Background-The aim of the current study was to investigate incidence and causes of surgical interventions in primarily nontreated aortic segments after previous aortic repair in patients with Marfan syndrome. Methods and Results-Retrospective analysis of 86 consecutive Marfan syndrome patients fulfilling Ghent criteria that underwent 136 aortic surgeries and were followed at this institution in the past 15 years. Mean follow-up was 8.8±6.8 y.Thirty-day, 6-month, 1-year, and overall mortality was 3.5%, 5.8%, 7.0%, and 12.8%, respectively. Ninety-two percent of patients initially presented with aortic root, ascending aortic or arch lesions, whereas 8% presented with descending aortic or thoraco-abdominal lesions. Primary presentation was acute aortic dissection (AAD) in 36% (77% type A, 23% type B) and aneurismal disease in 64%. Secondary complete arch replacement had to be performed in only 6% of patients without AAD, but in 36% with AAD (P=0.0005). In patients without AAD, 11% required surgery on primarily nontreated aortic segments (5 of 6 patients experienced type B dissection during follow-up), whereas in patients after AAD, 48% underwent surgery of initially nontreated aortic segments (42% of patients with type A and 86% of those with type B dissection; P=0.0002). Conclusions-The need for surgery in primarily nontreated aortic segments is precipitated by an initial presentation with AAD. Early elective surgery is associated with low mortality and reintervention rates. Type B dissection in patients with Marfan syndrome is associated with a high need for extensive aortic repair, even if the dissection is being considered uncomplicated by conventional criteria. (Circulation. 2013;127:1569-1575.)

MFS patients undergoing elective root repair have small risk of reinterventions on the aortic arch, and primary prophylactic replacement does not seem to be justified. In patients with AAD, the need for reinterventions is precipitated by the dissection itself and not by limiting the procedure to the hemi-arch replacement in the emergency setting. Limiting surgery to the aortic root, ascending aorta and proximal aortic arch is associated with low mortality in MFS patients presenting with AAD.

Gene-targeted therapy with the inotropic Ca2 + -sensor protein S100A1 rescues contractile function in post-ischemic heart failure and is being developed towards clinical trials. Its proven beneficial effect on cardiac metabolism and mitochondrial function suggests a cardioprotective effect of S100A1 in myocardial ischemia-reperfusion injury (IRI). Fivefold cardiomyocyte-specific S100A1 overexpressing, isolated rat hearts perfused in working mode were subjected to 28 min ischemia (37 °C) followed by 60 min reperfusion. S100A1 overexpressing hearts showed superior hemodynamic recover: Left ventricular pressure recovered to 57 ± 7.3% of baseline compared to 51 ± 4.6% in control (p = 0.025), this effect mirrored in LV work and dP/dt(max). Troponin T and lactate dehydrogenase was decreased in the S100A1 group, as well as FoxO pro-apoptotic transcription factor, indicating less tissue necrosis, whereas phosphocreatine content was higher after reperfusion. This is the first report of a cardioprotective effect of S100A1 overexpression in a global IRI model.

Objectives: The aim of this study was to investigate the fate of nonaortic arterial segments in patients with Marfan syndrome (MFS).Methods: This was a retrospective analysis of 100 consecutive patients with MFS fulfilling Ghent criteria who underwent 192 interventions on any segment of the arterial tree and were followed over the past 20 years. A review of the available imaging regarding 9 defined regions of interest of the carotid, innominate, subclavian, iliac, and femoral arteries was performed.Results: Mean follow-up interval was 11.6 AE 7.7 years. Of 600 measurements that were performed, 414 (69%) arterial segments showed dilatation above the upper range of normal. There were no significant sex differences. In 100 patients, 66 dissections in nonaortic arterial segments in 33 patients were identified. Nineteen patients with or without previous dissection underwent 34 interventions. Most interventions were performed on the iliac arteries (56%), followed by the subclavian arteries (21%), the intercostal arteries (9%), the carotid arteries (6%), the visceral arteries (6%), and the innominate artery (3%). Most iliac artery interventions (88%) were caused by dilatations due to previous dissections, whereas this was only the case in 17% of interventions on the subclavian arteries.Conclusions: Most patients with MFS presented with at least 2 dilated nonaortic arterial segments. The current data suggest that 20% of MFS patients will need some form of intervention on nonaortic arterial segments 5 to 6 years after their first aortic intervention, referring to the first aortic dissection of the patient if the patient had a history of dissection. Routine long-term follow-up imaging should include the iliac arteries as well as the supra-aortic branches.