survived the acute events and remain under radiological surveillance.</p> Conclusions <p>Distal aortic dissection may still occur in patients with HTAD who have previously undergone PEARS implantation. Although causality cannot be inferred from isolated observations, these cases emphasize the importance of continued imaging surveillance of the entire aorta after PEARS.</p>

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Type B aortic dissection following personalized external aortic root support (PEARS): a report of two cases

  • Alejandro González-Caldevilla Fernandez,
  • Shivan Edmond Saith,
  • Farhin Holia,
  • Ana Lopez-Marco,
  • Aung Ye Oo

摘要

Background

Personalized external aortic root support (PEARS) is a prophylactic surgical technique designed to stabilize the aortic root in patients with heritable thoracic aortic disease (HTAD), including Marfan and Loeys-Dietz syndromes. Although early outcomes have been encouraging, distal aortic complications after PEARS remain poorly characterized. Type B aortic dissection (TBAD) following PEARS implantation has rarely been reported.

Case presentation

We report two patients with HTAD who developed complicated TBAD several years after PEARS implantation. The first patient, a woman with Loeys-Dietz syndrome, presented with acute TBAD complicated by visceral malperfusion and underwent emergency total arch replacement using the frozen elephant trunk (FET) technique, followed by extensive abdominal surgery for bowel ischemia. The second patient, a man with Marfan syndrome, presented with TBAD and progressive descending thoracic aortic dilatation. He initially underwent open descending thoracic aortic replacement and subsequently required staged arch replacement with FET after retrograde extension of the dissection into the proximal aorta. Both patients survived the acute events and remain under radiological surveillance.

Conclusions

Distal aortic dissection may still occur in patients with HTAD who have previously undergone PEARS implantation. Although causality cannot be inferred from isolated observations, these cases emphasize the importance of continued imaging surveillance of the entire aorta after PEARS.