Nonsyndromic familial type B aortic dissection exhibits distinct clinical profiles and operative outcomes.

Abstract

Although 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 with sporadic TBAD and Marfan syndrome (MFS)-related TBAD to determine whether FTBAD exhibits features consistent with increased underlying aortic vulnerability. This is a retrospective analysis of TBAD patients enrolled in the International Registry of Acute Aortic Dissection between 1996 and 2024. Based on standardized clinical abstraction, patients were categorized as FTBAD, sporadic TBAD, or MFS-associated TBAD. Baseline characteristics, imaging features, surgical management, in-hospital outcomes, and postdischarge events across groups using bivariate and time-to-event analyses for ≤4 years after discharge were compared. Time-to-event analysis included reintervention, rupture, and survival. Among 2726 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 years vs 64.3 years; P < .001), but were older than patients with MFS (42.4 years). Compared with patients with sporadic TBAD, patients with FTBAD 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 patients with FTBAD (60.0%) and patients with MFS (66.7%) than in patients with sporadic TBAD (49.8%) and patients with FTBAD had more extensive dissections despite a similar aortic diameter at the time of dissection compared with patients with sporadic TBAD. The indication for endovascular intervention was more frequently visceral ischemia in FTBAD compared with sporadic TBAD (23.5% vs 10.9%; P < .05). Thoracic endovascular aortic repair was less frequently performed in FTBAD compared with sporadic TBAD (23.8% vs 29.7%; P = .081), and least common in MFS (15.6%). Postdischarge data were available for 56.9% of those discharged. The 3-year incidence of late surgical intervention was 23.78% for patients with FTBAD, higher than for patients with sporadic TBAD (15.7%; P = .023). The 1-year survival exceeded 93% in all groups, and the 4-year survival exceeded 83%. Nonsyndromic FTBAD 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.