Non-Syndromic Familial Type B Aortic Dissection Exhibits Distinct Clinical Profiles and Operative Outcomes.

Abstract

Background: While a heritable nature of type A aortic dissection is well established, the contribution of family history to type B aortic dissection (TBAD) remains less clearly characterized. This analysis evaluates the prevalence, clinical characteristics, and outcomes of familial TBAD (FTBAD) compared to sporadic TBAD and Marfan syndrome (MFS)-related TBAD to determine whether familial TBAD exhibits features consistent with increased underlying aortic vulnerability.

Methods: This is a retrospective analysis of TBAD patients enrolled in the International Registry of Acute Aortic Dissection (IRAD) between 1996 and 2024. Based on standardized clinical abstraction, patients were categorized as familial TBAD (FTBAD), sporadic TBAD, or MFS-associated TBAD. Baseline characteristics, imaging features, surgical management, in-hospital outcomes, and post-discharge events across groups using bivariate and time-to-event analyses for up to 4-years post discharge were compared. Time-to-event analysis included re-intervention, rupture, and survival.

Results: Among 2,726 patients (mean age 63.0±14.6 years; 64.2% male), 223 (8.2%) had FTBAD and 128 (4.7%) had MFS. Patients with FTBAD presented at a younger age than those with sporadic TBAD (60.8 vs. 64.3 years, p<.001) but older than patients with MFS (42.4 years). ompared with sporadic TBAD, FTBAD patients were more frequently normotensive at presentation and had higher rates of prior aortic dissection and prior cardiac or aortic surgery.Patent false lumen was more frequent in FTBAD (60.0%) and MFS (66.7%) compared to sporadic TBAD (49.8%) and FTBAD patients had more extensive dissections despite a similar aortic diameter at the time of dissection compared to sporadic TBAD. The indication for endovascular intervention was more frequently visceral ischemia in FTBAD compared to sporadic TBAD (23.5 vs. 10.9%, p<0.05). TEVAR was less frequently performed in FTBAD compared to sporadic TBAD (23.8% vs. 29.7%, p=0.081), and least common in MFS (15.6%). Post-discharge data were available for 56.9% of those discharged. Three-year incidence of late surgical intervention was 23.78% for FTBAD patients, higher than sporadic TBAD (15.7%, p=0.023). One-year survival exceeded 93% in all groups; four-year survival exceeded 83%.

Conclusions: Non-syndromic familial TBAD is present in a meaningful proportion of patients and is associated with distinct clinical features consistent with increased underlying aortic vulnerability compared with sporadic TBAD. Systematic assessment of family history may improve risk stratification, longitudinal surveillance, and patient shared decision-making in the management of TBAD.