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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

Abstract Objective Aortic valve involvement is not uncommon in patients with Takayasu arteritis (TAK) and leading to poor prognosis. The aim of our study was to explore the risk factors of aortic valve involvement and to evaluate the prognosis in TAK patients with aortic valve involvement. Method In this retrospective study, 172 TAK patients were divided into groups with or without aortic valve involvement to identify the risk factors. Patients who underwent aortic valve surgical treatment were followed up to assess cumulative incidence of postoperative adverse events. Results A total of 92 TAK patients (53.49%) had aortic valvular lesion. The infiltration of inflammatory cells was found in surgical specimens of aortic valve. Numano type IIb, elevated high-sensitivity C-reactive protein (hs-CRP) level, and dilation of ascending aorta and aortic root were statistically associated with aortic valvular lesion in TAK patients (OR [95%CI] 6.853 [1.685–27.875], p=0.007; 4.896 [1.646–14.561], p=0.004; 4.509 [1.517–13.403], p=0.007; 9.340 [2.188–39.875], p=0.003). The 1-, 5-, and 7-year cumulative incidence of postoperative adverse events were 14.7%, 14.7%, and 31.8%, respectively. Surgical methods (p=0.024, hazard ratio (HR) 0.082) and postoperatively anti-inflammatory therapy (p=0.036, HR 0.144) were identified as potential predictors of postoperative adverse events. Conclusions Regularly echocardiogram screening is suggested in patients with Numano type IIb and aggressive treatment should be performed early in TAK patients. As for TAK patients with aortic valve surgery, aortic root replacement seems to be the preferred option and regular anti-inflammatory therapy may reduce the occurrence of adverse events of them.

Introduction: Acute type A aortic dissection (ATAAD) is a catastrophic disease with fatal outcomes. Malperfusion syndrome (MPS) is a serious complication of ATAAD, with an incidence of 20–40%. Many studies have shown that MPS is the main risk factor for poor ATAAD prognosis. However, a risk scoring system for ATAAD based on MPS is lacking. Here, we designed a risk scoring system for ATAAD to assess mortality through quantitative assessment of relevant organ malperfusion and subsequently develop rational treatment strategies. Methods and analysis: This was a prospective observational study. Patients’ perioperative clinical data were collected to establish a database of ATAAD (N≥3000) and determine whether these patients had malperfusion complications. The Anzhen risk scoring system was established on the basis of organ malperfusion by using a random forest survival model and a logistics model. The better method was then chosen to establish a revised risk scoring system. Ethics and dissemination: This study received ethical approval from the Ethics Committees of Beijing Anzhen Hospital, Capital Medical University (KS2019034-1). Patient consent was waived because biological samples were not collected, and no patient rights were violated. Findings will be disseminated at scientific conferences and in peer-reviewed publications.

Introduction: Acute type A aortic dissection (ATAAD) is a catastrophic disease with fatal outcomes. Malperfusion syndrome (MPS) is a serious complication of ATAAD, with an incidence of 20-40%. Many studies have shown that MPS is the main risk factor for poor ATAAD prognosis. However, a risk scoring system for ATAAD based on MPS is lacking. Here, we designed a risk scoring system for ATAAD to assess mortality through quantitative assessment of relevant organ malperfusion and subsequently develop rational treatment strategies. Methods and analysis: This was a prospective observational study. Patients' perioperative clinical data were collected to establish a database of ATAAD (N = 3000) and determine whether these patients had malperfusion complications. The Anzhen risk scoring system was established on the basis of organ malperfusion by using a random forest survival model and a logistics model. The better method was then chosen to establish a revised risk scoring system. Ethics and dissemination: This study received ethical approval from the Ethics Committees of Beijing Anzhen Hospital, Capital Medical University (KS2019034-1). Patient consent was waived because biological samples were not collected, and no patient rights were violated. Findings will be disseminated at scientific conferences and in peerreviewed publications. [ABSTRACT FROM AUTHOR]