Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Zhu, Yu; Jian, Zhao; Ma, Ronghua; Wang, Yong; Xiao, Yingbin

doi:10.21037/jtd-21-577

Cited by 3 publications

(2 citation statements)

References 24 publications

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“…A total of 79,931 records were collected via online database review, out of which 7345 studies were eligible for abstract screening. After applying the exclusion criteria, 109 records were adopted for full‐text screening, and eventually, reported data of 43 studies including 5068 patients who underwent TAR with FET were reviewed 4,15–56 (Figure 1).…”

Section: Resultsmentioning

confidence: 99%

Aortic remodeling, distal stent‐graft induced new entry and endoleak following frozen elephant trunk: A systematic review and meta‐analysis

Nakhaei

Bashir

Jubouri

et al. 2022

Journal of Cardiac Surgery

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Background The introduction of the frozen elephant trunk (FET) technique for total arch replacement (TAR) has revolutionized the field of aortovascular surgery. However, although FET yields excellent results, the risk of certain complications requiring secondary intervention remains present, negating its one‐step hybrid advantage over conventional techniques. This systematic review and meta‐analysis sought to evaluate controversies regarding the incidence of FET‐related complications, with a focus on aortic remodeling, distal stent‐graft induced new entry (dSINE) and endoleak, in patients with type A aortic dissection (TAAD) and/or thoracic aortic aneurysm. Materials and Methods A comprehensive literature search was conducted using multiple electronic databases including EMBASE, Scopus, and PubMed/MEDLINE to identify evidence on TAR with FET in patients with TAAD and/or aneurysm. Studies published up until January 2022 were included, and after applying exclusion criteria, a total of 43 studies were extracted. Results A total of 5068 patients who underwent FET procedure were included. The pooled estimates of dSINE and endoleak were 2% (95% confidence interval [CI] 0.01–0.06, I2 = 78%) and 3% (95% CI 0.01–0.11, I2 = 89%), respectively. The pooled rate of secondary thoracic endovascular aortic repair (TEVAR) post‐FET was 7% (95% CI 0.05–0.12, I2 = 89%) while the pooled rate of false lumen thrombosis at the level of stent‐graft was 91% (95% CI 0.75–0.97, I2 = 92%). After subgroup analysis, heterogeneity for distal stent‐graft induced new entry (dSINE) and endoleak resolved among European patients, where Thoraflex Hybrid (THP) and E‐Vita stent‐grafts were used (both I2 = 0%). In addition, heterogeneity for secondary TEVAR after FET resolved among Asians receiving Cronus (I2 = 15.1%) and Frozenix stent‐grafts (I2 = 1%). Conclusion Our results showed that the FET procedure in patients with TAAD and/or aneurysm is associated with excellent results, with a particularly low incidence of dSINE and endoleak as well as highly favorable aortic remodeling. However the type of stent‐graft and the study location were sources of heterogeneity, emphasizing the need for multicenter studies directly comparing FET grafts. Finally, THP can be considered the primary FET device choice due to its superior results.

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Section: Resultsmentioning

confidence: 99%

Aortic remodeling, distal stent‐graft induced new entry and endoleak following frozen elephant trunk: A systematic review and meta‐analysis

Nakhaei

Bashir

Jubouri

et al. 2022

Journal of Cardiac Surgery

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“…However, with increasing survival time, descending aortic dilation or primary type B aortic dissection may occur in MFS patients after aortic root surgery (1,5,6). Aortic dissection and previous root replacement have been recognized as potential risk factors for lesions on the distal aorta in MFS (5, [7][8][9][10][11]. Some recent studies have also described aortic aneurysm without dissection and peripheral arterial aneurysms in MFS (12)(13)(14).…”

Section: Introductionmentioning

confidence: 99%

Fate of the distal aorta following root replacement in Marfan syndrome: a propensity score matched study

Líu

Chen

Luo

et al. 2023

Front. Cardiovasc. Med.

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ObjectiveThe aortic root is the most frequent segment involved in Marfan syndrome. However, Marfan syndrome is a systemic hereditary connective tissue disorder, and knowledge regarding the outcomes of the native distal aorta after prophylactic aortic root surgery is limited.MethodsFrom April 2010 to December 2020, 226 patients with Marfan syndrome and 1,200 patients without Marfan syndrome who underwent Bentall procedures were included in this study. By propensity score matching, 134 patients were assigned to each group. Clinical manifestations and follow-up data were acquired from hospital records and telephone contact. The cumulative incidence of aortic events was estimated in Marfan and non-Marfan patients with death as a competing risk.ResultsPatients with and without Marfan syndrome had similar baseline characteristics after propensity score matching. Differences in the aortic root (62.25 ± 11.96 vs. 54.03 ± 13.76, P < .001) and ascending aorta (37.71 ± 9.86 vs. 48.16 ± 16.01, P < .001) remained after matching. No difference was observed in the frequency of aortic adverse events between the two groups (10.5% vs. 4.6%, P = 0.106). The cumulative incidence of aortic events was not different between Marfan and non-Marfan patients (15.03% ± 4.72% vs. 4.18% ± 2.06%, P = 0.147). Multivariate Cox regression indicated no significant impact of Marfan syndrome on distal aortic events (HR: 1.172, 95% CI: 0.263–5.230, P = 0.835). Descending and abdominal aortic diameter above normal at the initial procedure were associated with the risk of distal aortic events (HR: 20.735, P = .003, HR: 22.981, P = .002, respectively).ConclusionsNew-onset events of the residual aorta in patients undergoing Bentall procedures between the Marfan and non-Marfan groups were not significantly different. Distal aortic diameter above normal at initial surgery was associated with a higher risk of adverse aortic events.

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Right ventricular function in Marfan patients remains stable despite multiple cardiac interventions

Mihalj,

Nucera,

Ferro

et al. 2024

European Journal of Cardio-Thoracic Surgery

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OBJECTIVES Mitral valve regurgitation and left ventricular dysfunction are cardiovascular symptoms of Marfan syndrome. There is a paucity of information on tricuspid valve regurgitation and right ventricular function. In patients with Marfan syndrome, we looked at long-term changes in right ventricular function, tricuspid valve regurgitation, and freedom from tricuspid valve repair. METHODS Retrospective-observational single-centre analysis on right ventricular function and tricuspid regurgitation in Marfan patients who underwent surgery with cardioplegic arrest between 1995 and 2020. Patients were followed-up from first operation until death, with echocardiographic changes analysed longitudinally. Composite end-point was TAPSE ≤ 16mm, severe tricuspid regurgitation, or tricuspid repair. RESULTS The study included 135 patients who underwent 193 operations, 58 of those were reoperations in 40 patients. Median age at first operation was 35 years (IQR 26–46), median follow-up was 8.0 years (IQR 3.0–16.0), and median time-to-first-reoperation was 7.5 years (IQR 3.4–12.5). The composite end-point occurred in 81 observations in 40 patients, mostly as a recurrent event, after median 7.0 years (IQR 1.0–13.0). 10-year-cumulative-incidence for composite end-point was 22.0% (95% CI 15–31), and 9.0% (95% CI 4.4–16) for new-onset TAPSE ≤ 16mm, but no significant change in TAPSE was observed at 10 years. Tricuspid regurgitation was associated with increased risk of annual progression (P < 0.001), but not clinically relevant at 10 years. Actuarial 10-year-survival was 91.1%. CONCLUSIONS In Marfan patients with a history of cardiac surgery and subsequent reoperations, the right-ventricular function remains stable. The incidence of severe tricuspid regurgitation and tricuspid repair remain low.

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Long-term outcomes after aortic root replacement for patients with Marfan syndrome

Cited by 3 publications

References 24 publications

Aortic remodeling, distal stent‐graft induced new entry and endoleak following frozen elephant trunk: A systematic review and meta‐analysis

Aortic remodeling, distal stent‐graft induced new entry and endoleak following frozen elephant trunk: A systematic review and meta‐analysis

Fate of the distal aorta following root replacement in Marfan syndrome: a propensity score matched study

Right ventricular function in Marfan patients remains stable despite multiple cardiac interventions

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