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Patients with severe mitral regurgitation (MR) frequently present with concomitant right ventricular (RV) dysfunction and tricuspid regurgitation (TR). We aimed to investigate the prognostic relevance of RV function, RV dimension, and TR in patients undergoing percutaneous intervention for MR.Consecutive patients undergoing percutaneous mitral valve intervention were enrolled in the prospective MitraSwiss registry. Tricuspid annular plane systolic excursion (TAPSE), pulmonary artery systolic pressure (PASP), right ventricular pulmonary arterial coupling (RVC, defined as TAPSE/ PASP ratio), indexed tricuspid annulus (TA) dimension, and TR severity grade were analyzed at baseline, post procedure, and at 6-month follow-up. The endpoints of all-cause mortality, hospitalization for heart failure, and the combined endpoint of the 2 were observed during long-term follow-up (up to 4 years).We analyzed 218 patients (mean age, 76 ± 9 years; 36% female). Edge-to-edge mitral valve repair resulted in an increase in TAPSE and RVC ratio and a decrease in indexed TA and PASP, but concomitant TR did not change significantly. In multivariable analysis, RV dysfunction and moderate/severe TR were independently associated with increased all-cause mortality (hazard ratio, 1.61; 95% confidence interval, 1.05-2.46; P=.03 and hazard ratio, 2.10; 95% confidence interval, 1.34-3.29; P.01, respectively) and moderate/severe TR was further an independent predictor for hospitalization for heart failure and for the combined endpoint.Treatment of MR resulted in favorable changes of RV function and dimension but did not reduce TR in the majority of patients. TR at baseline remained the strongest predictor for outcomes, outperforming parameters of RV function and dimension.

Die Poststernotomie-Mediastinitis (PSM) ist eine seltene, aber ernste Komplikation nach einem herzchirurgischen Eingriff, die mit einer erhohten Letalitat assoziiert ist. Es handelt sich um ein vielgestaltiges und multifaktorielles Krankheitsbild, dessen Behandlung haufig komplex und zeitaufwendig ist und mehrere Disziplinen einbindet. Zu Diagnostik und Therapie existiert kein einheitliches Vorgehen. In der Literatur wird eine Vielzahl chirurgischer Techniken beschrieben. Die Auswahl einer geeigneten chirurgischen Therapiestrategie ist haufig patientenindividuell zu treffen. Dennoch sollte sie evidenz- und konsensbasiert erfolgen. Der vorliegende Beitrag fasst die aktuellen Empfehlungen zu Diagnostik und Therapie der PSM zusammen, die auf der kurzlich publizierten S3-Leitlinie „Management der Mediastinitis nach herzchirurgischem Eingriff“ beruhen.

Investigate the impact of concomitant tricuspid regurgitation (TR) on clinical outcomes during long-term follow-up in patients undergoing percutaneous treatment of mitral regurgitation (MR) with the MitraClip system.Patients undergoing mitral repair using the MitraClip frequently present with concomitant TR. It is uncertain how the presence of TR impacts the long-term outcomes of such patients.We analyzed consecutive patients with MitraClip implantation from the prospective MitraSwiss registry. Endpoints were all-cause mortality, hospitalization for heart failure, and the composite endpoint of the two.We enrolled 177 patients (mean age 76 ± 9 years, 37% female). Acute procedural success was achieved in 149 (84%). Concomitant moderate or severe TR was present in 31% at baseline and 32% before discharge. After a median follow-up of 1103 days (IQR: 555-1766 days), 70 (40%) of patients had died, and 34 (19%) were hospitalized for heart failure. In multivariable analysis, TR at baseline was associated with an increase in all-cause mortality (HR: 2.34, 95% CI: 1.36-4.03, p 0.01), hospitalization for heart failure (HR: 3.19, 95% CI: 1.37-7.41, p = 0.01), and the composite endpoint (HR: 2.00, 95% CI: 1.19-3.36, p = 0.01).Despite treatment of MR, TR did not improve in most patients. The presence of relevant TR at baseline was associated with reduced survival and higher rates of hospitalization for heart failure. More research is needed to understand the causal role of TR in such patients and to investigate if simultaneous treatment of concomitant TR may improve prognosis in patients undergoing percutaneous treatment of MR.

Background In isolated mitral valve regurgitation general consensus on surgery is to favor repair over replacement excluding rheumatic etiology or endocarditis. If concomitant aortic valve replacement is performed however, clinical evidence is more ambiguous and no explicit guidelines exist on the choice of mitral valve treatment. Both, double valve replacement (DVR) and aortic valve replacement in combination with concomitant mitral valve repair (AVR + MVP) have been proven to be feasible procedures. In our single-center, retrospective, observational cohort study, we compared the outcome of these two surgical techniques focusing on mortality and morbidity. Methods 89 patients underwent DVR (n = 41) or AVR + MVP (n = 48) in our institution between 2009 and 2018. Follow-up data was collected using electronic patient records, by contacting treating physicians and by telephone interviews. We used the Kaplan–Meier method to analyze mortality during follow-up and Cox regression to investigate potential predictors of mortality. Results During a median follow-up duration of 4.5 [IQR 2.9 to 6.1] years, there was no significant difference in mortality between both cohorts. Thirty days mortality was 6.3% in the DVR and 7% in the AVR + MVP cohort. Overall mortality amounted to 17% for DVR and 23% for AVR + MVP. DVR was the preferred procedure for valve disease of rheumatic etiology and for endocarditis, while in degenerative valves AVR + MVP was predominant. More biological valves were used in the AVR + MVP cohort (p Conclusion Our data analysis showed that both DVR and AVR + MVP are safe and feasible options for double valve surgery. Based on our findings we could not prove superiority of one surgical technique over the other. Choosing the appropriate procedure for the patient should be influenced by valve etiology, patients’ comorbidities and the surgeons’ experience. Trial registration This was a retrospectively registered trial, registered on April 1st 2018, ClinicalTrials.gov Identifier: NCT03667274.

The Cross-Cutting zone of the Fowler ore body is one of the many ore bodies of the Balmat, NY massive sulfide zinc deposit that underwent remobilization during upper amphibolite metamorphism and associated deformation. The kilometer-scale remobilization is difficult to reconcile with purely solid-state processes. Given that other polymetallic ores of similar metamorphic grade have been shown to have undergone varying degrees of anatexis, SEM-EDS micro-petrographic analysis of ores was undertaken to determine if melts were present in Balmat ores during metamorphism and deformation. Low melting temperature micro-inclusions of sulfosalt and sulfide assemblages occur predominantly in Qtz-Py and Kfs-Py domains that cross-cut peak metamorphic assemblages. These results indicate that localized anatexis occurred on the prograde path, producing low volumes of melts of varying composition. Polymetallic sulfide melts were initiated by the prograde breakdown of minor phases containing low melting temperature chalcophile elements including As, Sb, Pb, and Cu. Alkaline silicate ± carbonate ± sulfide melts were fluxed by halogens, sulfur and other volatile components, released during metamorphism of evaporitic and organic-rich horizons in the marble-dominated host sequence. These low-volume melts and residual fluids would have wetted grain boundaries, affecting the rheology of the bulk ore, and facilitating remobilization of the ores through a combination of solid- and liquid-state processes.

OBJECTIVES Technical skills are an essential component of cardiac surgery, and the operating room is becoming an even more challenging environment for trainees who want to acquire such skills. Simulation, which partially overcomes this limitation, represents a valid adjunct to surgical education. We describe an original simulator and provide results in terms of trainees’ evaluations and ratings. METHODS We used a humanoid that is a silicone replica of the chest of an adult human that provides a complete anatomical platform for hands-on skin-to-skin practice of surgical techniques in arrested heart coronary artery bypass graft (CABG) surgery cases. Learners were residents in cardiac surgery. The teaching sessions included 2 full three-vessel CABG procedures using both mammary arteries and a hydrogel vein. Five board-certified cardiovascular surgeons scored the surgical activity of all trainees. The trainees were asked to complete an exit questionnaire to evaluate their course. RESULTS Overall, 16 residents participated in the simulation, including 5 women and 11 men, with a mean age of 30 ± 4 years, all of whom had at least 2 years of cardiac surgery training. All participants completed the 2 CABG operations. Three mammary arteries (4.6%) were seriously damaged during harvesting. In 1 case (3.1%), an aortic tear occurred during aortic cannulation. Each trainee performed overall 6 distal and 2 proximal coronary anastomoses. All participants agreed that the ‘humanoid reproduces real-life situations, the feeling is realistic, and they are now more confident in performing coronary anastomosis’. CONCLUSIONS Trainees involved in this simulation curriculum acquired and refined technical skills that could be applied directly to human patients. In addition, we were able to foster a higher level of teamwork within the operating room team., Technical skills are an essential component of cardiac surgery.

Background/Introduction MIDCAB (minimally invasive direct coronary artery bypass surgery) ± PCI/DES was compared to classical “off pump” coronary artery bypass surgery (OPCAB) for the treatment of severe coronary artery disease. Purpose We hypothesized that MIDCAB is associated with reduced perioperative morbidity and mortality. Methods Preoperative and postoperative clinical data were collected prospectively in 329 consecutive patients with severe coronary artery disease undergoing either a MIDCAB procedure ± PCI/DES (MIDCAB group), n=118 patients, or classical OPCAB (OPCAB group), n=211 patients, at our institution from January 2017 to July 2019. A matched analysis using the EuroSCORE II (81 patients per group) was done. Results The median of EuroSCORE II was 1.05 in both groups, p=1. All MIDCAB patients underwent a left-sided mini-thoracotomy and received a single LIMA-LAD graft, OPCAB patients received median 3 distal anastomoses, p Conclusions MIDCAB is a good and safe option to treat severe coronary artery disease. MIDCAB is not only less invasive, but associated with reduced perioperative risk compared to standard OPCAB surgery even if a hybrid procedure is needed. Funding Acknowledgement Type of funding source: None

We studied myocardial protection during coronary artery bypass graft surgery using low-volume cardioplegia (Cardioplexol) and minimal extracorporeal circulation (MECC) for different types of coronary artery diseases. In total, 426 consecutive patients were included and divided into four groups: those with left main stem stenosis (n = 45), those with three-vessel disease (n = 200), those with both (n = 141), and those with neither (n = 40). The peak postoperative myocardial markers and 30-day mortality were analyzed. Both myocardial markers and 30-day mortality were significantly elevated in patients with isolated main stem stenosis. We conclude that the use of low-volume cardioplegia and MECC is safe. However, patients with underlying isolated left main stem stenosis might be less protected.

The New Jersey Highlands and contiguous Hudson (New York) Highlands host hundreds of small, worked-out magnetite mines, and the major zinc-oxide deposits of Franklin and Sterling Hill. The origin of the magnetite ore remains controversial. Two temporally distinct genetic models have been proposed for magnetite: (1) a pre-Ottawan, sedimentary exhalative model in which ores were deposited on the seafloor as precipitates from iron-enriched hydrothermal fluids; (2) a late-Ottawan, fluid alteration model in which the current mineral composition of ores was derived from Fe-rich, alkaline fluids, associated with late episodes of granitic plutonism (low-Ti Kiruna-type deposits), or in which deposits derived from metamorphogenic fluids circulating in a regional shear zone leached metals from host rocks and precipitated them in veins and faults. Detailed mapping of ore deposits and host rocks near Wanaque and Ringwood, New Jersey, and Warwick, New York, reveal that ore bodies are hosted by supracrustal rocks deposited in a back-arc basin. At Wanaque and Warwick, ores are strataform and stratabound. Discordant ore bodies at Ringwood are associated with a feeder zone, presumed to have intersected the paleosurface at a steep angle. Sharply layered quartz–diopside gneiss is spatially associated with ore at Wanaque and Warwick, and massive diopsidite is spatially associated with ore at all three locations. Diopsidite in these rocks is nonaluminous, iron enriched, and shares these characteristics with modern metalliferous sediments. Massive diopsidite and quartz–diopside gneiss are interpreted to be metamorphosed carbonate facies iron formation, and associated magnetite to be metamorphosed oxide-facies iron formation.

OBJECTIVES The aim of this article is to report the mid-term results of ascending thoracic endovascular aortic repair using a custom-made device (CMD). METHODS This was a retrospective study performed at tertiary centres. Nine patients considered unfit for open surgery received elective total endovascular repair of the ascending aorta with a Relay® (Terumo Aortic, Sunrise; FL, USA) CMD: pseudoaneurysn (n = 5), localized dissection (n =3) and contained rupture (n = 1). RESULTS Primary clinical success was achieved in all patients with no major complications and no early conversion to open surgery. All patients were discharged home and independent: median length of stay was 7 days (interquartile range, 6–18). No patient was lost to follow-up at a median 26 months (interquartile range, 12–36). Three patients died 2, 6 and 24 months after intervention; 1 was aorta related (late aorto-atrial fistula due to infection that required open surgery). At the last follow-up available, no endoleaks, migrations, fractures or ruptures were observed in the remaining 6 patients. CONCLUSIONS Ascending thoracic endovascular aortic repair with Terumo Aortic CMDs was technically feasible, effective and safe in very selected lesions. CMDs showed good ascending aorta conformability with different configurations and diameters, and satisfactory mid-term durability as shown by both structural integrity and aortic lesion exclusion.

Die retrosternale Infektion des vorderen Mediastinums nach herzchirurgischem Eingriff mit Sternotomie (Poststernotomie-Mediastinitis, PSM) ist eine lebensbedrohliche Komplikation. Sie stellt ein multifaktorielles und komplexes Krankheitsbild dar, an deren Diagnostik und Therapie viele Disziplinen beteiligt sind. Da verschiedene Behandlungskonzepte existieren, die oft auf Erfahrungen einzelner Kliniken beruhen, hat es sich die Deutsche Gesellschaft fur Thorax-, Herz- und Gefaschirurgie (DGTHG) in Kooperation mit der Schweizerischen Gesellschaft fur Herz- und Thorakale Gefaschirurgie (SGHC) zum Ziel gesetzt, eine evidenz- und konsensbasierte Leitlinie zum „Management der Mediastinitis nach herzchirurgischem Eingriff“ zu entwickeln. Unter Einbeziehung aller an Diagnostik und Therapie der PSM beteiligten Berufsgruppen wird eine S3-Leitlinie gemas dem Regelwerk der Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF) erarbeitet, um die medizinische Versorgung von Patienten mit PSM zu verbessern. Hier werden die Ziele und die methodischen Verfahren dieser Leitlinie beschrieben.

The European Alps are well positioned to contribute significantly to the energy transition. In addition to sites with above-average potential for wind and solar power, the “water towers” of Europe provide flexible, low-carbon power generation as well as energy storage. In the future, hydropower systems are expected to become more than mere electricity generators, serving a key role as flexible complements to intermittent power generators and as providers of large-scale seasonal and daily energy storage. Energy transition on national and European scales can be facilitated by expanding the capacity of pumped storage hydropower (PSHP) plants. Yet the extension of hydropower production, in particular PSHP, remains controversial, primarily due to environmental concerns. Focusing on 2 Alpine countries, Austria and Switzerland, this paper provides a system view of hydropower production and energy storage in the Alps. It discusses advantages and drawbacks of various assessment tools and identifies gaps and needs for the integrated assessment of PSHP plants. It concludes that instruments that evaluate the impacts and sustainability of PSHP projects need to be developed, elaborated, and applied in a participatory manner, in order to promote public dialogue, increase social acceptance, and, ideally, encourage energy consumers to become advocates of a sustainable energy future.

Eine 42-jahrige Frau stellte sich wegen anhaltenden re­trosternalen Druckgefuhls und Ubelkeit nach Beendigung eines langen Trainingslaufes beim Hausarzt vor.

Background We hypothesized that preoperative computed tomography (CT) is a predictor of abdominal intervention for visceral malperfusion and stroke after emergent surgery for acute type A aortic dissection (AAAD). Methods A total of 90 patients, mean (± SD [standard deviation]) age 62 (± 12) years, 76% males, undergoing emergent surgery for AAAD at our institution from May 2008 to August 2013 were included. All clinical data were collected prospectively and correlated to CT images. Results At initial presentation 9 (10%) patients showed preoperative focal neurologic deficit or coma, 10 patients (11%) complained of abdominal pain, and the logistic EuroSCORE was 44 (± 22). Hemiarch replacement was performed in 96%, total arch in 4%. The duration of hypothermic circulatory arrest (HCA) at 28°C bladder temperature was 26 (± 19) minutes. Cross-clamp time was 88 (± 39) minutes, and cardiopulmonary bypass (CPB) time 148 (± 49) minutes. Overall 30-day mortality was 13%. Moreover, 12 (13%) patients required postoperative abdominal interventions for suspected visceral malperfusion; stroke occurred in 25 (28%) patients. Logistic regression revealed that “dissection of the celiac trunk and/or the superior mesenteric artery” in preoperative CT images is a predictor of postoperative abdominal interventions for visceral malperfusion ( p = 0.03), but preoperative abdominal pain is not similarly predictive. Postoperative stroke is best predicted by preoperative neurologic symptoms ( p = 0.01), but not by supra-aortic vessel dissection in preoperative CT images. Conclusion In patients undergoing surgery for AAAD, analysis of preoperative CT images allows identifying those with a high risk of postoperative abdominal intervention for visceral malperfusion. Postoperative stroke is best predicted by preoperative neurologic symptoms.

Objective: Invasive pulmonary aspergillosis is frequent in neutropenic patients. Usually localized in the beginning, the disease spreads and mortality is high despite antifungal treatment. The role of early adjuvant surgery is not clear. Surgery may help to confirm fungal disease, may control fungal disease locally and may prevent systemic spreading. This study examines effects of early resection on survival and dissemination in a rat model of localized invasive pulmonary aspergillosis. Methods: Forty persistently neutropenic male albino rats were challenged with standardized conidial aspergillus inoculum injected into peripheral lung tissue of the right upper lobe under direct vision. Animals were divided into four groups. Twenty animals were treated with amphotericin B at 1 mg/kg per day beginning 48 h after inoculation, 20 animals were left untreated. In each group half the animals underwent early resection of localized invasive aspergillosis by lobectomy. Animals were checked daily and mortality was recorded up to 28 days after which surviving animals were sacrificed. Results: Significantly higher survival was observed in resected animals in the non-Am B groups (survival: 10 ^ 19% without early resection and 50 ^ 32% with early resection; P ¼ 0:044). However, early resection did not lead to improved survival in animals treated with amphotericin B (survival 70 ^ 29% without early resection and 50 ^ 32% with early resection; P ¼ 0:316). Conclusions: In this rat model of localized invasive pulmonary aspergillosis effects of early resection on survival could be demonstrated only in animals not receiving amphotericin B treatment. q 2002 Elsevier Science B.V. All rights reserved.

Shear zone-hosted ore bodies at the Balmat, NY, zinc deposit were remobilized at the kilometer scale during amphibolite facies metamorphism ca. 1180 Ma. Despite there being little evidence for interaction of hydrous fluids with ores during deformation, such translocation distances are considered unlikely without the assistance of fluids. Measurements of Zn isotopic composition of sphalerite from six Balmat ore bodies that originated from the same stratigraphic level (Upper Marble unit 6) reveal variation between ore bodies, as well as intra-orebody trends of isotopic lightening down plunge. In general, increasing distance of remobilization correlates with decreasing ẟ66Zn. The syn-tectonic isotopic fractionation recorded in Balmat sphalerite is interpreted to have resulted from the interaction between the ore and sulfide melts that were fluxed by H2S localized primarily in Upper Marble unit 7 (fetid dolomite). Lighter isotopes of Zn were enriched in the melt, leaving the residual ore enriched in heavier Zn isotopes. These observations support previous petrographic evidence for the presence of anatectic melts at Balmat and help to explain the unusual scale of translocation at Balmat by means of fluid-assisted remobilization during which intergranular melts decreased rock competency.

Background We hypothesized that patients treated with prasugrel compared with those treated with clopidogrel before coronary artery bypass graft (CABG) surgery are at increased risk of hemorrhagic complications needing transfusions and are more likely to need surgical reexploration. Patients and Methods Preoperative and postoperative clinical data were collected prospectively on 143 consecutive patients treated preoperatively with prasugrel (n = 59) or clopidogrel (n = 84) before undergoing isolated CABG at our institution from January 2011 to October 2012. Results Patients receiving prasugrel showed a slightly higher additive EuroSCORE I compared with those treated with clopidogrel (7 vs. 5, p = 0.001), and more often underwent urgent/emergent CABG (47 vs. 27%, p = 0.01). Patients treated with prasugrel were more likely than those treated with clopidogrel to need perioperative platelet transfusions (25 vs. 12%, p = 0.04). The numbers of transfusions of red blood cells, fresh-frozen plasma, or fibrinogen were high in both groups but showed no statistical differences. Patients treated with prasugrel, however, more often needed surgical reexploration for bleeding complications (8 vs. 1%, p = 0.03). Logistic regression revealed that preoperative therapy with prasugrel (RR 2.9, p = 0.01) and urgent/emergent surgery (RR 2.2, p = 0.04) were predictors for the composite endpoint “need for perioperative platelet transfusion and/or surgical reexploration.” Conclusion Pretreatment with prasugrel, compared with clopidogrel, in patients undergoing isolated CABG is associated with an increased need for platelet transfusions and a higher risk of surgical reexploration for bleeding complications.

The Bernoullis were one of the most distinguished families in the history of science. It was Daniel Bernoulli who applied mathematical physics to medicine to further his understanding of physiological mechanisms that have an impact even in today's high-end medicine. His masterwork was the analysis of fluid dynamics, which resulted in Bernoulli's law. Most important for cardiac surgery, it describes how a centrifugal pump works within an extracorporeal circulation, lays the basis for measuring a gradient over a stenotic heart valve, and explains how to measure the transit time flow within a bypass graft.

Ein 77-jahriger Patient wird zur dringlichen operativen Myokardrevaskularisation bei schwerer koronarer 3-Gefasserkrankung an unser Herzzentrum uberwiesen.

Objectives We hypothesized that antegrade open stent graft implantation in the descending aorta during acute type A aortic dissection surgery is safe and improves patient outcome compared with the standard repair technique. Methods Hundred and forty-one consecutive patients underwent surgery for acute type A aortic dissection at our institution from 2010 to 2016. Of those, 104 patients underwent ascending aorta and hemiarch repair under hypothermic circulatory arrest with antegrade cerebral perfusion (standard group). Since 2013, 37 patients have undergone the standard procedure combined with antegrade stent implantation in the descending aorta (stented group). A matched analysis using the logistic EuroSCORE (37 patients per group) was done. All data were collected prospectively. Results The mean logistic EuroSCORE was 29 in both groups, P = 1. Cardiopulmonary bypass time was 150 ± 57 (standard) vs 157 ± 48 (stented) min, P = 0.6; aortic clamping 99 ± 47 (standard) vs 100 ± 36 (stented) min, P = 1. Stented patients had longer circulatory arrest times with antegrade cerebral perfusion, 23 ± 7 vs 15 ± 7 min, P < 0.001. Stroke occurred in 24.3% (standard) vs 8.1% (stented), P = 0.1; paraplegia developed in 2.7% (standard) vs 0% (stented), P = 1. Abdominal intervention due to suspected visceral ischaemia was needed in 18.9% (standard) vs 5.4% (stented), P = 0.2. 30-day mortality was 13.5% (standard) vs 0% (stented), P = 0.05. Survival at 6-month was 100% and 86.5% in patients with implanted stents and standard repair, respectively, P = 0.02. Conclusions Antegrade, open stent graft implantation into the descending aorta during acute type A aortic dissection repair is safe and is associated with improved outcomes at 6 months postoperatively compared to the standard repair technique.

Background Pulmonary invasive fungal disease is a frequent complication in patients with hematologic malignancies. Surgical resection in addition to antifungal therapy is an option for selected cases but often feared because of immunosuppression. Methods We analyzed the outcome of 71 patients undergoing lung resection for pulmonary invasive fungal disease. Most patients had leukemia, 44 underwent high-dose chemotherapy, and 18 underwent stem cell transplantation. Results On the day of surgery, 44 patients were neutropenic, and 41 had a platelet count 9 /L. Forty-five nonanatomic (atypical) resections and 26 lobectomies were performed. Fungal infection was histologically proven in 53 patients. Reoperation was needed in four patients (bronchial stump dehiscence, persistent air leak, chylothorax, and seroma). Minor complications at the site of surgery occurred in 14 patients. In only two, there was an uncontrolled disseminated fungal infection. Overall, mortality at 30 days was 7% (five of 71). Long-term survival was mainly influenced by the underlying hematologic disease. Conclusions Lung resection is a therapeutic option for hematologic patients with pulmonary fungal infection. Despite immunosuppression, the perioperative morbidity and mortality is acceptable, and, therefore, the prognosis is not determined by the surgical intervention.

The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene. Recent molecular studies, most performed in mouse models, revealed that the MFS is more a developmental abnormality with broad and complex effects on the morphogenesis and function of multiple organ systems. FBN1 haploinsufficiency and dysregulated transforming growth factor-beta (TGF-β) signaling seem to be critical for clinical manifestations in MFS including aortic root dilatation. Aortic root aneurysm and aortic dissection represent the main causes of morbidity and mortality in MFS. Most importantly, TGF-β antagonism through angiotensin II type 1 receptor blockers (ARBs), for example losartan, has been shown to prevent and possibly reverse aortic root dilatation in a mouse model of MFS. A first human study on a small pediatric cohort confirmed those promising results in reducing the aortic root growth over a follow-up period of 12 to 47 months. So, a large multicenter trial has been set up and results should be available soon. Other therapeutic strategies which might be combined with losartan include traditional β-blockade, doxycyclin and statins. Such management could offer the first potential for primary prevention of clinical manifestations in MFS.