Surgical Intervention Among Heritable Thoracic Aortic Disease Parent-Child Dyads.

BACKGROUND

Heritable thoracic aortic disease can affect different members of a family. Surgery remains the standard treatment for heritable thoracic aortic disease, yet the effect of timing and extent of repair on families remains unclear. We aimed to gain insight into the experience of parent-child dyads who underwent aortic repair.

METHODS

We examined aortic interventions in parent-child dyads at a high-volume aortic practice. To identify pairs, we selected individuals ("secondary") with a recent aortic intervention (2012-2024) who indicated that a parent or adult child ("index") had also undergone aortic repair at our practice. Dyads included 6 fathers (43%) and 8 mothers (57%).

RESULTS

Among 14 parent-child dyads (N = 28) who had 57 cumulative aortic interventions (1984-2024), 39 repairs (68%) were done by our practice, including 20 of 28 initial aortic operations. Secondary patients had initial surgery at a younger age (32 [interquartile range, 23-56] vs 43 [interquartile range, 30-56] years). Aortic root replacement was a common initial repair (17 of 28, 61%) for both index (8 of 14, 57%) and secondary patients (9 of 14, 64%). Aortic dissection occurred in 9 of 14 (64%) index persons and 5 of 14 (36%) secondary persons. At our center, despite familial experience with aortic disease, only 8 of 11 (73%) secondary persons underwent initial repair as an elective procedure. Of 39 within-practice repairs, none resulted in operative death.

CONCLUSIONS

Secondary persons with heritable thoracic aortic disease may benefit from family experience (eg, earlier diagnosis and clinical monitoring) but do not necessarily avoid nonelective repair or dissection. Additional research is needed to aid surgical decision-making from a patient's perspective.