Early outcomes of patients with Marfan syndrome and acute aortic type A dissection.

BACKGROUND: Acute aortic Stanford type A dissection remains a frequent and life-limiting event for patients with Marfan syndrome. Outcome results in this high-risk group are limited. METHODS: The German Registry for Acute Aortic Dissection Type A collected the data of 56 centers between July 2006 and June 2015. Of 3385 patients undergoing operations for acute aortic Stanford type A dissection, 117 (3.5%) were diagnosed with Marfan syndrome. We performed a propensity score match comparing patients with Marfan syndrome with patients without Marfan syndrome in a 1:2 fashion. RESULTS: Patients with Marfan syndrome were significantly younger (42.9 vs 62.2 years; P < .001), predominantly male (76.9% vs 62.9%; P = .002), and less catecholamine dependent (9.4% vs 20.3%; P = .002) compared with the unmatched cohort. They presented with aortic regurgitation (41.6% vs 23.0%; P < .001) and involvement of the supra-aortic vessels (50.4% vs 39.5%; P = .017) more often. Propensity matching revealed 82 patients with Marfan syndrome (21 female) with no significant differences in baseline characteristics compared with patients without Marfan syndrome (n = 159, 36 female; P = .607). Although root preservation was more frequent in patients with Marfan syndrome, procedure types did not differ significantly (18.3% vs 10.7%; P = .256). Aortic arch surgery was performed more frequently in matched patients (87.5% vs 97.8%; P = .014). Thirty-day mortality did not differ between patients with and without Marfan syndrome (19.5% vs 20.1%; P = .910). Multivariate regression showed no influence of Marfan syndrome on 30-day mortality (odds ratio, 0.928; 95% confidence interval, 0.346-2.332; P = .876). CONCLUSIONS: Marfan syndrome does not adversely affect 30-day outcomes after surgical repair for acute aortic Stanford type A dissection compared with a matched cohort. Long-term outcome analysis is needed to account for the influence of further downstream interventions.

Prediction of mortality rate in acute type A dissection: the German Registry for Acute Type A Aortic Dissection score.

OBJECTIVES: The goal was to develop a scoring system to predict the 30-day mortality rate for patients undergoing surgery for acute type A aortic dissection on the basis of the German Registry for Acute Type A Aortic Dissection (GERAADA) data set and to provide a Web-based application for standard use. METHODS: A total of 2537 patients enrolled in GERAADA who underwent surgery between 2006 and 2015 were analysed. Variable selection was performed using the R-package FAMoS. The robustness of the results was confirmed via the bootstrap procedure. The coefficients of the final model were used to calculate the risk score in a Web-based application. RESULTS: Age [odds ratio (OR) 1.018, 95% confidence interval (CI) 1.009-1.026; P < 0.001; 5-year OR: 1.093], need for catecholamines at referral (OR 1.732, 95% CI 1.340-2.232; P < 0.001), preoperative resuscitation (OR 3.051, 95% CI 2.099-4.441; P < 0.001), need for intubation before surgery (OR 1.949, 95% CI 1.465-2.585; P < 0.001), preoperative hemiparesis (OR 1.442, 95% CI 0.996-2.065; P = 0.049), coronary malperfusion (OR 1.870, 95% CI 1.386-2.509; P < 0.001), visceral malperfusion (OR 1.748, 95% CI 1.198-2.530; P = 0.003), dissection extension to the descending aorta (OR 1.443, 95% CI 1.120-1.864; P = 0.005) and previous cardiac surgery (OR 1.772, 95% CI 1.048-2.903; P = 0.027) were independent predictors of the 30-day mortality rate. The Web application based on the final model can be found at https://www.dgthg.de/de/GERAADA_Score. CONCLUSIONS: The GERAADA score is a simple, effective tool to predict the 30-day mortality rate for patients undergoing surgery for acute type A aortic dissection. We recommend the widespread use of this Web-based application for standard use.