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Objectives: The aim of this study was to determine the outcome of Stanford type B aortic dissection in patients with Marfan syndrome (MFS) and to evaluate aortic diameters at time of dissection as well as the impact of previous aortic root replacement., Methods: Analysis of all patients with MFS fulfilling Ghent criteria seen at this institution since 1995 until 2022., Results: Thirty-six (19%) out of 188 patients with MFS suffered from Stanford type B aortic dissection during the study period. The Mean aortic diameter at the time of dissection was 39.0 mm [95% confidence interval (CI): 35.6-42.3]. The mean pre-dissection diameter (available in 25% of patients) was 32.1 mm (95% CI: 28.0-36.3) and the mean expansion was 19% (95% CI: 11.9-26.2). There was no correlation between age and diameter at the time of dissection (<20, 21-30, 31-40, 41-50, 51-60, <61 years; P = 0.78). Freedom from intervention after dissection was 53%, 44% and 33% at 1, 5 and 10 years. Aortic growth rate in those patients that had to undergo intervention within the 1st year after dissection was 10.2 mm/year (95% CI: 4.4-15.9) compared to 5.8 mm/year (95% CI: 3.3-8.3, P = 0.109) in those thereafter. The mean time between dissection and intervention was 1.8 years (95% CI: 0.6-3.0). While type B dissection seems more frequent after previous elective aortic repair (58% vs 42%), there was no difference between valve-sparing root replacement (VSRR) compared to Bentall procedures [Hazard ratio (HR) for VSRR 0.78, 95% CI: 0.31-2.0, P-value = 0.61]. The mean age of the entire population at the end of follow-up was 42 years (95% CI: 39.2-44.7). The mean follow-up time was 9 years (95% CI: 7.8-10.4)., Conclusions: Stanford type B dissection in patients with MFS occurs far below accepted thresholds for intervention. Risk for type B dissection is present throughout lifetime and two-thirds of patients need an intervention after dissection. There is no difference in freedom from type B dissection between a Bentall procedure and a VSRR., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The prevalence and aetiology of acute aortic dissection type A (AADA) in patients ≤30 years is unknown. The aims of this clinical study were to determine the prevalence and potential aetiology of AADA in surgically treated patients ≤30 years and to evaluate the respective postoperative outcomes in this selective group of patients in a large multicentre study., Methods: Retrospective data collection was performed at 16 participating international aortic institutions. All patients ≤30 years at the time of dissection onset were included. The postoperative results were analysed with regard to connective tissue disease (CTD)., Results: The overall prevalence of AADA ≤30 years was 1.8% (139 out of 7914 patients), including 51 (36.7%) patients who were retrospectively diagnosed with CTD. Cumulative postoperative mortality was 8.6%, 2.2% and 1.4%. Actuarial survival was 80% at 10 years postoperatively. Non-CTD patients (n = 88) had a significantly higher incidence of arterial hypertension (46.6% vs 9.8%; P < 0.001) while AADA affected the aortic root (P < 0.001) and arch (P = 0.029) significantly more often in the CTD group. A positive family history of aortic disease was present in 9.4% of the study cohort (n = 13)., Conclusions: The prevalence of AADA in surgically treated patients ≤30 years is <2% with CTD and arterial hypertension as the 2 most prevalent triggers of AADA. Open surgery may be performed with good early results and excellent mid- to long-term outcomes., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The aim of this study was to explore sex and gender differences regarding aortic events in Marfan patients., Methods: We analysed all data from our connective tissue disorder database. Only patients with Marfan syndrome were included. For analysis, patients were divided by sex. Female patients were further divided into 2 subgroups: with versus without children. Aortic events were defined as Stanford type A aortic dissection (TAAD) or type B aortic dissection (TBAD) or any aortic intervention., Results: A population of 183 Marfan patients was analysed for the purpose of this study. One hundred four (57%) were male and 79 (43%) were female patients. Thirty-seven (47%) of the 79 female patients had at least 1 child. Male patients had a significantly higher probability of experiencing an aortic event (P = 0.015) compared to female patients. However, there was no increased probability for recurrent events in male patients compared to female patients (P = 0.063). Follow-up revealed no sex and gender differences in the occurrence of Stanford TAAD or TBAD between male and female patients (P = 0.324/P = 0.534). While 11% of women with children suffered from peripartum aortic events, 24% experienced Stanford TAAD unrelated to pregnancy., Conclusions: Male patients have a higher risk of aortic events than female patients. The majority of women were not aware of their Marfan syndrome diagnosis before conceiving. One out of 10 women suffered from peripartum Stanford TAAD or TBAD. Twice as many female patients with children suffered from aortic dissection unrelated to childbirth. There were no sex and gender differences affecting mortality in Marfan patients., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Open in new tab Download slide OBJECTIVES The aim of this study was to report on mid-term outcomes after endovascular aortic repair (EVAR) in patients with Marfan (MFS) or Loeys–Dietz (LDS) syndrome. METHODS We analysed data from 2 European centres of patients with MFS and LDS undergoing EVAR. Patients were analysed based on (i) timing of the procedure (planned versus emergency procedure) and (ii) the nature of the landing zone (safe versus non-safe). The primary end-point was freedom from reintervention. Secondary end-points were freedom from stroke, bleeding and death. RESULTS A population of 419 patients with MFS (n  = 352) or LDS (n  = 67) was analysed for the purpose of this study. Thirty-nine patients (9%) underwent EVAR. Indications for thoracic endovascular aortic repair or EVAR were aortic dissection in 13 (33%) patients, aortic aneurysm in 22 (57%) patients and others (intercostal patch aneurysm, penetrating atherosclerotic ulcer, pseudoaneurysm, kinking of frozen elephant trunk (FET)) in 4 (10%) patients. Thoracic endovascular repair was performed in 34 patients, and abdominal endovascular aortic repair was performed in 5 patients. Mean age at 1st thoracic endovascular aortic repair/EVAR was 48.5 ± 15.4 years. Mean follow-up after 1st thoracic endovascular aortic repair/EVAR was 5.9 ± 4.4 years. There was no statistically significant difference in the rate of reinterventions between patients with non-safe landing zone and the patients with safe proximal landing zone (P  =   0.609). Furthermore, there was no increased probability for reintervention after planned endovascular intervention compared to emergency procedures (P  =   0.916). Mean time to reintervention, either open surgical or endovascular, after planned endovascular intervention was in median 3.9 years (95% confidence interval 2.0–5.9 years) and 2.0 years (95% confidence interval –1.1 to 5.1 years) (P  =   0.23) after emergency procedures. CONCLUSIONS EVAR in patients with MFS and LDS and a safe landing zone is feasible and safe. Endovascular treatment is a viable option when employed by a multi-disciplinary aortic team even if the landing zone is in native tissue. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The aim of this study was to explore sex and gender differences regarding aortic events in Marfan patients. METHODS We analysed all data from our connective tissue disorder database. Only patients with Marfan syndrome were included. For analysis, patients were divided by sex. Female patients were further divided into 2 subgroups: with versus without children. Aortic events were defined as Stanford type A aortic dissection (TAAD) or type B aortic dissection (TBAD) or any aortic intervention. RESULTS A population of 183 Marfan patients was analysed for the purpose of this study. One hundred four (57%) were male and 79 (43%) were female patients. Thirty-seven (47%) of the 79 female patients had at least 1 child. Male patients had a significantly higher probability of experiencing an aortic event (P  = 0.015) compared to female patients. However, there was no increased probability for recurrent events in male patients compared to female patients (P  = 0.063). Follow-up revealed no sex and gender differences in the occurrence of Stanford TAAD or TBAD between male and female patients (P  = 0.324/ P  = 0.534). While 11% of women with children suffered from peripartum aortic events, 24% experienced Stanford TAAD unrelated to pregnancy. CONCLUSIONS Male patients have a higher risk of aortic events than female patients. The majority of women were not aware of their Marfan syndrome diagnosis before conceiving. One out of 10 women suffered from peripartum Stanford TAAD or TBAD. Twice as many female patients with children suffered from aortic dissection unrelated to childbirth. There were no sex and gender differences affecting mortality in Marfan patients. [ABSTRACT FROM AUTHOR]

OBJECTIVES Open in new tab Download slide Open in new tab Download slide The goal of this study was to report the long-term outcomes of patients with Marfan syndrome who had aortic surgery on any aortic segment except for the replacement of the aortic root itself. METHODS An observational retrospective single-centre study was conducted with 115 Marfan syndrome patients who underwent 189 major aortic interventions from 1995 until 2018. Patients without aortic root replacement were identified and aortic root growth was analysed over time. RESULTS Eleven of 115 patients (9.5%) did not have aortic root replacement during a follow-up of 10.5 [standard deviation (SD) 5.7] years and a mean age at last follow-up of 53.9 (SD 13.4) years. Patients without root replacement did not suffer less frequently from any type of acute aortic dissection (type A 27% vs 25%, P  = 0.999; type B 36% vs 25%, P  = 0.474). Patients with native aortic roots did not undergo fewer aortic interventions than those with aortic root replacement [12/11, mean 1.09 (SD 0.54) operations/patient vs 177/104, mean 1.7 (SD 1.3); P  = 0.128]. Progression of the aortic root dimension was 0.5 (SD 0.3) mm/year in the group of patients with native aortic roots. CONCLUSIONS Current data suggest that 10% of patients with Marfan syndrome with previous aortic surgery will be free from aortic root replacement until the sixth decade of life. [ABSTRACT FROM AUTHOR]