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Background and Aims To explore male–female differences in aneurysm growth and clinical outcomes in a two-centre retrospective Dutch cohort study of adult patients with ascending aortic aneurysm (AscAA). Methods Adult patients in whom imaging of an AscAA (root and/or ascending: ≥40 mm) was performed between 2007 and 2022 were included. Aneurysm growth was analysed using repeated measurements at the sinuses of Valsalva (SoV) and tubular ascending aorta. Male–female differences were explored in presentation, aneurysm characteristics, treatment strategy, survival, and clinical outcomes. Results One thousand eight hundred and fifty-eight patients were included (31.6% female). Median age at diagnosis was 65.4 years (interquartile range: 53.4–71.7) for females and 59.0 years (interquartile range: 49.3–68.0) for males (P <.001). At diagnosis, females more often had tubular ascending aortic involvement (75.5% vs. 70.2%; P =.030) while males more often had SoV involvement (42.8% vs. 21.6%; P <.001). Maximum absolute aortic diameter, at any location, at diagnosis did not differ between females (45.0 mm) and males (46.5 mm; P =.388). In females, tubular ascending growth was faster (P <.001), whereas in males, SoV growth was faster (P =.005), corrected for covariates. Unadjusted 10-year survival was 72.5% [95% confidence interval (CI) 67.8%–77.6%] for females and 78.3% (95% CI 75.3%–81.3%) for males (P =.010). Twenty-three type A dissections occurred, with an incidence rate of 8.2/1000 patient-years (95% CI 4.4–14.1) in females and 2.4/1000 patient-years (95% CI 1.2–4.5) in males [incidence rate ratio females/males: 3.4 (95% CI 1.5–8.0; P =.004)]. Conclusions In patients having entered a diagnostic programme, involvement of aortic segments and age- and segment-related growth patterns differ between women and men with AscAA, particularly at an older age. Unravelling of these intertwined observations will provide a deeper understanding of AscAA progression and outcome in women and men and can be used as an evidence base for patient-tailored clinical guideline development. [ABSTRACT FROM AUTHOR]

Marfan syndrome (MFS) is a systemic connective tissue disorder stemming from mutations in the gene encoding Fibrillin-1 (Fbn1), a key extracellular matrix glycoprotein. This condition manifests with various clinical features, the most critical of which is the formation of aortic root aneurysms. Reduced nitric oxide (NO) production due to diminished endothelial nitric oxide synthase (eNOS) activity has been linked to MFS aortic aneurysm pathology. Caveolin-1 (Cav1), a structural protein of plasma membrane caveolae, is known to inhibit eNOS activity, suggesting its involvement in MFS aneurysm progression by modulating NO levels. In this study, we examined the role of Cav1 in aortic smooth muscle and endothelial function, aortic wall elasticity, and wall strength in male and female MFS mice (FBN1+/Cys1041Gly) by generating developing Cav1-deficient MFS mice (MFS/Cav1KO). Our findings reveal that Cav1 ablation leads to a pronounced reduction in aortic smooth muscle contraction in response to phenylephrine, attributable to an increase in NO production in the aortic wall. Furthermore, we observed enhanced aortic relaxation responses to acetylcholine in MFS/Cav1KO mice, further underscoring Cav1's inhibitory impact on NO synthesis within the aorta. Notably, van Gieson staining and chamber myography analyses showed improved elastin fiber structure and wall strength in male MFS/Cav1KO mice, whereas these effects were absent in female counterparts. Cav1's regulatory influence on aortic root aneurysm development in MFS through NO-mediated modulation of smooth muscle and endothelial function, with notable sex-dependent variations. [ABSTRACT FROM AUTHOR]

Background: To evaluate different aortic root surgery techniques and their contemporary clinical outcomes in patients with regurgitant aortic valve and aortic root aneurysm. Methods: The study consisted of 141 adult patients who underwent aortic valve reimplantation (David group = 73) or aortic valve replacement surgery (Bentall group = 68) for aortic valve regurgitation (AR) and dilatation of the aortic root at our institution within the same period (April 2004–October 2016). Kaplan–Meier method was used to estimate survival and other clinically relevant outcomes between the groups. Results: The completeness of clinical follow-up was 100%, with a mean time of 8.0 ± 3.8 years. Thirty-day (in-hospital) mortality rates were equivalent between groups (1.3 and 1.5%, p = 1.0). The overall survival rates at 10 years were significantly better for the David group patients comparing to Bentall group patients (95.3 ± 2.6% vs 79.7 ± 6.8%; p = 0.04) with similar freedom from AV related reoperation (94.4 ± 2.7% vs 98.5 ± 1.5%; p = 0.2). Freedom from bleeding events at 10 years was 90.7 ± 3.6% for Bentall group patients and none were observed among David group patients (p = 0.01). Conclusions: Aortic valve and root surgery can be performed with equivalent safety and efficacy using either valve-sparing (David procedure) or valve-replacing (Bentall procedure) techniques in selected patients. Furthermore, patients after the David procedure demonstrated significantly improved survival and low risk of bleeding in comparison to the Bentall procedure with an acceptable risk of reoperation at 10 years follow-up. [ABSTRACT FROM AUTHOR]

Background: Aortic root and ascending aortic aneurysms are traditionally surgically treated through the deployment of a conduit with an artificial aortic valve, which significantly increases the risk of postoperative complications in the form of thrombosis.Materials& Methods: We report a case of Wolfe procedure in a 78-year-old female patient with aortic root aneurysm at high risk for conventional Bentall surgery.Discussion and Conclusion: We use this case to discuss the effectiveness and short-term results of this procedure. [ABSTRACT FROM AUTHOR]

The risk of aortic dissection and aortic aneurysm in patients with Marfan syndrome has been known for years. A 46-year-old man with Marfan's syndrome was admitted to our clinic with a complaint of chest pain and palpitation in the last year. Hemiarcus replacement, aortic valve resuspension and graft interposition between innominate artery and prosthetic ascending aorta was performed 4 years ago due to type 1 aortic dissection. Echocardiography and computed tomography revealed dilatation of aortic root (68 mm in diameter), with advanced aortic valve insufficiency. Hence, we successfully performed aortic root replacement with Bentall procedure. The postsurgical course was uneventful. We conclude that aortic root aneurysm is a significant long-term complication after repair of the ascending aorta in patients with Marfan's syndrome, and these patients should be follow-up close and lifelong in terms of development of aneurysm at the level of the residual aorta. [ABSTRACT FROM AUTHOR]

Concomitant replacement of the ascending aorta and heart transplantation are an infrequent procedure. This procedure was most often performed in patients with Marfan syndrome, however, it has its own technical difficulties. Hereby, we report on combined heart transplantation and aortic root replacement using donor's ascending aorta in a 25-year-old man diagnosed with dilated cardiomyopathy and ascending aorta aneurysm. This procedure was successful and beneficial to patients with aortopathy who are candidates for heart transplantation. [ABSTRACT FROM AUTHOR]

A 34 yrs old man of marfan syndrome, visited us with the complaints of palpitation, easy fatigability and chest discomfort for last 1year. He was pointed out to have the aortic root enlargement with severe aortic regurgitation & mild mitral regurgitation by echocardiography and CT angiogram. We excised the aneurysm, implanted a composite graft with prosthetic valve conduit (Bentall operation), directly attached the coronary arteries to the aortic graft, and made the distal anastomosis to the divided aorta. Postoperative course was uneventful. To our knowledge, this is the first successful case of Bentall operation in national Institute of cardiovascular Diseases. [ABSTRACT FROM AUTHOR]

An aortic aneurysm complicating Kawasaki disease (KD) is extremely rare. We herein report a case of 48-year-old KD patient with severe aortic regurgitation, aneurysm of the aortic root, ascending aorta and aortic arch, and giant heavily calcified coronary aneurysms. The patient underwent successful surgical management. This case raises some unusual technical issues, which are discussed. A review of the literature is also offered. [ABSTRACT FROM AUTHOR]

Purpose: The advantages of aortic valve-preserving surgery are still hampered by a higher rate of reoperations compared to root replacement with valved conduits. This study evaluates whether valve deterioration rate is related to the method or depends on stringent adherence to technical concepts, which might be lost once trainees perform this complex surgery on their own. Methods: From 1991 to 2011, the David procedure was performed in 209 consecutive patients. Mean age was 57 ± 14 years. The patients were operated either by the senior author or trainees under his supervision (group 1, n = 130) or by surgeons on their own after training by the senior author (group 2, n = 79). Clinical and echocardiography data were evaluated pre- and postoperatively and at follow-up (mean 6.0 ± 4 years). Results: In-hospital mortality was 1.5 % in group 1 and 5 % in group 2 ( p = 0.29), and late mortality was 12 % ( n = 12 in group 1 and n = 14 in group 2, p = 0.11), three were cardiac related. Nine patients (4.3 %) had to be reoperated; three for endocarditis in group 1, six for structural valve deterioration in group 2 ( p = 0.14). The 9-year freedom from reoperation or aortic valve insufficiency (AI) ≥2° was 93 % in group 1 and 78 % in group 2 ( p < 0.01). As groups showed differences in preoperative variables, results were compared also in a propensity matched subgroup. Despite no difference in perioperative results, long-term valve competence remained inferior in group 2. Conclusions: With stringent adherence to technical concepts, structural valve deterioration may virtually be considerably reduced in aortic valve reimplantation. Once performing this operation on their own, trainees-after training by the senior-achieved results as independent surgeons well comparable to published series. As long-term performance seems to depend more on judgment of the geometry achieved intraoperatively than on technical steps, a means of measurement of effective coaptation height with a caliper might facilitate evaluation of perfect repair. [ABSTRACT FROM AUTHOR]

OBJECTIVES We aimed at evaluating the results of aortic valve-sparing root replacement (AVSRR) in children with aortic root aneurysm (ARA) due to genetic disorders in terms of mortality, reoperation and recurrent aortic valve regurgitation (AVR). METHODS Thirteen patients (mean age 9.7 ± 6.5 years, 10 months–18 years) underwent AVSRR for ARA between 2002 and 2011. Six of the 13 patients had Marfan syndrome, 3 Loeys–Dietz syndrome (LDS), 2 bicuspid aortic valve syndrome and 2 an unspecified connective tissue disorder. AVR was graded as none/trace, mild and severe in 5, 7 and 1 patient, respectively. The mean pre-operative root diameter was 45 ± 10 mm (mean Z-score 10.3 ± 2.0). Remodelling of the aortic root was performed in 4 patients, reimplantation of the aortic valve in 9 and a concomitant cusp repair in 4. The diameter of the prostheses used for root replacement varied from 22 to 30 mm (mean Z-score = 2.3 ± 3). The follow-up was 100% complete with a mean follow-up time of 3.7 years. RESULTS There was no operative mortality. One patient with LDS died 2.5 years after the operation due to spontaneous rupture of the descending aorta. Root re-replacement with mechanical conduit was necessary in 1 patient for severe recurrent AVR 8 days after remodelling of the aortic root. At final follow-up, AVR was graded as none/trace and mild in all patients. Eleven patients presented in New York Heart Association functional Class I and 1 in Class II. CONCLUSIONS In paediatric patients with ARA, valve-sparing root replacement can be performed with low operative risk and excellent mid-term valve durability. Hence, prosthetic valve-related morbidity may be avoided. Due to the large diameters of the aortic root and the ascending aorta, the size of the implanted root prostheses will not limit later growth of the native aorta. [ABSTRACT FROM AUTHOR]

The aorta is a large blood vessel that carries blood from the heart to the rest of the body. The aortic root consists of the aortic valve and the openings for the coronary arteries (the coronary ostia). An aortic aneurysm is an abnormal bulge in the wall of the aorta. If an aneurysm develops in the aortic root, the aorta can dilate and the aortic valve can leak. If the aneurysm continues to expand, it can rupture and can cause life-threatening internal bleeding. Aneurysms of the aortic root are often due to degenerative disease of media. Aneurysm of the aortic root often affects patients in their second to fourth decades of life. These aneurysms can cause aortic insufficiency, dissection, and/or rupture. Small and slow-growing thoracic aortic aneurysms may not ever rupture, but large, fast-growing aneurysms may rupture. Depending on the size and growth rate of aortic aneurysm, treatment may vary from watchful waiting to emergency surgery. Ideally, surgery for a thoracic aortic aneurysm can be planned if necessary. Current guidelines recommend surgical treatment when the diameter of the aneurysm exceeds 50 mm. Here we report a case of thoracic aneurysm with postductal coarctation of aorta. [ABSTRACT FROM AUTHOR]

Introduction: Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age.Case: The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed 190.5 cm of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. Transthoracic echocardiography and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we performed Bentall procedure based on the results of multidisciplinary team discussion and intraoperative exploration. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later.Conclusion: It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient's specific situation should be taken into consideration. A mechanical Bentall procedure seems to be an acceptable option for some selected cases. [ABSTRACT FROM AUTHOR]