Table 1 Surgical recommendations in Loeys-Dietz syndrome (from Williams et al., modified) [19]
From: Neonatal presentation of Loeys-Dietz syndrome: two case reports and review of the literature
Children | Adults | |
|---|---|---|
Severe craniofacial features | Mild craniofacial features | |
1) Aortic root z-score > 3 or rapidly expanding (0.5 cm over 1 year) | 1) Aortic root z-score > 4 or rapidly expanding (0.5 cm over 1 year) | 1) Aortic root z-score > 4 or rapidly expanding (0.5 cm over 1 year) |
2) Effort made to delay surgery until the annulus reaches 1.8 cm, allowing placement of a valve sparing graft to sufficient size to accommodate growth | 2) Large size or rapid expansion of the descending aorta or other vessels | 2) Descending thoracic aorta > 5 cm or rapidly expanding (0.5 cm over 1 year) |
3) Abdominal aorta > 4 cm or rapidly expanding (0.5 cm over 1 year) | ||
4) Rapid expansion of peripheral aneurysm | ||