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Background: There is concern about the durability of mitral valve repair (MVr) for mitral regurgitation (MR) in Marfan patients due to limited long-term data. Furthermore, a detailed time course of changes in cardiac function after MVr in Marfan patients has not been reported. We examined repair techniques, postoperative cardiac function, and outcomes of MVr in Marfan patients., Methods and Results: We retrospectively reviewed 29 Marfan patients (mean [±SD] age 27.4±14.8 years) who underwent MVr at The University of Tokyo Hospital from 2010 to 2022. The mean follow-up period was 5.2±3.2 years. The causes of MR were isolated anterior leaflet prolapse in 25% of patients, isolated posterior leaflet prolapse in 11%, and bileaflet prolapse in 64%. Echocardiographic findings showed significant decreases in left ventricular (LV) diastolic and left atrial diameters 1 week after MVr. LV systolic diameter was significantly decreased 3 years after MVr, and LV ejection fraction initially declined before subsequently increasing. The in-hospital and 30-day mortality rates were 0%. At 5 years, the overall survival rate was 94% and the rate of freedom from MR was 84%., Conclusions: The mid- to long-term outcomes after MVr in Marfan patients were satisfactory, supporting the durability of MVr in these patients. Postoperative cardiac reverse remodeling occurred in a phased manner in Marfan patients, similar to that in patients with degenerative MR.

Objective: Thoracic endovascular aortic repair (TEVAR) in patients with genetic aortopathies (GA) is controversial, given concerns of durability. We describe characteristics and outcomes after TEVAR in patients with GA., Methods: All patients undergoing TEVAR between 2010 and 2023 in the Vascular Quality Iniatitive were identified and categorized as having a GA or not. Demographics, baseline, and procedural characteristics were compared among groups. Multivariable logistic regression was used to evaluate the independent association of GA with postoperative outcomes. Kaplan-Meier methods and multivariable Cox regression analyses were used to evaluate 5-year survival and 2-year reinterventions., Results: Of 19,340 patients, 304 (1.6%) had GA (87% Marfan syndrome, 9% Loeys-Dietz syndrome, and 4% vascular Ehlers-Danlos syndrome). Compared with patients without GA, patients with GA were younger (50 years [interquartile range, 37-72 years] vs 70 years [interquartile range, 61-77 years]), more often presented with acute dissection (28% vs 18%), postdissection aneurysm (48% vs 17%), had a symptomatic presentation (50% vs 39%), and were less likely to have degenerative aneurysms (18% vs 47%) or penetrating aortic ulcer (and intramural hematoma) (3% vs 13%) (all P < .001). Patients with GA were more likely to have prior repair of the ascending aorta/arch (open, 56% vs 11% [P < .001]; endovascular, 5.6% vs 2.1% [P = .017]) or the descending thoracic aorta (open, 12% vs 2% [P = .007]; endovascular, 8.2% vs 3.6% [P = .011]). No significant differences were found in prior abdominal suprarenal repairs; however, patients with GA had more prior open infrarenal repairs (5.3% vs 3.2%), but fewer prior endovascular infrarenal repairs (3.3% vs 5.5%) (all P < .05). After adjusting for demographics, comorbidities, and disease characteristics, patients with GA had similar odds of perioperative mortality (4.6% vs 7.0%; adjusted odds ratio [aOR], 1.1; 95% confidence interval [CI], 0.57-1.9; P = .75), any in-hospital complication (26% vs 23%; aOR, 1.24; 95% CI, 0.92-1.6; P = .14), or in-hospital reintervention (13% vs 8.3%; aOR, 1.25; 95% CI, 0.84-1.80; P = .25) compared with patients without GA. However, patients with GA had a higher likelihood of postoperative vasopressors (33% vs 27%; aOR, 1.44; 95% CI, 1.1-1.9; P = .006) and transfusion (25% vs 23%; aOR, 1.39; 95% CI, 1.03-1.9; P = .006). The 2-year reintervention rates were higher in patients with GA (25% vs 13%; adjusted hazard ratio, 1.99; 95% CI, 1.4-2.9; P < .001), but 5-year survival was similar (81% vs 74%; adjusted hazard ratio, 1.02; 95% CI, 0.70-1.50; P = .1)., Conclusions: TEVAR for patients with GA seemed to be safe initially, with similar odds for in-hospital complications, in-hospital reinterventions, and perioperative mortality, as well as similar hazards for 5-year mortality compared with patients without GA. However, patients with GA had higher 2-year reintervention rates. Future studies should assess long-term durability after TEVAR compared with the recommended open repair to appropriately weigh the risks and benefits of endovascular treatment in patients with GA., Competing Interests: Disclosures S.L.Z. reports scientific advising/consulting for W. L. Gore & Associated, Cook Medical, and Terumo Aortic., (Copyright © 2024 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Marfan syndrome (MFS) affects the cardiovascular system. Aortic root aneurysm is a pathognomonic feature of MFS; however, the abdominal aorta is rarely affected. A consensus on surveillance for the abdominal aorta in patients with MFS has not been established. In the present study, we compared the outcomes after open surgical repair (OSR) of abdominal aortic aneurysms (AAAs) in patients with and without MFS., Methods: We conducted a retrospective, single-center cohort study from 2003 to 2020. We reviewed and compared 28 patients with MFS and 426 patients without MFS who had undergone OSR for AAAs. The baseline characteristics, medical comorbidities, previous cardiovascular surgery, anatomic features of the AAAs, and surgical treatment outcomes were compared between the two groups., Results: The patients with MFS were younger than those without MFS at the AAA diagnosis (47.2 ± 12.3 vs 70.6 ± 7.9 years; P < .001). The proportion of women was also greater for those with MFS (46.4% vs 15.7%; P < .001). The AAAs were most often located at the infrarenal aorta in both groups. However, thoracoabdominal AAAs were more often found among patients with MFS (10.7% vs 0.9%; P < .012). The proportion of symptomatic patients was lower in the MFS group (3.6% vs 21.6%; P = .022). The maximum median diameter of the AAA at surgery was smaller in the patients with MFS (52 mm vs 58 mm; P = .001). However, concomitant aortic dissection (32.1% vs 3.3%; P < .001) was more prevalent among the patients with MFS. Consequent aneurysmal changes in the iliac artery after AAA repair were more frequent in the patients with MFS (7.1% vs 0%; P = .004). No significant differences were found in 30-day or overall mortality between the patients with and without MFS during a median follow-up period of 71 months (interquartile range, 24.7-121.1 months) and 26.7 months (interquartile range, 7.4-69.5 months), respectively., Conclusions: The surgical outcomes of OSR for AAAs for patients with MFS were not significantly different from those for patients without MFS in a well-established surveillance program of MFS., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Competing interests: None declared.

Objectives: Thoracic endovascular aortic repair (TEVAR) remains controversial in patients with connective tissue disorders given the concern for durability. We report on the largest series to date on outcomes of patients with thoracic aortic disease and connective tissue disorders treated with TEVAR., Methods: The Vascular Quality Initiative registry identified 12 207 patients treated with TEVAR from January 2010 to December 2018, including 102 with Marfans, Ehlers-Danlos, or Loey-Dietz syndrome. Outcomes were analyzed per the Society for Vascular Surgery reporting standards., Results: Median age was 50.6 years (interquartile range: 57.0-75.0), and 62 (60.7%) were male. Eighty-eight (86.3%) patients had Marfan, 9 (8.8%) had Ehlers-Danlos, and 5 (4.9%) had Loey-Dietz syndrome. Twenty-six (25.5%) patients were treated for degenerative aneurysmal disease and 76 (74.5%) patients for type B dissections (33 acute, 31 chronic). Most common indications for interventions in patients with type B dissection were pain (n = 41), aneurysmal degeneration (n = 16), and malperfusion (n = 8), with 3 patients who presented ruptured. There was no significant difference in perioperative complications between acute/chronic dissections and aneurysms ( P = .14). Percutaneous access was utilized in 61.7% of patients, with a 2.9% rate of arterial injury requiring reintervention. Follow-up data were available for 75 (73.3%) patients at a mean follow-up of 15.6 months. Overall mortality was 5.3%. There were 30 patients with follow-up endoleak data, and 8 (26.7%) endoleaks were identified. All endoleaks were in patients treated for acute type B dissection, and all resolved after a mean of 2.1 reinterventions. Three patients treated for acute Type B Aortic Dissection (TBAD) had retrograde dissections requiring intervention., Discussion: Thoracic endovascular aortic repair for patients with connective tissue disorders can be performed with low perioperative mortality, spinal cord ischemia, or Cerebrovascular Accident (CVA). On follow-up, acute type B aortic dissections represent a higher risk subgroup with increased rates of endoleak and retrograde dissection. Closer follow-up for these patients and early reintervention may be beneficial.

Objectives: We sought to develop strategies for management of the aortic arch in patients with Loeys-Dietz syndrome (LDS) through a review of our clinical experience with these patients and a comparison with our experience in patients with Marfan syndrome (MFS)., Methods: We reviewed hospital and follow-up records of 79 patients with LDS and compared them with 256 patients with MFS who served as reference controls., Results: In the LDS group, 16% of patients presented initially with acute aortic dissection (AAD) (67% type A, 33% type B) or developed AAD during follow-up, compared with 10% of patients with MFS (95% type A, 5% type B). There was no difference between patients with LDS or MFS in need for subsequent arch interventions after aortic root surgery (46% vs 50%, P = 1.0). Among the patients who never had AAD, the need for arch repair at initial root surgery was greater in patients with LDS (5% vs 0.4%, P = .04), as was the need for any subsequent aortic surgery (12% vs 1.3%, P = .0004). Late mortality in patients with LDS after arch repair was greater than in those patients who had no arch intervention (33% vs 6%, P = .007)., Conclusions: In the absence of dissection, patients with LDS have a greater rate of arch intervention after root surgery than patients with MFS. After a dissection, arch reintervention rates are similar in the 2 groups. Arch intervention portends greater late mortality in LDS., (Copyright © 2019. Published by Elsevier Inc.)

Background: Patients presenting with descending aortic aneurysms developing after aortic dissection often undergo continued aortic expansion which may require operative interventions to address the risk of aortic rupture. In light of the current advances in various treatment options, including endovascular approaches, we analyzed our experience with open surgical repair (OSR) of aneurysms of the descending aorta following aortic dissection., Methods: Patients who underwent open repair for aneurysmal changes of the descending aorta after chronic dissection were retrospectively studied. The 30-day operative mortality rate, midterm survival, and major complications were analyzed. Patients were divided into two categories; primary chronic type B aortic dissection and remnant repaired type A aortic dissection (RTAAD)., Results: There were 149 patients with enlargement of the descending thoracic aorta developing after aortic dissection. Of these, 49 patients had medical management, while the remaining 100 patients received OSR. These patients were included in the present analysis. The 30-day mortality and permanent paraplegia rates were 9% and 4%, respectively. The 1-, 3-, and 5-year survival rates were 83%, 80.9%, and 76.1%, respectively. The 1- and 5-year survival rates between the primary chronic type B aortic dissection and remnant RTAAD groups showed no significant between-group differences at 86.7% and 84.3%, and 80% and 71.3%, respectively (P = .289). The overall outcomes of other complications such as renal injury, bleeding reoperation, and extracorporeal membrane oxygenation support showed no significant between-group differences, including an insignificantly higher neurologic complication rate in the remnant RTAAD group. The survival rate in patients with Marfan syndrome was significantly higher than in the patients without Marfan syndrome (P = .033)., Conclusions: OSR for descending aortic aneurysms developing after chronic aortic dissection showed good early and mid- to long-term outcomes, with acceptably low complication rates. OSR for descending aortic aneurysm after chronic aortic dissection associated with Marfan syndrome also showed good early and mid- to long-term outcomes., (Copyright © 2020 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Patients with Marfan syndrome (MFS) often present with acute catastrophic aortic events at a young age and have a shortened life span. This study examines the impact of presentation and demographics on late survival in patients with MFS., Methods: Adults with confirmed MFS in our thoracic aortic center dataset were identified and statistical analysis performed to identify the incidence and predictors of aortic interventions and late mortality., Results: We identified 301 patients with a MFS initial diagnosis at age 17 years (interquartile range, 4-30 years) with presentation into our thoracic aortic center at 21 years (interquartile range, 8-34 years). The average follow-up in our center was 10 ± 10 years. Clinical features were 41% male, 86% white race, coronary artery disease 28%, hypertension 40%, peripheral vascular disease 19%, and anti-impulse agent in 51% (β-blocker, angiotensin-converting enzyme inhibitor/angiotensin receptor blocker, calcium channel blocker). Distribution of operative aortic pathology was isolated to the ascending aorta (70%) and descending aorta (8%). One hundred seventy-eight patients (59%) required primary aortic surgery (36% emergent). Primary procedures were cardiac (aortic valve/root) in nature in 94%. Seventy-four patients (42%) required multiple aortic procedures at a mean of 9.2 ± 6.9 years, involving the thoracoabdominal aorta in 65%, thoracic aorta in 37%, and abdominal aorta in 21%. Patients who required multiple aortic procedures were more likely (P < .05) to have coronary artery disease (50% vs 30%), and peripheral vascular disease (43% vs 18%). Multiple aortic procedures were also more likely (P < .05) in patients who developed de novo distal dissection (14% vs 0%), had prior dissection (47% vs 18%), or unknown MFS at the time of the initial procedure (27% vs 63%). Multivariable analysis identified prior dissection as an independent predictor of need for emergent surgery (odds ratio, 13.20; 95% confidence interval, 4.64-37.30; P < .05), as well as additional aortic surgery (odds ratio, 4.42; 95% confidence interval, 1.87-10.50; P < .05). Kaplan-Meier analysis showed similar 10-year survival with or without aortic interventions (82% with vs 89% without; P = .08). Late survival was decreased in patients undergoing emergent initial procedures (66% vs 89%; P < .01), as well as those undergoing multiple operations (74% vs 86%; P = .03)., Conclusions: These data indicate that, in the modern era, the mode of presentation and need for multiple procedures have a detrimental impact on late survival. Additionally, the presence of acute or chronic dissection predicts the need for additional aortic procedures during follow-up., (Copyright © 2019. Published by Elsevier Inc.)

Early onset Marfan syndrome is the most severe form of Marfan syndrome diagnosed during perinatal period. Early onset Marfan syndrome is associated with high mortality rates, usually within the first 2 years of life. First, we present a case of prenatally diagnosed early onset Marfan syndrome in a dichorionic diamniotic twin pregnancy, where suspicion was raised at 35 weeks of gestation. Ultrasound and fetal magnetic resonance imaging were used to assess prenatal findings in the affected fetus. She presented right diaphragmatic eventration, elongation of humerus and femur and subluxation of the crystalline lens. She died 3 months after birth. Secondly, we present a PubMed-based review of the published articles on early onset Marfan syndrome, with pre- or postnatal suspicion or diagnosis. We found 39 articles published between 1981 and 2017, arising information on 55 cases. Including ours, early onset Marfan syndrome was prenatally diagnosed in 34.54% of the cases. In these cases, the most frequent prenatal findings were cardiomegaly, dilatation of the great vessels and mitral or tricuspid regurgitation. Mortality rate during the first 15 months after birth was 73.68%. In the postnatally diagnosed cases, the most frequent findings were arachnodactyly, dilatation of the great vessels and mitral or tricuspid regurgitation. Mortality rate was 61.11%. Overall genetic confirmation was performed in 67.27% of the cases. Prenatal diagnosis of early onset Marfan syndrome is challenging but of utmost importance, since management should take place in a tertiary care center, by a multidisciplinary team. Differential diagnosis is essential in order to perform an adequate genetic counseling.

Background: Aortic risk has not been evaluated in patients with Marfan syndrome and documented pathogenic variants in the FBN1 gene., Objectives: This study sought to describe aortic risk in a population with Marfan syndrome with pathogenic variants in the FBN1 gene as a function of aortic root diameter., Methods: Patients carrying an FBN1 pathogenic variant who visited our reference center at least twice were included, provided they had not undergone aortic surgery or had an aortic dissection before their first visit. Aortic events (aortic surgery or aortic dissection) and deaths were evaluated during the 2 years following each patient visit. The risk was calculated as the number of events divided by the number of years of follow-up., Results: A total of 954 patients were included (54% women; mean age 23 years). During follow-up (9.1 years), 142 patients underwent prophylactic aortic root surgery, 5 experienced type A aortic dissection, and 12 died (noncardiovascular causes in 3, unknown etiology in 3, post-operative in 6). When aortic root diameter was <50 mm, risk for proven type A dissection (0.4 events/1,000 patient-years) and risk for possible aortic dissection (proven aortic dissection plus death of unknown cause, 0.7 events/1,000 patients-years) remained low in this population that was treated according to guidelines. Three type A aortic dissections occurred in this population during the 8,594 years of follow-up, including 1 in a patient with a tubular aortic diameter of 50 mm, but none in patients with a family history of aortic dissection. The risk for type B aortic dissection in the same population was 0.5 events/1,000 patient-years., Conclusions: In patients with FBN1 pathogenic variants who receive beta-blocker therapy and who limit strenuous exercise, aortic risk remains low when maximal aortic diameter is <50 mm. The risk of type B aortic dissection is close to the remaining risk of type A aortic dissection in this population, which underlines the global aortic risk., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Little is known about the incidence of cardiovascular events (CVEs) and their associated risk markers in children with Marfan syndrome (MFS)., Aims: To assess the incidence of CVEs and determine risk markers in a cohort diagnosed with Marfan syndrome during childhood and followed for several years., Methods: From a French multicentre nationwide database, 462 patients with MFS diagnosed during childhood were included prospectively. Patients' files were screened for a period of 20 years (1993-2013). CVEs (e.g. death, aortic dissection, cardiac valve or aortic root surgery) were assessed during the prospective follow-up., Results: Median (interquartile range) age at the end of follow-up was 17.2 (11.1-21.3) years. CVEs were reported for 35 participants (7.6%; 95% confidence interval [CI] 5.3-10.4%). First CVEs were prophylactic aortic root surgery (n=29), aortic dissection (n=4; two aged <18 years) and death (n=2). Kaplan-Meier cumulative incidence of CVEs was 5.3% (95% CI 3.3-8.7%) during childhood (aged≤18 years) and 19.4% (95% CI 13.3-27.9%) at 25years of age. The cumulative rate of CVEs was higher in case of Valsalva sinus Z-score increase of≥0.1 per year (P=0.0003), maximal Valsalva sinus diameter growth speed ≥5mm per year (P=0.03), aortic regurgitation≥2 (P=0.0005) and maximal Valsalva sinus Z-score≥3 before 16 years of age (P<0.0001). In a multivariable Cox proportional analysis, the Valsalva sinus Z-score remained significantly related to outcome. Considering aortic root evolution, aortic regurgitation, age at diagnosis and beta-blocker therapy were related to Valsalva sinus Z-score evolution during follow-up., Conclusions: CVEs in children with MFS are mainly related to prophylactic aortic root surgery. Aortic dissections are rarely observed in children. The Valsalva sinus Z-score is a strong indicator of subsequent CVEs in children with MFS. Attention to follow-up and beta-blocker observance may be warranted in high-risk children., (Copyright © 2019. Published by Elsevier Masson SAS.)

Patients with Marfan syndrome (MFS) often require surgical intervention on the mitral valve (MV), aortic root or valve (AV), or thoracic aorta (TA) during childhood and adolescence. We aim to utilize a national database to evaluate outcomes in pediatric and young adult patients with MFS undergoing MV, AV, and aortic surgical procedures, and describe factors associated with increased mortality. The Pediatric Hospital Information System (PHIS) database, a multi-institutional administrative database of 48 pediatric hospitals, was queried for patients less than 25 years of age with a diagnosis of MFS (ICD-9 759.82) who underwent MV, AV, or thoracic aortic surgery between January 2004 and October 2015. We assessed comorbidities and complications, and performed univariate analysis to evaluate factors associated with inpatient mortality. Included were 321 hospital encounters in 294 patients. Fifty-one patients underwent 54 MV surgeries, 213 patients underwent 224 aortic/AV surgeries, and 43 patients underwent both MV and aortic/AV surgery in the same encounter. Postoperative complications were common for all surgeries (46.3% for MV procedures and 45.5% for aortic/AV procedures). Overall in-hospital mortality was 2.2% (3.7% for MV procedures, 1.8% for AV/aortic procedures, and 2.3% in the combined MV and aortic/AV procedure group). Aortic dissection or rupture was reported in 3.4%, with no in-hospital mortalities. Death after MV as well as after aortic/AV surgery was associated with younger age. Postoperative complications are common in pediatric and young adult patients with MFS after intervention on the MV, AV, and TA, although mortality is relatively low., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aims: Life expectancy in Marfan syndrome patients has improved thanks to the early detection of aortic dilation and prophylactic aortic root surgery. Current international clinical guidelines support the use of aortic root diameter as a predictor of complications. However, other imaging markers are needed to improve risk stratification. This study aim to ascertain whether proximal aorta longitudinal and circumferential strain and distensibility assessed by cardiac magnetic resonance (CMR) predict the aortic root dilation rate and aortic events in Marfan syndrome., Methods and Results: One hundred and seventeen Marfan patients with no previous aortic dissection, cardiac/aortic surgery, or moderate/severe aortic regurgitation were prospectively included in a multicentre protocol of clinical and imaging follow-up. At baseline, CMR was performed and proximal aorta longitudinal strain and ascending aorta circumferential strain and distensibility were obtained. During follow-up (85.7 [75.0-93.2] months), the annual growth rate of aortic root diameter was 0.62 ± 0.65 mm/year. Fifteen patients underwent elective surgical aortic root replacement and four presented aortic dissection. Once corrected for baseline clinical and demographic characteristics and aortic root diameter, proximal aorta longitudinal strain, but not circumferential strain and distensibility, was an independent predictor of the aortic root diameter growth rate (P = 0.001, P = 0.823, and P = 0.997, respectively), z-score growth rate (P = 0.013, P = 0.672, and P = 0.680, respectively), and aortic events (P = 0.023, P = 0.096, and P = 0.237, respectively)., Conclusion: Proximal aorta longitudinal strain is independently related to the aortic root dilation rate and aortic events in addition to aortic root diameter, clinical risk factors, and demographic characteristics in Marfan syndrome patients., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2019. For permissions, please email: journals.permissions@oup.com.)

Objective: Valve-sparing root replacement is an attractive alternative to composite mechanical or biologic prostheses for aortic root aneurysms in children. Data on outcomes in pediatric patients are limited. We present our institutional experience with 100 consecutive pediatric valve-sparing aortic root procedures., Methods: All children who underwent valve-sparing root replacement at our institution from May 1997 to August 2017 were identified, and echocardiographic and clinical data were reviewed. The primary end point was mortality, and secondary end points included complications, further interventions, and subsequent valvular dysfunction., Results: Median age at operation was 13.6 years (interquartile range, 9.42-15.9); 51 patients (51%) had Marfan syndrome, and 39 patients (39%) had Loeys-Dietz syndrome. Mean preoperative maximum sinus diameter was 4.4 ± 0.71 cm (z score 7.3 [5.7-9.3]). Most patients (n = 80, 80%) underwent reimplantation procedures with a Valsalva graft. Four patients (4%) underwent David I reimplantation with a straight-tube graft, 13 patients (13%) underwent a Yacoub remodeling procedure, and 3 patients (3%) underwent a Florida sleeve procedure. Perioperative valve-sparing root replacement mortality was 2% (n = 2). Six patients required late reintervention for development of pseudoaneurysms. Eight patients underwent additional aortic surgery. Average time to reoperation was 7.23 ± 4.56 years. Of the 84 patients undergoing a reimplantation procedure, 5 (5.9%) underwent late valve replacement versus 5 (33.3%) of the 15 patients who received a remodeling procedure (P = .001)., Conclusions: Valve-sparing root replacement is a safe and effective option for children with aortic root aneurysms in children. The reimplantation procedure is preferred. Late aortic insufficiency and pseudoaneurysm formation remain late concerns., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Despite improvements in prevention and management, aortic aneurysm repair remains a high-risk operation for patients with Marfan syndrome (MFS) and Ehlers-Danlos syndrome (EDS). The goal of this study was to examine differences in characteristics and outcomes of patients with MFS or EDS undergoing aortic aneurysm repair at teaching versus nonteaching hospitals., Methods: We used the National Inpatient Sample to study patients with MFS or EDS undergoing open or endovascular aortic aneurysm repair from 2000 to 2014., Results: Of 3487 patients (MFS = 3375, EDS = 112), 2974 (85%) had repair at a teaching hospital. Patients who underwent repair at a teaching hospital were slightly younger than those who underwent repair at a nonteaching hospital (38 vs. 43 years, P < 0.01) but otherwise were similar in gender (29% vs. 28% female), race (70% vs. 78% white), and connective tissue disorder diagnosis (97% vs. 97% MFS, all P ≥ 0.1). There were no differences in anatomy (17% vs. 19% abdominal, 67% vs. 66% thoracic, and 15% vs. 15% thoracoabdominal, all P ≥ 0.1) or type of repair (5% vs. 5% endovascular), but patients at nonteaching hospitals were more likely to have a dissection (49% vs. 38%, P = 0.02). There was no difference in perioperative mortality (4% vs. 6%, P = 0.5) or length of stay (median 8 days vs. 7 days, P = 0.3) between teaching and nonteaching hospitals. There was also no difference in hemorrhagic (47% vs. 43%), pulmonary (9% vs. 16%), renal (12% vs. 14%), or neurologic (5% vs. 6%) complications between teaching and nonteaching hospitals, respectively (all P ≥ 0.05). In analysis stratified by anatomic extent of repair, there was a lower prevalence of pulmonary complications in thoracic aorta repairs at teaching hospitals (8.1% vs. 18.4%, P = 0.01) but a higher prevalence of hemorrhage in abdominal aortic repairs at teaching hospitals (45.6% vs. 20.6%, P = 0.04) as compared with nonteaching hospitals., Conclusions: Patients with MFS and EDS who undergo aortic aneurysm repair have their operations predominantly at teaching hospitals, but those patients who undergo repair at nonteaching hospitals do not have worse mortality or morbidity despite a higher incidence of dissection., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To describe maternal deaths in France associated with Marfan's syndrome or vascular Ehlers-Danlos syndrome., Study Design: A retrospective descriptive study based on data from the national confidential enquiry into maternal deaths, in France, during 2001-2012. Characteristics of the patients, their pregnancies and details of their deaths were analysed. The specific maternal mortality ratio by Marfan's syndrome or vascular Ehlers-Danlos syndrome was estimated., Results: Among 973 maternal deaths that occurred during the study period, five (0.4%) had a Marfan's syndrome (n=3) or a vascular Ehlers-Danlos syndrome (n=2), confirmed or suspected. The maternal mortality ratio due to Marfan's syndrome or vascular Ehlers-Danlos syndrome between 2001 and 2012 was 0.04/100,000 live births (IC 95% [0.011-0.2]). Three maternal deaths were caused by aortic dissections and two by other arterial ruptures. The deaths have occurred after 37 weeks of pregnancy for 4 patients, and at fifteen days of post-partum for one patient. The median age of death was 30 years. Three patients were nulliparous. Marfan's syndrome and vascular Ehlers-Danlos syndrome were not identified before the death of these five patients., Conclusion: Five patients with, or suspected to have, Marfan's syndrome or vascular Ehlers-Danlos syndrome were identified. Early diagnosis of these syndromes in pregnant women before life threatening events is very important, especially to refer them to appropriate care., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: To explore survival, causes of death, and the prevalence of cardiovascular events in a Norwegian Marfan syndrome (MFS) cohort. MFS is a heritable connective tissue disorder associated with reduced life expectancy-primarily due to aortic pathology., Methods: A follow-up study of 84 MFS adults, initially investigated in 2003-2004. In 2014-2015, 16 were deceased, 47 of 68 survivors consented to new clinical investigations. Analyses of events were performed for 47 survivors and 16 deceased at follow-up. Standardized mortality ratios (SMR), using the mortality rate of the Norwegian population as reference, were calculated for all 84 and calculated for men and women separately. Causes of death and information on cardiovascular events were retrieved from death certificates and medical records., Results: Standardized mortality ratios (95% confidence interval): for the whole cohort: 5.24 (3.00-8.51); for men: 8.20 (3.54-16.16); for women: 3.85 (1.66-7.58). Cardiovascular causes were found in 11 of 16 deceased, eight of these related to aortic pathology. Cancer was the cause of death in three patients. At follow-up, 51% had new cardiovascular events; 59% had undergone aortic surgery. Men experienced aortic events at younger age than women. 32% of the survivors were not followed-up as recommended., Conclusion: Life expectancy is reduced in this MFS cohort compared to the Norwegian population. Cardiovascular complications develop throughout life, particularly aortic pathology, the major cause of death in MFS. Death and aortic pathology seem to occur earlier in men. There is a need to improve follow-up according to guidelines., (© 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.)

Background: Beta-blockers are the standard treatment in Marfan syndrome (MFS). Recent clinical trials with limited follow-up yielded conflicting results on losartan's effectiveness in MFS., Objectives: The present study aimed to evaluate the benefit of losartan compared with atenolol for the prevention of aortic dilation and complications in Marfan patients over a longer observation period (>5 years)., Methods: A total of 128 patients included in the previous LOAT (LOsartan vs ATenolol) clinical trial (64 in the atenolol and 64 in the losartan group) were followed up for an open-label extension of the study, with the initial treatment maintained., Results: Mean clinical follow-up was 6.7 ± 1.5 years. A total of 9 events (14.1%) occurred in the losartan group and 12 (18.8%) in the atenolol group. Survival analysis showed no differences in the combined endpoint of need for aortic surgery, aortic dissection, or death (p = 0.462). Aortic root diameter increased with no differences between groups: 0.4 mm/year (95% confidence interval: 0.2 to 0.5) in the losartan and 0.4 mm/year (95% confidence interval: 0.3 to 0.6) in the atenolol group. In the subgroup analyses, no significant differences were observed considering age, baseline aortic root diameter, or type of dominant negative versus haploinsufficient FBN1 mutation., Conclusions: Long-term outcome of Marfan syndrome patients randomly assigned to losartan or atenolol showed no differences in aortic dilation rate or presence of clinical events between treatment groups. Therefore, losartan might be a useful, low-risk alternative to beta-blockers in the long-term management of these patients., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

The Marfan syndrome (MFS) is strongly associated with aortic disease causing a high prevalence of prophylactic aortic surgery, aortic dissection, and sudden death. The aim of the present study was to evaluate mortality in a nationwide Danish MFS population diagnosed by the Ghent II criteria. In a register-based setting, we identified all Danish patients with MFS (n = 412, men n = 215) by assessment of their medical records. We established a gender and age matched control cohort based on 41,000 control patients (men n = 21,500). MFS cases risk time was 6,669 patient years. We applied Cox regression using each case and his/her control as one stratum, adjusting for age and calendar time. We found a significantly decreased lifespan of 50years compared with 60years among controls. The mortality hazard ratio among MFS compared with controls was significantly increased to3.6 (CI 2.8-4.7, p < 0.001); men 4.0 (CI 2.8-5.7, p < 0.001); women 3.2 (CI 2.1-4.8,p < 0.001). Aorta disease represented the main reason for the overall increased mortality with a hazard ratio of 194.6 (CI 67.4-561.7, p < 0.0001); men 208.7 (CI 53.8-809.1, p < 0.001); women 173.4 (CI 31.5-954.5, p < 0.001). In addition, an unexplained mortality due to respiratory illness was not attributed to pneumothorax. Excluding cardiovascular and respiratory causes of death, we found no indication that MFS is associated with increased mortality for other reasons., (Copyright © 2018. Published by Elsevier Inc.)

This systemic review of the literature and meta-analysis aimed to evaluate the current state of the evidence for and against reimplantation of the aortic valve (RAV) versus the composite valve graft (CVG) intervention in patients with Marfan syndrome. Random effects meta-regression was performed across the study arms with logit-transformed proportions of in-hospital deaths as an outcome measure when possible. Results are presented as odds ratios with 95% confidence intervals (CIs) and P-values. Other outcomes are summarized with medians, interquartile ranges (IQR) and ranges and the numbers of patients at risk. Twenty retrospective studies that included a combined 2156 patients with long-term follow-up were identified for analysis after a literature search. The in-hospital mortality rate favoured the RAV procedure with an odds ratio of 0.23 [95% CI 0.09-0.55, P = 0.001]. The survival rate at mid-term for the RAV cohort was 96.7% (CI 94.2-98.5) vs. 86.4% (CI 82.8-89.6) for the CVG group and 93.1% (CI 66.4-100) for the RAV group vs. 82.6% (CI 74.9-89.2) for the CVG group for the long term. Freedom from valve-related reintervention (median percentages) for the long term was 97.6% (CI 90.3-100%) for the RAV procedure and 88.6% (CI 79.1-95.5) for a CVG. This systematic review of the literature stresses the advantages of the RAV procedure in patients with Marfan syndrome in regard to long- and short-term results as the treatment of choice in aortic root surgery. The RAV procedure reduces in-hospital as well as long-term deaths and protects against aortic valve reintervention.

Background: The effect of FBN1 mutation type on the severity of cardiovascular manifestations in patients with Marfan syndrome (MFS) has been reported with disparity results., Objectives: This study aims to determine the impact of the FBN1 mutation type on aortic diameters, aortic dilation rates and on cardiovascular events (ie, aortic dissection and cardiovascular mortality)., Methods: MFS patients with a pathogenic FBN1 mutation followed at two specialised units were included. FBN1 mutations were classified as being dominant negative (DN; incorporation of non-mutated and mutated fibrillin-1 in the extracellular matrix) or having haploinsufficiency (HI; only incorporation of non-mutated fibrillin-1, thus a decreased amount of fibrillin-1 protein). Aortic diameters and the aortic dilation rate at the level of the aortic root, ascending aorta, arch, descending thoracic aorta and abdominal aorta by echocardiography and clinical endpoints comprising dissection and death were compared between HI and DN patients., Results: Two hundred and ninety patients with MFS were included: 113 (39%) with an HI- FBN1 mutation and 177 (61%) with a DN- FBN1 . At baseline, patients with HI- FBN1 had a larger aortic root diameter than patients with DN- FBN1 (HI: 39.3±7.2 mm vs DN: 37.3±6.8 mm, p=0.022), with no differences in age or body surface area. After a mean follow-up of 4.9±2.0 years, aortic root and ascending dilation rates were increased in patients with HI- FBN1 (HI: 0.57±0.8 vs DN: 0.28±0.5 mm/year, p=0.004 and HI: 0.59±0.9 vs DN: 0.30±0.7 mm/year, p=0.032, respectively). Furthermore, patients with HI- FBN1 tended to be at increased risk for the combined endpoint of dissection and death compared with patients with DN- FBN1 (HR: 3.3, 95% CI 1.0 to 11.4, p=0.060)., Conclusions: Patients with an HI mutation had a more severely affected aortic phenotype, with larger aortic root diameters and a more rapid dilation rate, and tended to have an increased risk of death and dissections compared with patients with a DN mutation., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: Valve-sparing root replacement (VSRR) is an established therapy for aortic root pathology. However, late aortic valve dysfunction requiring reoperation remains a primary concern of this procedure. This study examines risk factors for late aortic insufficiency (AI) and aortic stenosis (AS) after David V VSRR., Methods: A retrospective review from 2005 to 2015 at a US academic center identified 282 patients who underwent VSRR. Cox proportional hazards regression analysis was used to identify risk factors for late AI and AS after VSRR., Results: The mean age was 46.4 years. Sixty-four patients (22.7%) had bicuspid valves, and 41 patients (14.5%) had Marfan syndrome. The incidence of reoperations was 27 (9.6%), and 42 cases (14.9%) presented with acute type A dissection. Operative mortality was 8 (2.8%). Seven-year survival was 90.9%. Seven-year cumulative incidence of reoperation, greater than 2+ AI and greater than moderate AS were 3.1%, 2.2%, and 0.8%, respectively. Multivariable analysis showed aortic root size 55 mm or larger (hazard ratio 3.44, 95% confidence interval: 1.27 to 9.29, p = 0.01) to be a risk factor for late AI whereas bicuspid valve (hazard ratio 16.07, 95% confidence interval: 3.12 to 82.68, p = 0.001) and cusp repair were found to be risk factors (hazard ratio 5.91, 95% confidence interval: 1.17 to 29.86, p = 0.03) for late AS., Conclusions: Valve-sparing root replacement can be performed with low operative risk and good overall long-term survival even in complex clinical settings. Durable valve function can be expected; however, aortic root size 55 cm or more, bicuspid valve anatomy, and cusp repair represent independent risk factors for late aortic valve dysfunction after these procedures., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background and Aim of the Study: The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation., Methods: Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29)., Results: No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33)., Conclusions: The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

Objective To evaluate the clinical features, risk factors, and prognostic significance of different Stanford types of acute aortic dissection (AAD). Methods We retrospectively analyzed the clinical data and prognostic predictors in 105 patients with AAD (37 with Stanford type A and 68 with Stanford type B) at Tianjin Medical University General Hospital and Tianjin 4th Central Hospital from January 2014 to November 2015. Results Patients with Marfan syndrome and bicuspid aortic valve constituted 24.3% and 8.1%, respectively, of patients with type A AAD; these proportions were significantly higher than those of patients with type B AAD (7.4% and 0.0%, respectively). The proportion of iatrogenic causes of type A AAD (8.1%) was significantly higher than that of type B AAD (0.0%). Computed tomography angiography showed that the proportion of involvement of the aortic arch and pericardial effusion (86.5% and 18.9%, respectively) in patients with type A AAD were higher than those in patients with type B AAD (23.5% and 5.9%, respectively). Endovascular treatment was performed in a higher proportion of patients with type B than A AAD (70.6% vs. 5.4%, respectively). Conclusion Systolic blood pressure, pericardial effusion, periaortic hematoma, conservative treatment, and open surgery were independent predictors of increased mortality in patients with AAD.

Background: In patients with Marfan syndrome (MFS), distal aortic dissection can necessitate thoracoabdominal aortic aneurysm (TAAA) repair in survivors of acute DeBakey type I dissection and those with DeBakey type III dissection. We examined outcomes of surgical repair of TAAA in patients with MFS with distal aortic dissection., Methods: Data were analyzed for 127 consecutive TAAA repairs performed between January 2004 and June 2014 in patients with MFS and distal aortic dissection-DeBakey types I (n = 73) and III (n = 54). The median time from dissection onset to TAAA repair was 5.2 years (interquartile range [IQR]: 2.1 to 9.8 years) for the overall group and was longer in patients with DeBakey I (6.5 years, IQR: 3.5 to 13.9 years) than patients with DeBakey III (2.9 years, IQR: 0.6 to 6.0 years, p < 0.001). Eleven patients (9%) had acute or subacute dissection at the time of repair. Sixty-six patients (52%) underwent Crawford extent II TAAA repair. A composite end point, adverse event, was defined as operative death or permanent stroke, renal failure, paraplegia, or paraparesis., Results: Eight patients had adverse events (6%), including 5 operative deaths (4%). There was no permanent stroke and 1 case each of permanent paraplegia and paraparesis. At discharge, 2 early survivors (2%) had renal failure. Extent II repairs did not have substantially different outcomes from other repairs., Conclusions: In these patients with MFS with aortic dissection, open TAAA repair incurred reasonable operative risk, but improvements are needed to reduce rates of renal failure. Extent II TAAA repair does not appear to increase operative risk in patients with MFS., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Pathologic conditions of the aorta remain a major source of morbidity and mortality for patients with Marfan syndrome (MFS). Extensive thoracoabdominal aortic aneurysm (TAAA) repair can prevent aortic catastrophe but carries substantial risk of perioperative adverse events. We evaluated midterm survival and quality of life (QoL) after contemporary Crawford extent II TAAA repair in patients with MFS., Methods: From 2004 to 2010, 49 consecutive patients with MFS (mean age, 43.4 ± 12.0 years) underwent extent II TAAA repair (41 elective and 8 urgent/emergent procedures) with intercostal reimplantation. Thirty-six patients (73%) had aorta-related symptoms, and 45 (92%) had distal aortic dissection. Operative adjuncts included cerebrospinal fluid drainage (n = 47 [96%]), left heart bypass (n = 46 [94%]), and cold renal perfusion (n = 47 [96%]). Kaplan-Meier survival analysis was performed. QoL was assessed in 24 patients with a 12-item survey (12-Item Short Form Health Survey version 2 [SF-12v2]) a median of 5.3 (interquartile range [IQR], 4.0-7.9) years postoperatively. QoL data were normalized and compared with data from the general population., Results: There were no operative deaths, strokes, paraparesis, or paraplegia. Two patients (4%) had permanent renal failure necessitating hemodialysis. The most frequent complication was vocal cord paralysis (n = 21 [43%]). Six-year Kaplan-Meier survival was 84% ± 6%. The 24 patients with QoL data had slightly worse physical component scores (46.0 ± 10.6) and slightly better mental component scores (51.4 ± 10.4) than the general population (50 ± 10 for both scores)., Conclusions: Operative treatment of extensive TAAA in patients with MFS enables excellent midterm survival and QoL. Cerebrospinal fluid drainage, left heart bypass, and cold renal perfusion probably aid in achieving excellent outcomes., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Genetic aortopathy (GA) underlies thoracic aortic aneurysms (TAA) in younger adults. Comparative survival and predictors of outcomes in nonsyndromic TAA (NS-TAA) are incompletely defined compared to Marfan syndrome (MFS) and bicuspid aortic valve (BAV)., Objectives: The study sought to compare survival and clinical outcomes for individuals with NS-TAA, MFS, and BAV., Methods: From 1988 to 2014, all patients presenting with GA 16 to 60 years of age were enrolled in a prospective study of clinical outcomes. Risk factors for death and aortic dissection were identified by Cox proportional hazards modeling and a mortality risk score developed., Results: Diagnosis of GA was made for 760 patients (age 36.9 ± 13.6 years, 26.8% female; NS-TAA, n = 311; MFS, n = 221; BAV, n = 228). MFS patients were younger than NS-TAA and BAV. Presentation with aortic dissection was more common for NS-TAA than MFS or BAV. The 687 patients surviving >30 days after presentation were followed for a median of 7 years. Calculated 10-year mortality was 7.8% for NS-TAA, 8.7% for MFS, and 3.5% for BAV (NS-TAA and MFS vs. BAV p <0.05). Factors associated with all-cause mortality were MFS (p = 0.04), age at presentation, and family history of dissection., Conclusions: Clinical outcomes for MFS and NS-TAA are similar but worse than BAV. Independent predictors of mortality, including family history of aortic dissection and age, can be included in an Aortopathy Mortality Risk Score to predict survival. Management of NS-TAA, including surgical intervention, should be similar to that of MFS., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined., Methods: Adult patients with Marfan syndrome who had Bentall or aortic valve-sparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact., Results: One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n = 98; 69 reimplantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P = .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P = .003), more aortic dissections (25.4% vs 4.1%; P < .001), higher incidence of moderate or severe aortic insufficiency (49.3% vs 14.4%; P < .001) and more urgent or emergent operations (24.6% vs 3.3%; P < .001). There were no hospital deaths and 9 late deaths in more than 17 years of follow-up (median, 7.8 deaths). Ten-year survival was 90.5% in patients undergoing Bentall procedure and 96.3% in patients undergoing VSRR (P = .10). Multivariable analysis revealed that VSRR was associated with fewer thromboembolic or hemorrhagic events (hazard ratio, 0.16; 95% confidence interval, 0.03-0.85; P = .03). There was no independent difference in long-term survival, freedom from reoperation, or freedom from endocarditis between the 2 procedures., Conclusions: After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery., Methods: Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 ± 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts., Results: Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) patients had already existing ischaemic cardiomyopathy before the first various cardiovascular surgeries, while ischaemic cardiomyopathy in the other 8 (72.7%) developed postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 8.0 ± 07 years. In Group B, 5 (38.4%) had existing primary cardiomyopathy prior to surgery, while 8 (61.5%) developed end-stage cardiomyopathy postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 9.0 ± 4 months. In Group C, 5 (21.7%) had been diagnosed with cardiomyopathy prior to the cardiovascular surgery, while 18 (78.2%) developed end-stage cardiomyopathy postoperatively. The mean interval between previous surgery and development of cardiomyopathy was 3 ± 0.9 years. At a mean follow-up of 9.4 ± 1.37 years, the overall survival rate is 51.8%. Categorized based on the surgical treatment done for cardiomyopathy, survival rates of 54.5% (the mean follow-up of 9.35 ± 1.8 years), 40.1% (mean follow-up of 7.01 ± 2.8 years) and 70% (mean follow-up of 10.5 ± 2.0 years) were seen in Groups A, B and C, respectively. There is no significant difference in survival rates (P = 0.56) among groups. Likewise, the type of myocardial protection and duration of ischaemic times were not significant (P > 0.78) to the development of cardiomyopathy., Conclusions: Our finding supports the existence of cardiomyopathy in a subset of patients with MFS. Marfan cardiomyopathy appears to be independent of the type of myocardial protection and duration of ischaemia., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Purpose: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy., Materials and Methods: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed β-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available., Results: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05)., Conclusion: In MFS patients who underwent ARR, the addition of RAAS blockade to β-blocker was associated with reduction of aortic dilatation and clinical events.

Background: In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes., Objectives: This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome., Methods: All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons., Results: A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%., Conclusions: Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: To evaluate the benefit of adding Losartan to baseline therapy in patients with Marfan syndrome (MFS)., Methods and Results: A double-blind, randomized, multi-centre, placebo-controlled, add on trial comparing Losartan (50 mg when <50 kg, 100 mg otherwise) vs. placebo in patients with MFS according to Ghent criteria, age >10 years old, and receiving standard therapy. 303 patients, mean age 29.9 years old, were randomized. The two groups were similar at baseline, 86% receiving β-blocker therapy. The median follow-up was 3.5 years. The evolution of aortic diameter at the level of the sinuses of Valsalva was not modified by the adjunction of Losartan, with a mean increase in aortic diameter at the level of the sinuses of Valsalva of 0.44 mm/year (s.e. = 0.07) (-0.043 z/year, s.e. = 0.04) in patients receiving Losartan and 0.51 mm/year (s.e. = 0.06) (-0.01 z/year, s.e. = 0.03) in those receiving placebo (P = 0.36 for the comparison on slopes in millimeter per year and P = 0.69 for the comparison on slopes on z-scores). Patients receiving Losartan had a slight but significant decrease in systolic and diastolic blood pressure throughout the study (5 mmHg). During the study period, aortic surgery was performed in 28 patients (15 Losartan, 13 placebo), death occurred in 3 patients [0 Losartan, 3 placebo, sudden death (1) suicide (1) oesophagus cancer (1)]., Conclusion: Losartan was able to decrease blood pressure in patients with MFS but not to limit aortic dilatation during a 3-year period in patients >10 years old. β-Blocker therapy alone should therefore remain the standard first line therapy in these patients., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

Background: Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers., Methods: We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events., Results: From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [±SD] age, 11.5±6.5 years in the atenolol group and 11.0±6.2 years in the losartan group), who had an aortic-root z score greater than 3.0. The baseline-adjusted rate of change in the mean (±SE) aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139±0.013 and -0.107±0.013 standard-deviation units per year, respectively; P=0.08). Both slopes were significantly less than zero, indicating a decrease in the aortic-root diameter relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups., Conclusions: Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aortic-root dilatation between the two treatment groups over a 3-year period. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00429364.).

Objectives: Technical variations of the David reimplantation valve-sparing aortic root replacement (V-SARR) procedure have been proposed to be advantageous in patients with connective tissue disease, such as the Marfan syndrome (MFS). We report results of a Marfan cohort treated exclusively with the non-modified David I procedure., Methods: Forty-eight Marfan patients (25 males, mean age 33 ± 12 years, range 15-62 years) underwent the original variant of the David V-SARR (David I) between 1997 and 2013. Forty-two operations (88%) were performed as elective procedures for aortic root aneurysms and six for acute dissections (12%). Seventeen had aortic regurgitation (AR) grades ≥2+ preoperatively, and 3 had AR >2+. No patients with severe AR (4+) were selected for V-SARR. Three full or hemi-arch replacements were performed. Patients who were operated on using a variation of the David I or David II procedure were excluded., Results: Mean prosthesis size was 28 ± 3 mm (18-30 mm). Mean clinical and echocardiographic follow-up (98% complete) was 3.8 ± 3.7 years with a cumulative follow-up of 178 patient-years. The early mortality rate was 2% (one hospital death). The survival rate was 98% (95% confidence 84-99%) at 4 years and 90% (57-98%) at 8 years with 5 patients at risk at 10 years. The rate of freedom from root or valve reoperation was 97% (79-99%) and 97% (79-99%) at 4 and 8 years, respectively. Only one patient required mechanical aortic valve replacement for progression of AR., Conclusions: Despite potential theoretical drawbacks of the David I V-SARR technique without neo-sinuses or a neo-sinotubular junction, it results in a favourable mid-term outcome in Marfan patients and compares well with reported results of different modifications of David V-SARR., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The study objective was to evaluate patients with Marfan syndrome and mitral valve regurgitation undergoing valve repair or replacement and to compare them with patients undergoing repair for myxomatous mitral valve disease., Methods: We reviewed the medical records of consecutive patients with Marfan syndrome treated surgically between March 17, 1960, and September 12, 2011, for mitral regurgitation and performed a subanalysis of those with repairs compared with case-matched patients with myxomatous mitral valve disease who had repairs (March 14, 1995, to July 5, 2013)., Results: Of 61 consecutive patients, 40 underwent mitral repair and 21 underwent mitral replacement (mean [standard deviation] age, 40 [18] vs 31 [19] years; P = .09). Concomitant aortic surgery was performed to a similar extent (repair, 45% [18/40] vs replacement, 43% [9/21]; P = .87). Ten-year survival was significantly better in patients with Marfan syndrome with mitral repair than in those with replacement (80% vs 41%; P = .01). Mitral reintervention did not differ between mitral repair and replacement (cumulative risk of reoperation, 27% vs 15%; P = .64). In the matched cohort, 10-year survival after repair was similar for patients with Marfan syndrome and myxomatous mitral disease (84% vs 78%; P = .63), as was cumulative risk of reoperation (17% vs 12%; P = .61)., Conclusions: Patients with Marfan syndrome and mitral regurgitation have better survival with repair than with replacement. Survival and risk of reoperation for patients with Marfan syndrome were similar to those for patients with myxomatous mitral disease. These results support the use of mitral valve repair in patients with Marfan syndrome and moderate or more mitral regurgitation, including those having composite replacement of the aortic root., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background and Aim of the Study: Several recent studies have compared the clinical results of valve-sparing (VS) surgery and composite valve graft (CVG) surgery in the aortic root reconstruction of Marfan syndrome (MS) patients. The study aim was to investigate whether it is appropriate to preserve the native aortic valve in root surgery of MFS patients when taking the short-term and long-term prognoses into consideration., Method: A thorough literature search of PubMed, Embase and Cochrane library was conducted to identify studies comparing the outcomes of VS and CVG surgery in MFS patients. The Newcastle-Ottawa Scale evaluation scheme was used to assess the methodological quality of the included articles. Data were extracted from reports and analyzed using Revman 5.0, supplied by Cochrane collaboration., Result: Six clinical trials incorporating 539 patients were included. Compared to CVG, VS surgery was associated with a lower risk for re-exploration (RR 0.48, 95% CI 0.24-0.97; p = 0.04), thromboembolic events (RR 0.17, 95% CI 0.05-0.57; p = 0.004) and endocarditis (RR 0.31, 95% CI 0.11-0.94; p = 0.04). Despite an inherent incidence of aortic regurgitation, VS surgery resulted in an elevation of long-term survival rate; however, no statistical differences were found between groups with regards to reoperation (RR 1.07, 95% CI 0.35-3.27; p = 0.91)., Conclusion: Root reconstruction with VS surgery can effectively improve the prognosis of MFS patients and provide a promising alternative for surgical treatment. However, the results must be interpreted with caution due to the retrospective nature of the included studies; large-scale prospective control trials are needed to confirm these findings.

Objective: To compare the 1-year results after aortic valve-sparing (AVS) or valve-replacing (AVR) aortic root replacement from a prospective, international registry of 316 patients with Marfan syndrome (MFS)., Methods: Patients underwent AVS (n = 239, 76%) or AVR (n = 77, 24%) aortic root replacement at 19 participating centers from 2005 to 2010. One-year follow-up data were complete for 312 patients (99%), with imaging findings available for 293 (94%). The time-to-events were compared between groups using Kaplan-Meier curves and Cox proportional hazards models., Results: Two patients (0.6%)--1 in each group--died within 30 days. No significant differences were found in early major adverse valve-related events (MAVRE; P = .6). Two AVS patients required early reoperation for coronary artery complications. The 1-year survival rates were similar in the AVR (97%) and AVS (98%) groups; the procedure type was not significantly associated with any valve-related events. At 1 year and beyond, aortic regurgitation of at least moderate severity (≥2+) was present in 16 patients in the AVS group (7%) but in no patients in the AVR group (P = .02). One AVS patient required late AVR., Conclusions: AVS aortic root replacement was not associated with greater 30-day mortality or morbidity rates than AVR root replacement. At 1 year, no differences were found in survival, valve-related morbidity, or MAVRE between the AVS and AVR groups. Of concern, 7% of AVS patients developed grade ≥2+ aortic regurgitation, emphasizing the importance of 5 to 10 years of follow-up to learn the long-term durability of AVS versus AVR root replacement in patients with MFS., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Marfan syndrome (MFS) is an autosomal dominant disorder caused by mutations in the fibrillin 1 gene (FBN1). Neonatal form of MFS is rare and is associated with severe phenotype and a poor prognosis. We report on a newborn girl with neonatal MFS who displayed cyanosis and dyspnea on the first day of life. The main clinical features included mitral and tricuspid valve insufficiency, aortic root dilatation, arachnodactyly, and loose skin. Despite the presence of severe and inoperable heart anomalies, the girl was quite stable on symptomatic treatment and lived up to the 7th month of age when she died due to cardiorespiratory failure. Molecular-genetic studies revealed a novel intronic c.4211-32&#95;-13del mutation in the FBN1 gene. Subsequent in vitro splicing analysis showed this mutation led to exon 35 skipping, presumably resulting in a deletion of 42 amino acids (p.Leu1405&#95;Asp1446del). Interestingly, this mutation is localized outside the region of exons 24-32, whose mutation is responsible for the substantial majority of cases of neonatal MFS. Although the family history of MFS was negative, the subsequent molecular genetic examination documented a mosaicism of the same mutation in the maternal blood cells (10-25% of genomic DNA) and the detailed clinical examination showed unilateral lens ectopy., (© 2014 Wiley Periodicals, Inc.)

Objectives: Mitral valve (MV) regurgitation is a common manifestation in patients with Marfan syndrome (MFS) and is age dependent. Valve pathology shares some features with myxomatous MV disease. Surgical treatment is still being debated and not well characterized in patients with MFS., Patients and Methods: We retrospectively evaluated the results of mitral valve repair (MVR) of symptomatic patients with MFS who underwent surgery between January 2004 and April 2011. MFS was diagnosed following the Ghent criteria. MVR was performed in 12 patients. Three patients underwent minimally invasive MVR despite severe thorax deformities. Mean follow-up was 60.1 months (95% CI: 48-72) and was complete., Results: Thirty-day mortality was 0%. One patient died because of arrhythmia 66 months after MVR. Transthoracic echocardiography at last visit showed mild mitral regurgitation in one patient (8.3%) and no mitral regurgitation in the remaining patients (91.7%)., Conclusion: MVR was associated with excellent survival and a low rate of complications. Transthoracic echocardiography showed good results of the repaired valves even years later. Minimally invasive repairs are feasible even in deformed thoraces, lowering the risk for future aortic surgery. Because of excellent mid-term to long-term results, MVR may also be justified in asymptomatic Marfan patients., (Georg Thieme Verlag KG Stuttgart · New York.)

Background and Aim of the Study: The Bentall operation is a 40-year-old standardized procedure for treating aortic valve diseases and aneurysms involving the aortic root. The study aim was to analyze the results and predictors of long-term outcome after the Bentall procedure for aortic root diseases., Methods: Between January 1990 and December 2007, a total of 375 patients (296 males, 79 females) underwent the Bentall operation at the authors' institution. Bicuspid aortic valve (BAV) was present in 91 patients, and Marfan syndrome in 13. Thirty-six patients were treated as emergencies, and 30 for acute dissection. A concomitant surgical procedure was performed in 78 patients. The operative procedure included both classic Bentall and button techniques. Follow up data were obtained from hospital and office records and from telephone contacts. Kaplan-Meier survival analysis and Cox regression analysis were performed to investigate the predictors of long-term outcome., Results: The overall in-hospital mortality was 4.5%, and after elective operations was 2.3%. A 20-year long-term follow up included 32 late deaths, of which 14 were cardiac-related. Freedom from late all-cause mortality at 5, 10, and 15 years was 97.1%, 81.9%, and 53.9%, respectively. At univariate analysis, long-term mortality was associated with age, diabetes, BAV, NYHA class III/IV, emergency treatment, cardiopulmonary bypass time, and coronary artery bypass grafting. Independent predictors of long-term mortality were age (OR 1.16; CI: 1.08-1.23), emergency surgery (OR 28; CI: 4-192) and BAV (OR 3; CI: 1.3-6.9)., Conclusion: The Bentall procedure is a safe and durable operation, with a very good early and long-term results and a low rate of reoperation. In the present series, age, BAV and emergency surgery were important independent predictors of mortality.

Objectives: To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair., Background: MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear., Methods: Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%., Results: The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients., Conclusions: Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair., (Copyright © 2013 Wiley Periodicals, Inc.)

Background: Marfan syndrome (MFS) is a variable, autosomal-dominant disorder of the connective tissue. In MFS serious ventricular arrhythmias and sudden cardiac death (SCD) can occur. The aim of this prospective study was to reveal underlying risk factors and to prospectively investigate the association between MFS and SCD in a long-term follow-up., Methods: 77 patients with MFS were included. At baseline serum N-terminal pro-brain natriuretic peptide (NT-proBNP), transthoracic echocardiogram, 12-lead resting ECG, signal-averaged ECG (SAECG) and a 24-h Holter ECG with time- and frequency domain analyses were performed. The primary composite endpoint was defined as SCD, ventricular tachycardia (VT), ventricular fibrillation (VF) or arrhythmogenic syncope., Results: The median follow-up (FU) time was 868 days. Among all risk stratification parameters, NT-proBNP remained the exclusive predictor (hazard ratio [HR]: 2.34, 95% confidence interval [CI]: 1.1 to 4.62, p=0.01) for the composite endpoint. With an optimal cut-off point at 214.3 pg/ml NT-proBNP predicted the composite primary endpoint accurately (AUC 0.936, p=0.00046, sensitivity 100%, specificity 79.0%). During FU, seven patients of Group 2 (NT-proBNP ≥ 214.3 pg/ml) reached the composite endpoint and 2 of these patients died due to SCD. In five patients, sustained VT was documented. All patients with a NT-proBNP<214.3 pg/ml (Group 1) experienced no events. Group 2 patients had a significantly higher risk of experiencing the composite endpoint (logrank-test, p<0.001)., Conclusions: In contrast to non-invasive electrocardiographic parameter, NT-proBNP independently predicts adverse arrhythmogenic events in patients with MFS., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Objective: Although implicitly accepted by many that the durability of valve-sparing aortic root replacement in patients with bicuspid aortic valve disease and connective tissue disorders will be inferior, this hypothesis has not been rigorously investigated., Methods: From 1993 to 2009, 233 patients (27% bicuspid aortic valve, 40% Marfan syndrome) underwent Tirone David valve-sparing aortic root replacement. Follow-up averaged 4.7 ± 3.3 years (1102 patient-years). Freedom from adverse outcomes was determined using log-rank calculations., Results: Survival at 5 and 10 years was 98.7% ± 0.7% and 93.5% ± 5.1%, respectively. Freedom from reoperation (all causes) on the aortic root was 92.2% ± 3.6% at 10 years; 3 reoperations were aortic valve replacement owing to structural valve deterioration. Freedom from structural valve deterioration at 10 years was 96.1% ± 2.1%. No significant differences were found in survival (P = .805, P = .793, respectively), reoperation (P = .179, P = .973, respectively), structural valve deterioration (P = .639, P = .982, respectively), or any other functional or clinical endpoints when patients were stratified by valve type (tricuspid aortic valve vs bicuspid aortic valve) or associated connective tissue disorder. At the latest echocardiographic follow-up (95% complete), 202 patients (94.8%) had none or trace aortic regurgitation, 10 (4.7%) mild, 0 had moderate to severe, and 1 (0.5%) had severe aortic regurgitation. Freedom from greater than 2+ aortic regurgitation at 10 years was 95.3% ± 2.5%. Six patients sustained acute type B aortic dissection (freedom at 10 years, 90.4% ± 5.0%)., Conclusions: Tirone David reimplantation valve-sparing aortic root replacement in carefully selected young patients was associated with excellent clinical and echocardiographic outcome in patients with either a tricuspid aortic valve or bicuspid aortic valve. No demonstrable adverse influence was found for Marfan syndrome or connective tissue disorder on durability, clinical outcome, or echocardiographic results., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background and Aim of the Study: In patients with Marfan syndrome (MFS), valve reimplantation has been proposed as superior to root remodeling. In the present study, both forms of valve-preserving root repair were applied and mid-term results analyzed in MFS patients compared to a propensity score-matched cohort., Methods: Among 604 patients who underwent valve-preserving aortic root surgery between 1995 and 2011 at the authors' institution, 33 MFS patients (16 males, 17 females; mean age 31 +/- 12 years) underwent either remodeling (n=21) or reimplantation (n=12). All patients were followed up echocardiographically, and the outcome with regard to late aortic valve regurgitation (AR) grade EII and reoperation on the aortic valve was compared between MFS patients and the matched cohort (n=33)., Results: Baseline characteristics and operative data were similar between the groups. Actuarial freedom from AR > or = II at seven years was 86 +/- 8% in MFS patients and 90 +/- 10% in matched non-MFS patients (p = 0.94). Actuarial freedom from reoperation at seven years was 90 +/- 7% in MFS patients and 100% in non-MFS patients (p = 0.79). In Cox's proportional hazard's model, no independent risk factor, including MFS, was found for recurrent AR or reoperation. Within the MFS patients, remodeling and reimplantation provided an almost identical freedom from late AR > or = II and reoperation up to five years postoperatively (p = 0.55 and 0.99, respectively)., Conclusion: The stability of valve-preserving aortic root repair was comparable between patients with or without MFS. Both forms of valve-preserving root repair can provide similar mid-term results for MFS patients, primarily according to their root geometry. However, additional long-term follow up data based on a larger number of patients are required to confirm the evidence obtained to date.

Purpose: We retrospectively analyzed 28 years of experience with the Bentall procedure in patients with aortic valve, aortic root, and ascending aortic disease., Materials and Methods: Between March 1982 and December 2010, a total of 218 patients underwent the Bentall procedure using a composite valved conduit. The "inclusion technique" was used in 30 patients (13.8%), the "open-button technique" in 181 patients (83.0%), and the Cabrol technique in 7 patients (3.2%)., Results: The early mortality rate was 5.5% (12/218). The mean follow-up duration was 108.0±81.0 months (range: 1-329 months). Seven patients required re-operation, and 1 patient required stent graft insertion at the descending thoracic aorta for progression of aortic arch or descending thoracic aortic dissection or aneurysm after the first operation, and 5 of them had Marfan syndrome. Kaplan-Meier estimated survival rates at 1, 5, 10, 20, and 25 years were 90.4%, 82.7%, 77.6%, 65.3%, and 60.3%, respectively. Freedom from reoperation rates at 1, 5, 10, 20, and 25 years were 99.0%, 98.3%, 95.5%, 90.8%, and 90.8%, respectively., Conclusion: In our experience, the Bentall procedure provided optimal survival with improved functional status. The disease of the aorta may progress, especially in patients with Marfan syndrome. Therefore, careful follow-up with regular computed tomography angiograms should be performed in these patients.