Surgical Intervention Among Heritable Thoracic Aortic Disease Parent-Child Dyads

B. D. Rhoades¹, G. E. Martinez², V. Glover², S. Y.. Green², T. Tran², S. Chatterjee², M. R. Moon², J. Coselli^2
1Baylor College of Medicine, Fulshear, Texas ²Baylor College of Medicine, Houston, Texas

Purpose: Thoracic aortic disease can affect different family members. Surgery remains the standard for heritable treatment, yet the impact of timing and extent of repair on specific dyad pairs remains unclear. Our goal is to gain insights into the experiences of parent-child dyads undergoing aortic repair.

Methods: We identified parent-child dyads with recent aortic interventions at a high-volume aortic center in the South-Central United States. To identify these pairs, we selected individuals (“secondary person”) who had at least one aortic intervention within the specified time frame (2012-2024) and indicated that a parent or adult child (“index person”) had a previous aortic repair at our institution. We included 14 pairs (n=28) of parent-child dyads who had a cumulative total of 55 aortic interventions that were performed between 1984 to 2024; of these, 17 repairs were performed elsewhere, and 38 repairs were performed within our practice. All individuals were ≥18 years at the time of in-service repair and underwent ≥1 aortic repair within our center (Table).

Results: Heritable thoracic aortic disease (HTAD) dyads primarily comprised Father-son (36%) and Mother-son (36%) dyads, where parents were most often the initial aortic intervention at our center (86%, n=12). The median age at the time of initial surgery for the index and secondary person was 42.5 years and 32 years of age, respectively; aortic root replacement was the most common initial repair (n=8, 57%) for both primary and secondary persons. Of the 14 dyads, 4/14 had at least one person in dyad documented to have Marfan syndrome at the time of repair. Regarding patient characteristics, of the repairs performed at our center (n=38), aortic dissection was present in 21% of index persons and 14% of secondary persons. Hypertension affected half (50%) of secondary persons and over one third (36%) of secondary persons presented with an acute onset of symptoms at the time of initial aortic intervention. Despite familiarity with aortic disease, only 73% of secondary persons underwent elective aortic repair. No operative deaths were identified in the HTAD parent-child dyads.

Conclusion: Staging and other parameters of surgical treatment in parent-child dyads with HTAD are imperfectly understood. After initial aortic intervention among the parent-child dyads, the secondary individual underwent repairs at a younger age; however, the expectation that all secondary persons would be better managed and undergo elective repair was not supported. More research is required to understand the challenges of hypertension management among HTAD patients, the nuances of preoperative surveillance management so as to avoid aortic dissection or symptomatic presentation, and any unnecessary delay in surgical repair.

Identify the source of the funding for this research project: This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.