Valve-Sparing Aortic Root Replacement in an 8-Month-Old Infant With Loeys-Dietz Syndrome

Aortic root aneurysm repair in a neonate with Loeys–Dietz syndrome

“…It is exceedingly rare for LDS to present for cardiovascular intervention in early infancy, with only a handful of cases reported in the literature. 6,7 We believe this to be the first case of an infant with LDS requiring aortic aneurysm repair in the neonatal period. In this case, the presenting pathophysiology was particularly impressive, resembling that of tricuspid atresia with restrictive pulmonary blood flow.…”

“…Although patients with LDS typically present with cardiovascular incidents starting in their teenage years, aneurysm rupture and dissection have been reported in infants as young as 3-6 months with aortic diameters less than 4.0 cm. [5][6][7] Due to the disease's malignant phenotype and high mortality, prophylactic surgical intervention is recommended at smaller aortic diameters compared to patients with other CTDs. 2,4,5 Studies have shown favourable outcomes in LDS patients undergoing surgical intervention in childhood with low morbidity and mortality.…”

“…1,3 Compared to other CTDs, LDS presents in a more aggressive manner and is associated with earlier morbidity and mortality due to the earlier occurrence of catastrophic cardiovascular events 3,4 Aortic dissections and aneurysms have been reported in patients as young as 3-6 months of age, with surgical intervention often required. [5][6][7]…”